Vpeds

Front 1

what are the components of a well child visit

Back 1

H-DOPIA
H- history - age specific trigger questions
D- age spec. ques, questionairres, school performance
O- obs. of parent/child interaction
P- physical examination - focuses on growth
I - immunizations
A - anticipatory guidance

Front 2

what measures of growth are used for a child

Back 2

height/weight - use standardized growth curves
head circumference
body mass index

Front 3

1. what is failure to thrive
2. what growth measurement is usually not effected by failure to thrive

Back 3

1. growth rate of less than expected for a child (most commonly weight)
>2 major percentile bars = MAJOR CONCERN
2. HEAD CIRCUMFERANCE

Front 4

what is isolated short stature

Back 4

NOT FAILURE TO THRIVE *****
height is the most abnormal growth parameter

Front 5

expected weight gains*
1. birth - 3 months
2. 3-6 months
3. 6-12 months
4. 1-2 years
5. 2 years to adolescence

Back 5

1. 30g/day ~ regain birth weight by 2 weeks
2. 20g/day ~ double brith weight by 4-6 months
3. 10g/day
4. 250g/month
5. 2kg/year

Front 6

at what age should an child
1. regain birth weight
2. double birth weight
3. triple birth weight

Back 6

1. by 2 weeks
2. by 4-6 months
3. by 12 months

Front 7

expected height gains*
1. 0-12 months
2. 13-24 months
3. 2 years- adolescence

Back 7

1. 25cm/year
2. 12.5cm/year
3. 6.25 cm/year

Front 8

at what age should a child
1. increase birth length by 50%
2. double birth length
3. triple birth length

Back 8

1. 12 months
2. 4 years
3. 13 years

Front 9

1. inorganic failure to thrive
2. organic failure to thrive

Back 9

1. disturbed parent/child bond - poor formula, poor feeding, abuse/neglect, maternal depression, family violence, poverty, no support systems
2. pathology, infection, chromosomal disorders, systemic illness

Front 10

list some head growth abnormalities

Back 10

microcephaly
craniosynostosis
deformational plagiocephaly
macrocephaly

Front 11

head size progression from birth to one year

Back 11

25% adult size--> 75% adult size

Front 12

cephalohemotoma

Back 12

superiosteal hemorrhage of the cranium after traumatic delivery, DOES NOT CROSS SUTURES

Front 13

expected head size circumference increase**
1. 0-2 months
2. 2-6 months
3. total increase at 12 months

Back 13

1. 0.5 cm/week
2. 0.25cm/week
3. 12 cm since birth

Front 14

1/1000 children has microcephaly
1. congenital microcephaly
2. acquired microcephaly

Back 14

1. abnormal induction/migration of brain tissue
2. cerebral insult in third trimester/perinatal period of life

Front 15

clinical features of microcephaly

Back 15

small brain
developmental delay/intellectual impairment
cerebral palsy
seizures

Front 16

causes of microcephaly

Back 16

TORCH
fetal alcohol/fetal hydantoin
trisomys (21, 13, 18)
perinatal infections
hypoxia/ischemia
metabolic defects
PKU
hypothyroidism

Front 17

craniosynotosis
1. definition
2. etiology
3. dx
4. tx

Back 17

1. premature closure of sutures
2. sporadic (90%), familial (crouzon, apert), hyperthyroid, hypercalcemia, intrauterine crowding
3. radiographs/CT
4. surgical repair

Front 18

when does suture closure normally occur

Back 18

90% by age 2, complete by age 5

Front 19

dolichocephaly/scaphocephaly

Back 19

CRANIOSYNOTOSIS
premature closture of the saggital suture-->elongated skull
* most common form of craniosynostosis

Front 20

brachycephaly

Back 20

CRANIOSYNOTOSIS
premature closure of the coronal suture leading to shortend skull ** more commonly associated with neurological complications

Front 21

trigonocephaly

Back 21

CRANIOSYNOTOSIS
premature closure of the metopic suture leads to angular shaped head

Front 22

plagiocephaly
1. definition
2. cause
3. clinical association
4. tx

Back 22

1. asymmetry of infant head not associated with premature suture closure
2. infant sleeping on their back (trying to avoid SIDS)
3. congenital muscular torticollis
4. ROM exercises, head repositioning, "tummy time"

Front 23

positional plagiocephaly

Back 23

flattening of the occiput and prominence of ipsilateral frontal area

Front 24

macrocephaly
1. does macrocephaly reflect brain size
2. causes
3. dx/tx

Back 24

1. NO ~ (compare to microcephaly)
2. familial,
overgrowth (ex. sotos syndrome)+
metabolic storage (canavan syndrome, gangliosidoses)
neurofibromatosis
achondroplasai
hydrocephalus
3. dx rule out elevated intracranial pressure, measure parental head circumference --> CT scan to rule out hydrocephalus

Front 25

sotos syndrome

Back 25

hereditary gigantism

Front 26

cornerstone of pediatric preventative care =?

Back 26

VACCINES!

Front 27

live vaccines
1. duration of immunity
2. contraindications
3. list live vaccines

Back 27

1. LONG
2. contraindicated in patients with compromised immunity
3. MR VZ MAPSY - measels, rubella, varicella, zoster, mumps, adeno, polio (oral), yellow fever

Front 28

non - live vaccines
1. duration of immunity
2. contraindicatiosn
3. list of non-live vaccines

Back 28

1. short (need boosters)
2. none
3. DTap (and Tdap), hepatitis A/B, inactivated polio, HITB, influenza, pneumococcal, meningococcal

Front 29

diseases for which passive immunity can be administered

Back 29

VZIG- varicella
HBIG - hepatitis B - given to newborns born to seropositive mothers
HAIG- hepatiatis A - given to those visiting high risk areas

Front 30

hepatits B vaccine
1. components
2. timing of administration

Back 30

1. recombinant HbSAg
2. three shot series given in first year

Front 31

DTaP
1. components
2. timing
3. DTP?
4. dT?

Back 31

1. purified acellullar pertussis, tetanus toxoid, diptheria toxoid
2. 2,4,6 months + boosters 12-18 months, and 4-6 years of age
3. old vaccine - contained whole cell killed pertussis ~ replaced with DTaP b/c it had high rate of side effects
4. 1/10 dose diptheria + tetanus toxoid given as booster --given every 10 years to those greater than 7 yo

Front 32

polio disease

Back 32

enterovirus (picornavirus)
destroys gray matter of the spinal cord in the anterior horn leading to lower motor neuron weakness and transient/perminent paresis and meningoencephalitis

Front 33

live polio (sabin)
1. advantages
2. disadvantages

Back 33

1. induces host immunity + herd immunity (secondary immunity) because the vaccine is excreted in the stool
2. vaccine related polio

Front 34

inactivated polio (salk)
1. advantages/disadvantages
2. timing

Back 34

1. no vaccine associated polio, no secondary (herd) immunity
2. 2,4 months boosters at 6-18 months and 4-6 years

Front 35

haemophilus influenza type B disease

Back 35

meningitis, epiglottitis, sepsis

Front 36

HITB vaccine
1. components
2. timing

Back 36

1. CONJUGATE - H-influenza polysaccharide linked to protein antigens including diptheria/tetanus toxoid
2. 2,4,6 months + booster at 12-15 months OR
2,4,12 months (if different conjugate vaccine...)

Front 37

MMR vaccine
1. diseases of MMR
2. timing

Back 37

1. measels - pneumonia/koplic spots + encephalitis
mump s - parotitis, meningoencephalitis, orchitis
rubella - severe birth defects in infants born to effected mothers infected during pregnancy, mild viral syndrome in children
2. 12-15 months with booster at 4-6 years or 11-12 years

Front 38

varicella vaccine
1. timing

Back 38

1. 12-18 months

Front 39

1. hepatitis A disease
2. high risk grouups

Back 39

1. MCC viral hepatitis on earth (up to 70% asymptomatic in children, more severe in older children/adults, RARELY fulminant hepatitis
2. homosexuals/bisexula, illicit drugs, clotting factor disorders reciving blood products, occupational

Front 40

hep A vaccine
1. timing

Back 40

1. 2 years or older with booster 6 months later for susceptible groups (high hep A communities and travelers to endemic areas)

Front 41

pneumococcal disease

Back 41

MCC otitis media, invasive bacterial infections, pneumonia in children younger than 3 years of age

Front 42

Pneumovax
1. pneumovax components
2. disadvantage
3. indications in children

Back 42

1. polysaccharide capsule of 23 serotypes covering ALL bacterimia/meningitis causing strains
2. little immunogenicity in children younger than 2 years old
3.older children/adults who are anatomical or functionally asplenic (Sickle cell), immunodeficiency, liver disease, nephrotic syndrome

Front 43

Prevnar
1. prevnar components
2. disadvantage
3. timing

Back 43

1. conjugated polysaccharide contains 7 pneumococcal serotypes
2. covers less of a broad spectrum of pneumococcal strains compared to pneumovax
3. 2,4,6 months with booster at 12-15 months
children
children > 2yo with high risk for pneumococcal disease

Front 44

side effects of
1. most vaccines
2. MMR
3. sabin

Back 44

1. very mild, local inflamm/low grade fever
2. fever and rash 1-2 weeks after immunization (live virus that incubates)
3. vaccine related polio

Front 45

contraindications to vaccines (5)

Back 45

anaphylaxis to vaccine/constituents (eggs etc)
encephalopathy 7 days after DTaP
neurological disorders/epilepsy no DTaP
immunodeficient no live attenuated OPV/MMR/varicella
immunodeficient patinets in home of someone who recieved sabin (excreted in stool)

Front 46

vaccine precautions
1. general
2. DTaP
3. MMR and varicella (live)

Back 46

1. moderate to severe illness NOT MILD ILLNESS (+/- fever)****
2. DTaP - >40.5 fever within 48 hours after vaccination
shocklike state 48 hours after vaccination
seizures 3 days after vaccination
persistent crying >3 hours within 48 hours after vaccination
3. IVIG administration in the preceeding 3-11 months

Front 47

1. why is universial newborn hearing screening carried out today
2. what are the two types of newborn hearing screen tests

Back 47

1. because hearing loss in infancy is associated iwth impaired language development
2. brainstem auditory evoked response (EEG waves from infants scalp)
evoked otoacoustic emission - sound generated from cochlear hair cells detected by a microphone

Front 48

what are the components of neonatal metabolic screening

Back 48

congenital hypothyroidism
PKU
galactosemia
sickle cell anemia

Front 49

is newborn cholesterol/lipid screening always indicated, when is it indicated

Back 49

no not always indicated (cost)
it is indicated in fmily history of hypercholesterolemia (cholesterol)i, or early MI/lipidemia (lipids)
**if high cholesterol is found, fasting lipid panel should be obtained (cholesterol, triglycerides, HDL/LDL)

Front 50

iron deficiency anemia screening
1. risk factors
2. when should screening occur

Back 50

1. prematurity, low birth weight, early cows milk introduction
low iron intake, low socioeconomic status
2. 9 and 15 months of age

Front 51

1. when should children get a PPD
2.when is a PPD read after it is placed

Back 51

1. contact with suspected/confirmed TB cases
children in contact with high risk groups
suspicious radiographic findings on CXR
imigrants from endemic areas
children with HIV
living in high prevelance areas
2. 48-72 hours after it is placed

Front 52

1. risk factors for lead intoxication
2. clinical findings of acute lead intoxication
3. clinical findings of chronic lead intoxication

Back 52

1. eat lead paint (old houses), lead pipes, lead glazed pottery, lead containing folk remedies
2. anorexia, apathy, lethargy, anemia, irritability, vomiting, encephalopathy
3. asymptomatic (most common), developmental delay, learning problems, mental retardation

Front 53

when should children screened for lead

Back 53

living in old housing, family members effected by lead poisoning, children living near lead smetlters, and normal routine screening starting at 9 months

Front 54

treatment for lead poisonign

Back 54

decrease exposure, chelation thearpy (EDTA and Meso-2,3-dimercaptosuccimic acid (DMSA succimer)

Front 55

1. what does circumcision decrease the incidence of in males
2. is circumcision medically indicated?

Back 55

1. decereases penile cancer
decreases cervical cancer in female sexual partners
urinary tract infections (10x more common in uncircumcised)
2. no

Front 56

indications for circumcisions (10% of uncircumcised males
1. phimosis
2. paraphimosis
3. balinitis

Back 56

1. inability to retract the foreskin
2. retracted foreskin cannot return to original position and cuts off lymphaic flow causing edema
3. inflammation of the glans by candida/gram negative infections in infants (STDs in adults)

Front 57

circumcision complications

Back 57

bleeding
infection
poor cosmesis
phimosis
urinary retention

Front 58

contraindications to circumcision

Back 58

penile abnormalities (ex. hypospadias), prematurity, bleeding disorders

Front 59

pediatric teeth
1.when is first tooth eruption/primary teeth (all 20)
2. secondary tooth eruption

Back 59

1. 6 months (3-16 months range) usually lower central incisor, full set of 20 primary teeth by 2 years of age
2. begins with lower central incisor, between 6-8 years of age leading to a full set of 32 secondary/permanent teeth

Front 60

what are the causes of delayed tooth eruption

Back 60

(>16 monts of age)
familial
hypothyroidism/hypopituitarism
downs syndrome
ectodermal dysplasia

Front 61

what are the clinical features of ectodermal dysplasia

Back 61

conical shaped teeth
delayed dental erruption
dysmorphic facial features
alopecia
decreased sweat glands

Front 62

what is/are the causes of early dental eruption

Back 62

primary dental eruption before three months of age
familial
hyperthyroidism
precocious puberty
growth hormone excess

Front 63

when should dental hygene be initiated

Back 63

start as soon as teeth erupt
at age 2-3 should vbe able to assist in brushing their own teeth
dental floss as soon as tight space before teeth forms
fluride consuption before adolescence dec tooth decay by 50-75%

Front 64

1. what are the sources of floride
2. what is clincical pres. of excess fluoride
3. when is a child most succeptible to fluorosis
4. what children should get flouride supplementation

Back 64

1. supplementation
fluoridated water
fluoride toothpaste
2. flurosis = cosmetic white streaks/brown staining
3. between 2 and 4 years of age
4. exclusivly breast fed children > 6 mo of age
geographic locations where fluoride is <0.3 ppm

Front 65

1. natal teeth
2. neonatal teeth
3. when to initiate intervention with natal/neonatal teeth

Back 65

1. teeth at birth
2. teeth that emerge during first month of life
3. HYPERMOBILE teeth, or if they cause breast feeding difficulty or trauma to the lip or tongue

Front 66

1. what age does neonatal bottle caries occur
2. pathogenesis of neonatal bottle caries (+infectious organism)

Back 66

1. 24-30 months
2. falling asleepe wit a nipple in the mouth with any other liquid besides water --> inc. risk of caries with s. mutans OF THE MAXILLARY INCISORS/CANINES/PRIMARY FIRST MOLARS (from parents/siblings)

Front 67

what are the factors in dental trauma and tooth salvage

Back 67

extraoral time * (most improtant)
highest prognosis if avulsed tooth is stored in milk (or other liquid)
**dry tooth is useless after 30 minutes

Front 68

page 20/21 for age appropriate anticapatory guidance print copy and put in coat

Back 68

..w

Front 69

when should hepatitis A vaccine be given

Back 69

first dose between 12-24 months, second dose between 2-6 years

Front 70

when must a TORCH infection be acquired to cause microcephaly

Back 70

in the first trimester

Front 71

why does cows milk cause IDA

Back 71

less bioavailable iron in the milk
increased stool loss

Front 72

1. what intrinsic factors effect development
2. what extrinsic factors effect development

Back 72

intrinsic factors = physical characteristics, state of health, temperment
extrinsic = family member personality, economic status, depression/mental illness

Front 73

can developmental milestones be skipped

Back 73

no, one almost always follows the next

Front 74

what is developmental quotient used for and what does it indicate

Back 74

determines if childs development is delayed
DQ = (developmental age/ chronologic age) *100

DQ>85 nomral
DQ<70 abnormal
DQ 70-85 ~ close followup (gray area)

Front 75

1. when do primitive reflexes normally disappear, and what is the effect of CNS injury on the primitive reflexes

Back 75

1. normally disappear by 3-6 mo
CNS injury increases the strength and prolongs the presence of the primitve reflexes

Front 76

what is a postural reaction,
when do they develop
what is the effect of CNS injury on postural reaction

Back 76

reactions that help orient the body in space, help facilitate orientation of the body
develop from 4-9 months of age
CNS injury delays the development of postural reactions

Front 77

what is the difference between the generation of a primitive reflex vs the generation of a postural reaction

Back 77

primitive reflexes are the response of ONE SENSORY STIMULUS
postural reactions involve proprioceptive, visual, and vestibular input and cerebral/cerebellar processing

Front 78

GROSS MOTOR MILESTONES
1. turns head side to side
2. lifts head while prone
3. head lag when pulled from supine
4. roll over
5. no head lag when pulled from supine
6. pushes chest up with arms
7. sits alone
8. leads with head when pulled from supine
9. pulls to stand
10. cruises
11. walks

Back 78

1. birth
2. 2 months
3. 2 months
4. 4 months
5. 4 months
6. 4 months
7. 6 months
8. 6 months
9. 9 months
10. 9 months
11. 12 months

Front 79

1. moro reflex
2. appears/dissapears

Back 79

1. symmetric abduction of arms with trunk extension frollwed by adduction of upper extremities
2. birth/4 months

Front 80

1. hand grasp
2. appears/dissapears

Back 80

1. grab anything placed in palm
2. birth/1-3 months

Front 81

1. atonic neck reflex
2. appears/dissapears

Back 81

1. arms and legs extend on same side as head is turned, flex on opposite side
2. 2-4 weeks/6 months

Front 82

1. rooting reflex
2. appears/dissapears

Back 82

1. turning head toward smae side as stimulus on corner of the mouth
2. birth/six months

Front 83

1. head righting
2. appears/dissapears

Back 83

POSTURAL REACTION
1. keep head veritcal despite body being tilted
2. 4-6 months/persists

Front 84

1. parachute
2. appears/dissapears

Back 84

1. arms/legs outstretch when moved head first downward
2. 8-9 mo/persisits

Front 85

FINE MOTOR MILESTONES
1. keeps hands fisted
2. brings hands together to midline then to mouth
3. reaches for objects
4. rakes objects with whole hand
5. transfers objects from hand to hand
6. uses immature pincer (thumb and index
7. uses mature pincer (thumb and tip of index finger

Back 85

1. birth
2. 3-4mo
3. 4-5 mo
4. 6-7mo
5. 6-7mo
6. 9mo
7. 12mo

Front 86

what fine motor skills are learned in the second year of life

Back 86

using tools

Front 87

motor development red flags
1. persistant fisting >3mo
2. early rolling over, early pull to stand, persistant toe walking
3. spontaneous postures such as scisoring or frog leg in hypotonic infant
4. early hand dominance before 18 months of age

Back 87

1. neuromotor problems
2. spasticity
3. motor abnormalities (cerebral palsy
4. motor weakness of the opposite upper extremity

Front 88

when does left or right hand dominance normally occur

Back 88

after 18 months of age

Front 89

1. what is most common developmental delay domain
2. what is the best indicator for intellectual potential

Back 89

language
language

Front 90

language vs speech

Back 90

languagge = abiltiy to communicate with symbols
speech = vocal expression of language

Front 91

when is the optimal time for language acquisition

Back 91

first 2 years of life

Front 92

periods of speech development
1. prespeech period
2. naming period
3. work combining period

Back 92

1. 0-10 months - musical like vowels (cooing), + mixing consonants and vowels (babbeling)
2. 10-18 months - infant understands that people have names and objects have identities
3. 18-24 months - early word combinations are telegraphic ~ combinatiosn occur 6-8 months after first words

Front 93

LANGUAGE MILESTONES
1.attunes to human voices, can differentiate parents voice
2. cooing
3. babbeling
4. jargoning
5. 1-3 words mama dada
6. 20-50 words
7. two word phrases / 50% of speech inetlligbale
8. three word senstences, 75% of speech intelligible

Back 93

1. birth
2. 2-3mo - vowels(oooooooooo aaaaaaaaaa)
3. 6 mo - consonants and vowels (ba ba ba)
4. 9-12 mo babbeling with mixed consonants, inflection, cadence
5. 12 months
6. 18 mo
7. 18mo
8. 3 years

Front 94

what are the causes of language delay

Back 94

mental retardation/global delay
hearing impairment
environmental deprivation
developmental disorders (ex. autism)

Front 95

what is cognitive development

Back 95

thinking, memory, learning, problem solving

Front 96

what does discrepency between verbal and non-verbal skills on standardized intelligence tests in school age children indicate

Back 96

learning disability

Front 97

sensorimotor period of cognitive development
1. timespan
2. characteristics

Back 97

1. birth to 2 years
2. learning to manipulate --> manipulate to learn~ time of physical manipulation/inspection of objects

Front 98

COGNITIVE DEVELOPMENT
stage of functional play

Back 98

1 year, child recognizes objects and associates them with their function ~ playing with toy telophone

Front 99

COGNITIVE DEVELOPMENT
stage of imaginative play

Back 99

24-30 months - child uses blocks to build forts, or sticks as play swords

Front 100

COGNITIVE DEVELOPMENT
concrete thinking

Back 100

preschool/elementary school years - interpreting things literally

Front 101

COGNITIVE DEVELOPMENT
abstract thinking

Back 101

adolescent years - manipulating concepts and contingencies

Front 102

COGNITIVE DEVELOPMENT
object permanence

Back 102

9 montsh - people/objects exist when out of site

Front 103

COGNITIVE DEVELOPMENT
separation anxiety

Back 103

6-18 months - crys when loved one leaves the room

Front 104

COGNITIVE DEVELOPMENT
cause and effect

Back 104

9-15 months - ex. dropping toys from high chair makes them fall to floor

Front 105

COGNITIVE DEVELOPMENT
magical thinking

Back 105

presschool toddler years- child thinks objects are alive/thinking

Front 106

what does defect in BOTH verbal and non verbal (problem solving) indicate

Back 106

intellectual disability

Front 107

what does defect in language, but normal non verbal (problem solving) indicate

Back 107

hearing impairment, or communication disorder
*increased risk for learning disability*

Front 108

what does defect in non verbal (problem solving) but normal language indicate

Back 108

visual or fine motor problems that interfere with manipulative tasks
*increased risk for learning disability*

Front 109

1. define attachment
2.when does separation and individuation begin
3. when does parallel play and social play occur

Back 109

1. bonding with primary caregiver
2. 15 months of age
3. parallel play 2 years
4. social play 3 years

Front 110

1. cerebral palsy definition
2. cerebral palsy diagnosis

Back 110

1. static encephalopathies caused by injury to developing brain that primarily effects motor funciton primarily, +/- normal intelligence, inc. risk of seizures, cognitive deficits, MR, seizures, sensory/visual/auditory deficits
2. repeated neurodevelopmental exams showing inc. tone, spasticity, hypotonia, asymmetric reflexes, abnormal disappearance of reflexes/postural response emergence

Front 111

when can injury which causes cerebral palsy occur + causes

Back 111

- maternal - (multiple gestation/preterm labor)
-prenatal - (IGR, congenital malformations, congenital infections)
-perinatal - prolonged traumatic delivery, apgar <3 @ 15 minutes, <36 or >42 wks gestation
-post natal - hypoxic ischemic encephalopathy, intraventricular hemorrhage, trauma, kernicterus

Front 112

CEREBRAL PALSY
1. what is the prevalence of cerebral palsy in the general population
2. what is prevelacnce of cerebral palsy in children <1500 grams birth weight

Back 112

1. 0.2-0.5%
2. 5-15%

Front 113

CEREBRAL PALSY
1. spastic diplegia
2. . risk factors]
3. . clinical presentation

Back 113

1. weakness of lower extremitis>upper extremities/face
2. prematurity
3. early rolling over (<4 months), inc. tone, scissoring with standing or vertical suspension

Front 114

CEREBRAL PALSY
1. spastic hemiplegia
2. risk factors
3. clinical presentation

Back 114

1. unilateral spastic motor weakness
2. perinatal vasuclar insults, CNS infections, trauma
3. upper>lower, early hand preference, grasp with one side

Front 115

CEREBRAL PALSY
1. spastic quadriplegia
2. risk factors
3. clinical presentation

Back 115

1. head, neck and all four limbs
2 hypoxic ischemic encephalopathy, CNS infections, trauma, malformations
3. seizures, scoliosis, face weakness, dysphagia, GI reflux, aspiration pneumonia, speech/sensory impariments

Front 116

CEREBRAL PALSY
1. extrapyramidal cerebral palsy
2. risk factors
3. clinical presentation

Back 116

1. extrapyramidal motor system damage
2. full term infant with hypoxia/ischemia, kernicterus (damages basal ganglia)
3. hypotonia of neck/trunk, athetoid or writhing movements,posturing of head/neck/limbs, feeding/speech defecects, DROOLING (impaired ORAL motor)

Front 117

define mental retardation
define adaptive behavior

Back 117

1. subaverage general intellectual function with defecits of adaptive behavior
2. self-care, social skills, work, leisure (seen before age 18)

Front 118

degree of mental retardation classification

Back 118

mild - IQ 55-59
moderate - IQ 40-54
severe - 25-39
profound - <25

Front 119

what are the most common genetic causes of mental retardation

Back 119

downs syndrome, fragile X (#1 and 2)
Hurlers (metabolic)
tuberous sclerosis (single gene)

Front 120

what pre-natal and perinatal factors can cause mental retardation

Back 120

TORCH
fetal malnutrition
matenral drug and alcohol
perinatal hypoxia/asphyxia
placental insufficincy

Front 121

what environmental problems can cause mental retardation

Back 121

psychosocial depravation
parental mental illness

Front 122

what post natal acquired insults can cause mental retardation

Back 122

infection
head truama
near drowning

Front 123

1. define learning disability
2. what are two types of learnign disability
3. what are the causes of learning disabilities

Back 123

1. discrepancy between childs academic achievement and level expected and the level expected on the basis of age/intelligence
2. specific academic subjects (ex. reading), or processing information (ex. visual or auditory)
3. fragile X (genetic), galactosemia (metabolic), idiopathic

Front 124

1. define pervasive developmental disorder
2. autism clinical features
3. aspergers syndrome

Back 124

1. dev. disabiltiy that effects behavior/learning with varying severity
2. onset <3 years, more boys, LANGUAGE DELAY but large range of severity, atypical intonation, echolalia, decreased eye contact, restricted range of interest/play, sterotypic movment rituals, preoccupations with certain objects/symbols, self injury, seizures, MR, diarrhea/constipation, ear infections, sensitivity to sensory input, unusual pain threshods
3. NO LANGUAGE DELAY qualitative impairment in peer relationships/social interactions, repetative stereotyped patterns of behavior, activities, interests

Front 125

1. ADHD diagnostic criteria
2. ADHD risk factors
3. ADHD clinical features
4. non-pharm treatment
5. pharm first line treatment
6. pharm second line treatmnet

Back 125

1. onset before age 7, symptoms in >1 environment, impaired school/personal resationships, inattention, hyperactivity, impulsivity
2. first relative with ADHD (genetic), DA/NE function ab
3. poor selective attention, difficulty focusing, distractibility, impulsivity, distractivbility, disinhibition, behavior immaturity, low self esteem, impaired relationships, diffuclty learning
4. demystification, classroom modifications, educational assistance (1 on 1, organizational skills), counseling.
4. methylphenidate, dextroamphetiamine-amphetamine
5. clonidine (A2 agonist) - if comorbid aggression, tic, or before bedtime
TCA, SSRI, buproprion - if comorbid depression

Front 126

ddx for ADD

Back 126

hearing/vision defecits, OSA, food reactions, thyroid disease, anemia, heavy metal exposure, anemai, anxiety, depression, family dysfunction

Front 127

side effets of stimulants used to treat ADHD

Back 127

anorexia, INSOMNIA, N/V, headache, irritability, hypertension/palpatations, dec. growth velocity, tics (9%)

Front 128

what is the sequealae of of late identification of hearing problems

Back 128

DELAYED SPEECH AND LANGUAGE, academic/behavioral problems

Front 129

1. genetics of hearing loss
- other 20% is congenital infections, prematurity, meningitis
2. prognostic factors in hearing loss
3. evalulation of deafness etiology

Back 129

1. 80% of all hearing loss, AUTOSOMAL RECESSIVE
2. degree of loss, inherited>acquired, older age>younger, early amplification, cochlear implants
3. H+P, GENETICS, creatinine (alport), viral serologies (CMV, toxo, rubella, herpes)

Front 130

1. top causes of blindness in children
- delays locomotion, dec. fine motor skills, dec. attachment
2. adaptive skills in blindness

Back 130

1. trachoma infection, retinopathy of prematurity, congenital cataracts
2. auditory perception skills, haptic perception

Front 131

1. clincial presentation of colic
2. what fractions of newborns experience colic
3. treatment

Back 131

1. crying >3 hours/day, for more than 3 days a week typically in evening at age 2-4 weeks resolves by 3-4 months, unknown cause in healthy well fed infants,
2. 10 % of newborns
3. reassure the parents that it is not their fault, decreased senory stimulation, increased sensory stimulation, positioning

Front 132

***ENURESIS IS NORMAL UNTIL AGE 5
1. primary vs secondary enuresis
2. incidence of enuresis in 4 vs 5 vs 6 vs 12 year olds
3. causes of enuresis

Back 132

1. child has never been dry vs 6 months of prior dryness
2. 30%, 20%, 10%, 3%
3. gene on chromosome 13, stress, death of family member, chaos at home (diurnal), deep sleep with difficult arousal, lack of diurnal vasopressin , bladder capacity, UTI, abuse, diabetes (polyuria/polydipsia)

Front 133

comorbidity for enuresis

Back 133

encorpresis
hard stool

Front 134

treatment of nocturnal enuresis

Back 134

demystification+removal of blame
conditional alarming- alarm goes off when urine is produced
behavioral modification - reward systems, hypnotherapy, treat constipation
DDAVP - but common relapse after stopping tx
imipramine (TCA)

Front 135

which antidepressant is used for enuresis, and what is the major side effect

Back 135

imipramine
fatal cardiac arrythmias with overdose

Front 136

treatment of diurnal enuresis

Back 136

bladder stretching exercisies
scheduled voids (every 60-90mins)

Front 137

how does sleep pattern change after birth

Back 137

first 4 weeks random with day/night reversal
50% of infants sleep thorugh the night at 3 months of age (>5 hours after midnight

Front 138

what is trained night waking
what is trained night feeding

Back 138

1. 4-8 months- infant does not resettle without parental intervention
2. infant wakes to eat because parents respond with feeding- tx= inc. interval of daytime feeding

Front 139

1. at what age, and what phase of sleep does nightmares occur
2. diagnosis and treatment

Back 139

1. >3 years, REM
2.detailed recall of nightmare, reassurance and remove inciting cause (movies etc)

Front 140

1. at what age, and what phase of sleep does night terrors occur
2. diagnosis and treatment

Back 140

1. age 3-5, 90-120 minutes after onset of sleep in non-REM phase
2. child awakes tachycardic, diaphroetic, tachypneic, child stares "glass eyed" without seeing, child CANNOT remember in the morning
tx = reassurance

Front 141

1. why do parents overfeed children
2. why do toddlers have decreased feeding/food resistance after 1 year of age
3. treatment of food resistance

Back 141

1. parents think its a sign of love
2. normal decrease, control/autonomy
3. avoid bribes, pressuring, or forcing (avoiding power struggles)_

Front 142

social phobia - child fears leaving home/caregiver
1. clinical presentation
2. tx

Back 142

1. child who misses school b/c of abdominal pain/diarrhea/fatigue that occur in the morning on departure for school, and disappear on weekends/summer
2. hx/px, return child to school, if still wants to stay home visit physician

Front 143

temper tantrums - expression of anger beyone childs control
1. age of onset
2. cause
3. tx

Back 143

1. 1-3 years
2. frustration/fatigue more likely with coexisting fine motor/expressive language delays
3. ignore demands, wait until age 3 when emotion can be verbalized, holding by parent to help calm

Front 144

breath holding spells
1. definition
2. age of onset
3. two subtypes
4. tx

Back 144

1. HARMLESS INVOLUNTARY episodes in which child holds their breath long enough to alarm parent
2. 5% of children between age 6 and 18 months , disappears by 5 years of age
3. cyanotic: frustration/anger-->cyanosis-->apnea/seizure (peaks @ 2 years of age)
pallid: fright-->hypervasovagal response--> pale and limp
4. reassure parents, tell parents NOT to do resusitation, iron supplementation

Front 145

managment of breath holding spell precipitated by exercise/excitement

Back 145

this may NOT BE A BREATH HOLDING SPELL, COULD BE SVT OR LONG QT SYNDROME -- MUST DO EKG

Front 146

sibling rivalry
1. cause
2. tx

Back 146

1. arrival of newborn is very stressful for child <3 yo, responds with jealosy
2. praise older child for mature behavior, do not teach toilet training/other skills at this time, encourage mature behavior and mutual respect between older children

Front 147

toilet training
1. average and range of age of bowel control
2. "" bladder
3. how to toilet train

Back 147

1. 29 month average (16-48)
2. 32 month average (18-60)
3. social reinforcement, sometimes treats, NO PRESSURE OR FORCE = POWER STRUGGLE

Front 148

disciplining child
1. when should child begin showing self control
2. before age 6 months
3. 18-3 years
4. preschool
5. >5 years
*make punishment brief, followed with love/trust, direct toward BEHAVIOR not PERSON

Back 148

1. between ages 3 and 4 (parent should begin providnig external control at 6 months)
2. none, distraction/redirection
3. ignoring, time out, disapproval
4. logical consequences - lose toy being used to hurt another child
5. negotiation/restriction of privileges

Front 149

why give a misbehaving child time out, how long

Back 149

interupt misbehavior to give child time to think about behavior
1 minute/year of age to a maximum of 5 minutes

Front 150

milestones
1. walk
2. hand preference
3. mama/dada + 1 or 2 more words
4. stranger anxiety
5. 20-30 words understood, 12-15 spoken
6. telegraphic two word sentences
7. adjectives/adverbs, questions, >2 word sentences
8. symbolic play (fort out of blocks)
9. cause and effect
10. object perminence
11. separation anxiety
12. immature pincer grip
13. parachute reaction
14. mixing vowels/consonants (babbel)

Back 150

1. 12 mo (R=9-15mo)
2. >18mo
3. 12 months
4. 6-18mo
5. 15 months
6. 24 months
7. 30 months
8. 24-30 months
9. 9 months
10. 9 monts
11. 6-18 months
.12. 9 months.
13. 8 months
14. 6 months

Front 151

1. when in maturation does growth spurt occur
2. what hormones control this process
3. what fraction of adult weight/height is gained in adolescence

Back 151

1. adolescence
2. GROWTH HORMONE, also insulin/thyroid/sex contribute
3. 50% of ideal body weight, 25% of adult height

Front 152

1. what two endocrine changes occur during puberty
2. what is adrenarche
how long is puberty
3. what is true puberty

Back 152

1. adrenarche and true puberty
2. onset of ADRENAL androgen synthesis, occurs 2 years before HPG axis turns on
3. LH and FSH and sex steroids increase (HPG axis is now on)

Front 153

how long is puberty

Back 153

3-4 years

Front 154

1. timing of growth spurt in males vs females
2. timing of onset of puberty in males vs females

Back 154

1. 18-24 months earlier in females
2. 6-12 months earlier (9.5 in females)

Front 155

male physical changes in puberty

Back 155

testicular enlargemen/pubic hairt --> 2 years later facial/axillary hair grows

Front 156

female puberty
1. onset of puberty age + physical finding
2. onset of menstruation age

Back 156

1. THELARCHE avg 9.5 years, development of breast buds,
2. MENARCHE - 12.5 (2-3 years after thelarche)

Front 157

male tanner staging

Back 157

1. preadolescnet, no pubic hair + pre-pubertal testes
2. testes larger, LONG downy hair
3. testes larger darker, coarser curlier hair
4. glans develops, scrotal skin darkens, coarse curly hair over pubis symphysis
5. adult size/shape, pubic hair spreads to medial surface of thigh

Front 158

FSH
1. effect in males
2. effect in females

Back 158

1. inc. spermatogenesis in the seminiferous tubules
2. stimulates ovarian follicles, stimulates ovarian granlosa cells to produce estrogen

Front 159

LH
1. effect in males
2. effect in females

Back 159

1. causes leydig to produce testosterone
2. stimulates theca cells to produce androgens
stimulates corpus luteum to make progesterone, ovulatory surge

Front 160

testosterone
1. effect in males
2. effect in females`

Back 160

1. linear growth/muscle mass, penis, scrotoum, prostate, seminal vesicle development, pubic/axillary/facial hair, deepens voice, inc. libido
2. stimulates linear growth, stimulates growth of pubic/axillary hair

Front 161

estradiol
1. effect in males
2. effect in females

Back 161

1. increases rate of epiphyseal plate fusion
2. breast dev, midcycle luteinizing hormone, labial/vaginal/uterine dev, proliferation of endometrium,
*low level = linear growth
*high level = inc. epiphyseal plate fusion

Front 162

progesterone
1. effect in males
2. effect in females

Back 162

1. NONE
2. converts endometrium to secretory endometrium

Front 163

adrenal androgens
1. effect in males
2. effect in females

Back 163

1. growth of pubic hair, linear growth
2. growth of pubic hair, linear growth

Front 164

breast tanner stages

Back 164

1. preadolescent
2. elevation of breast/nipple as small projection
3. enlargement of breast
4. areaola and nipple form secondary mound above breast level
5. only nipple projects, areola recedes, adult breast size

Front 165

female genetalia tanner stages

Back 165

1. preadolescnet
2. sparse long downy hair along labia
3. darker coarser curlier
4. coarse and curly adult type hair covering symphysis pubis
5. adult type hair spread onto medial surface of thighs

Front 166

early adolescence psychosocial development
1. age
2. features

Back 166

1. 10-13 years of age
2. early shift to independence from parents, pre-occupation with pubertal body changes, same sex peer relationships, abstract thinking

Front 167

middle adolescnece psychosocial developmetn
1. age
2. features

Back 167

1. 14-17 years
2. inc. conflicts with parents, dec. preoccupation with pubertal changes, inc. desire to improve attractiveness, intense peer group involvement, initiation of romantic relationships, inc. abstract reasoning/risk taking

Front 168

late adolescnece psychosocial development
1. age
2. features

Back 168

1. 18-21
2. dev. of self as distinct from parents, more likely to seek advice from parents, comfort with body image,shared intimate relationships, abstract thought processes, abstract thought, fewer risk taking behaviros

Front 169

how best to establish rapport with an adolescen

Back 169

begin history with non-threatening hobbies, interest or activities

Front 170

what information gained from the history of an adolescent (or any patient) must be reported

Back 170

sexual/physical abuse, suicidal/homicidal intention

Front 171

what condiditons can patients under age of 18 consult for without a parent

Back 171

pregnancy related care, dx/tx of STDs, reproductive health care, counseling and treament of drug/alcochol problems, mental health treatment

Front 172

acronymn for the psychosocial hisotry

Back 172

HEADSS
home
education/employment
activities
drugs
sexual activity
suicide/depression

Front 173

what vaccines for the adolescent

Back 173

Td booster@ 11 or 12 years of age, and every 10 years after this
Varicella vaccine if havent had chicken pox
If not given earlier: give MMR and hep B series

Front 174

what special things should be looked for in the adolescent physical exam

Back 174

height/weight
BP, vision, and hearing
acne/fungal infections of the skin
oral hygene
enlargement/nodules of the thyroid
scoliosis/kyophosis
tanner rating
male genetalia for hernias/scrotal masses
female complete pelvic exam should be performed annually if patient is sexually active, or if hx of pelvic pain, discharge of abnormal bleeding

Front 175

what self exam instructions should be given to adolescent male/female

Back 175

male = testicular exam
female = breast exam

Front 176

what labs for adolescent screening (non sex related)

Back 176

hemoglobin/hematocrit
urinalysis - for proteinuria/hematuria
cholesterol/fasting lipid
HIV if indicated
PPD - once in adolescent

Front 177

what labs for adolescent screening (sex related)
1. females
2. males

Back 177

1. sexually active females- gonorrhoeae, chlamydia, syphillus, Pap smea, wet mount for trichomonas vaginalis
2. sexually active males - syphillus serology, urinalysis for pyuria, urine ligase for c.trochomatis

Front 178

1. what fraction of adolescents are clinically depressed
2. which gender has more depression

Back 178

5%
2. girls 2x>boys

Front 179

1. behavioral signs of depression
2. physical """

Back 179

1. missing school, change in school performance, acting out, lack of interest in activities
2. abdominal pain, headaches, weight loss, overeating, insomnia, anxiety, fatigue

Front 180

what are the top three causes of adolescent death

Back 180

1. unintentional injuries
2. homicide
3. suicide

Front 181

dx criteria for major depression

Back 181

5/9 almost every day for 2 weeks
depressed/irritable mood,
diminished interest or pleasure in activivities
weight gain/loss
insomnia/hypersomnia
psychomotor agitation/retardation
fatigue/energy loss
feelings of worthlessness
dec. ability to concnetrate
recurrent thoughts of death or suicide

Front 182

dysthymia clinical features

Back 182

>1 year
2/5
poor appetite or overeating
insomnia or hypersomnia
decreased energy
difficulty concentrating
feelings of hopelessness

Front 183

what fraction of high school senioirs have:
1. tried alcohol
2. tried illegal drugs
3. tried cigarettes

Back 183

1. 90%
2. 50%
3. 60%

Front 184

what are the changes that indicate substance abuse in an adolescent

Back 184

mood/sleep disturbance, dec. school performance, changes in family/friends diminished appetite, depression

Front 185

what defines a drinking problem

Back 185

intoxicated >6 times a year or having drinking assocated problems with school/friends/family

Front 186

1. define alcoholism
2. how to screen for alcoholism

Back 186

1. preoccupation with over drinking despite adverse consequences
2. CAGE questionaire- have you tried to cut down, have people annoyed you by criticizing your drinking, do you feel guilty, hhave you ever drank first thing in the morning (eye opener)

Front 187

what are the health risks of smoking

Back 187

coronary disease/stroke
cancers - lung/mouth/esophagus/stomach/larynx/urinary tract
lung dz - COPD/asthma
peptic ulcer disease
pregnancy complications - stillbirth, low birth weight, infant mortality

Front 188

physical effects of marijuana acute and long term

Back 188

tachycardia, mydriasis, sleepiness, conjunctival injection, dry mouth, auditory/visual hallucinations

asthma, impaired memory/learning, diminished interpersonal interactions, depression

Front 189

clinical definition of obesity

Back 189

body weight >20% IBW
BMI > 95% for age/sex

Front 190

causes of obesity

Back 190

95% - genetics + inc. caloric intake, dec. energy expenditure, poor eating behaviors
5% = cushings, hypogonadism, hypothyroidism, prader willi

Front 191

what are the health effects of obesity

Back 191

1. early puberty
2. hypertension/heart disease
3. hypercholesterolemia/inc. triglycerides
4. type 2 diabetes
5. gallbladder disease
6. back pain/tibia vara
7 depression/poor body image

Front 192

epidemiology of anorexia/bulemia

Back 192

1% of adolescents have anorexia,
1-5% bulemia

Front 193

anorexia nervosa
1. diagnostic criteria
2. physical exam findings
3. lab findings

Back 193

1. body weight 15% BELOW NORMAL, ABSENCE OF 3 OR MORE CONSECUTIVE MENSTURAL CYCLES
too low caloric intake to maintain weight, delusion of being fat, obsessed with being thin, refusal to maintain body weight, fear of weight gain, excessive exercise, withdrawal from peers/family, disturbed body image
2. hypothermia, hypotension, bradycardia, malnourishment, hypoactive bowel sounds, lanugo, dehydration
3. LOW MG, LOW PHOSPHORUS, LOW CALCIUM, HIGH BUN, high LFTs anemia, leukopenia, low sex steroids, low thyroxine

Front 194

bulemia nervosa
1. diagnostic criteria
2. physical exam findings

Back 194

1. recurrent binging, >2x in the past 3 months, purging using vomiting, laxatives, diuretics, enemas, rigorous fasting exercise or diet pills, disturbed body image
2. NORMAL WEIGHT, hypothermia, hypotension, bradycardia, sequelae of vomitting, trauma to palate/hands, loss of dental enamel, parotid swelling, swollen uvula.
3. LOW CHLORIDE, LOW POTASSIUM (vomitting or diurects or laxatives)

Front 195

2. treatment for anorexia/bulimia nervosa

Back 195

normal nutrition
hospitalization- for severe weight loss, dehydration, eleectrolyte abnormalaties, dehydration, abnormal vitals, seizures, arrythmias, pancreatitis

Front 196

what fraction of females are sexually active by end of high school, and what fraction of adolescent females become pregnant

Back 196

50% sexually active
1/9 adolescents become sexually active

Front 197

what are the complications of high risk pregnancy for mother/infant

Back 197

low birth weight (infant)
higher infant mortality (infant)
preterm labor/anemia/hypertension (mother)

Front 198

why do 1/2 of sexually active adolescents not use contraception

Back 198

ignorance
denial - of preg. risk
confidentiality/cost
refusal by partner to use
religeon
desire for pregnancy

Front 199

what kinds of condoms protect from HIV, and what are the disadvantages to condoms

Back 199

latex ONLY
interference with spontaneity of intercourse and allergic reactions

Front 200

what are the disadvantages to polyurethane female condom

Back 200

vaginal irritation, allergy or awkwardness

Front 201

vaginal diaphragm
1. mechanism
2. adv
3. disadv

Back 201

mechanical barrier placed cervix, used with spermacide
2. can be put in six hours before sex (doesn't impede spontaneity)
3. INDIVIDUAL FITTING health care professional is neccessary, awkward to place, inc risk of UTI

Front 202

cervical cap
1. mechanism
2. adv
3. disadv

Back 202

1. cuplike diaphragm placed over cervix,
2. can be left in place for 48 hours
3. need for individual fitting, inc. risk for UTIs, CERVICAL DYSPLASIA (must follow up with Pap smear)

Front 203

IUD
1. mechanism
2. adv
3. disadv

Back 203

1. copper = impedes sperm transport
progesterone - induces endometrial atrophy
2. convenience/privacy
3. NO STD PROTECTION, higher initial cost, inc. RISK OF PID

Front 204

oral contraceptives
1. mechanism
2. adv
3. disadv
4. contraindications**

Back 204

1. estrogen/progesterone combination or progesterone- inhibits ovulation (E/P), thickens cervical mucus(P)
2. dec. dysmenorrhea, protects against endometrial/ovarian cancer, improved acne, spontaneity
3. headache, weight gain, amenorrhea, BTB, mood changes, no STD protection
4. pregnancy, breast/endometrial cacner, STROKE, CAD, LIVER DISEASE, smoking, hypertension, migraines, diabetes, SSD, elevated triglycerides,

Front 205

contraceptive injections
1. mechanism

Back 205

1. depomedroxyprogesterone (depo provera) slow release progestin given 1x every 3 months by intramuscular injection
2. 3 months protection
3. need for injection, BTB, weight gain, no STD protection

Front 206

which 3 contraceptives have the lowest failure rate

Back 206

depo provera
IUD
OCP

Front 207

three most common STDs in the united states

Back 207

HSV
HPV
c. trochomatis
**HAVING 1 STD INC. LIKELYHOOD OF HAVING ANOTHER STD

Front 208

what is cervical ectopy and what STD does this inc. the risk for

Back 208

seen in adolescent females cervical columnar epithelium outside the external os to which C.trachomatis and N.gonorrhoeae attach

Front 209

vaginitis
1. organisms
2. clinical findings
3. dx.
4. tx

Back 209

1. trichomonas vaginals (STD), or bacterial or candidia(non-STD)
2. 50% asymptomatic,
symptomatic odiferous yellow green discharge, petechiae covered "strawberry cervix", VULVAR INFLAMMATION/itching, dyspareunia
3. wet mount slide, T. vaginalis culture, vaginal pH <4.5
4. metranidazole for effected AND partners

Front 210

what is the side effect of metranidazole

Back 210

disulfiram like reaction if alcohol is consumed

Front 211

bacterial vaginosis (non-STD)
1. pathogenesis
2. clinical findings of bacterial vaginosis
3. dx
4. tx

Back 211

1. dec. lactobaccili --> inc. GARDNRELLA VAGINALLIS, MYCOPLASMA HOMINIS, anaerobic gram negative rods
2. gray white vaginal discharge, FISHY odor, LESS**vaginal/vulvar inflammation
3. fish smelling whiff test w/ 10% KOH, CLUE CELLS, thin gray vaginal discharge, vaginal pH <4.5%.
4. metronidazole or topical clinda or topical metranidazole *NO TREATMENT FOR PARTNERS****

Front 212

Candidal vulvovaginitis
1. clinical presentation
2. dx
3 tx

Back 212

1. SEVERE ITCHING with white CURDLIKE discharge, vulvar/vaginal infalmation
2. fungal hyphae on wet mount, NORMALVAGINAL PH, positive yeast cultures
3. oral fluconazole, topical intravaginal anti-yeast therapies NO TREATMENT FOR PARTNERS

Front 213

chlamydia trochomatis cervicitis
1. pathogesnsis
2. clinical presentation
3. diagnosis
4. complications
5. tx

Back 213

1. c.trachomatis trasmitted as an STD intracellular pathogen of the endocervix
2. 50-75% asymptomatic--- purulant endocervical discharge, edematous erythematous cervix, dysuria/freq,
3. endocervical culture, rapid antigen detection by flurescent antibody or ELISA, PCR
4. PID, tuboovarian abscess, ECTOPIC PREG,
5. doxy, erythro, azithro + TREAT PARTNER

Front 214

n. gonorrhoeae cervicitis
1. pathogenessis
2. clinical presentations
3. dx
4. compliations
5. treatment

Back 214

1. STD intracellular gram negative diplococcus of the endocervix
2. often asymptomatic ---MUCOPURULENT cervical discharge +/- vaginal bleeding, dysuria/freq, dysparunia,
3. culture on THAYER MARTIN MEDIA shows intracellular gram negative diplococci, PCR, nucelic acid hybridization
4. PID, TOA, fitz hugh curtis syndrome, infertility, disseminiation --> asymmetric polyarthritis, papular/pustular skin lesions, meningitis, endocarditis, epsis
5. cetriaxone or ofloxacin, cefixime, ciprofloxacin + assume c.trochomatis co-infection + TREAT PARTNERS

Front 215

PID
1. pathogensis
2. when in the menstrual cycle is PID most common
3. clinical presentation
4. tx

Back 215

1. c. trochomatis, and n.gonorrhoeae ascending infection that spreads to uterus/fallopian tubes
2. first half (less cirvical mucus)
3 .lower abdominal pain, cervical motion tenderness, adnexal tenderness + fever, leuckocytosis >10k, elevated ESR/CRP, pos. culture
4. in patient - IV cefoxitin + oral doxy or IV clinda + gentamycin
outpatient - 14 day oflaxacin, clindamycin, or single dose IM ceftriaxone + 14 days doxy

Front 216

urethritis
1. pathogensis
2. clinical presentation
3. tx

Back 216

1. gonococcal or non gonoccoccal (u.urealyticum, mycoplasma genitalium, HSV, t. vaginalis, chlamydia)
2. often asymptomatic dysuria/freuq, mucopurulent discharge
3. >5 wbc per high power field on gram stain, >10wbc on first void urine, + LEUKOCYTE ESTERASE, urethral swab + culture
4. ceftriaxone + doxy (same as cervicitis)

Front 217

geneital warts
1. pathogenesis
2. clinical presentation.
3. dx
4. tx

Back 217

1. sexually transmitted 6/11 cause warts 16/18 cause cervical cancer /warts
2. itching, pain, dyspareunia, visible on external genetalia, often asymptomatic
3. pap smear (koilocytes/dysplasia/cervical cancer), 3% acetic acid wash during colposcopy (turns white)
4. cryo, laser, podophyllin, trichloroacetate

Front 218

phases of menstural cycle

Back 218

follicular proliferative - pulsatile GnRH =-->FSH-> matruatio of ovarian foliceles -> inc. estradiol->endometrial prolifeation
ovulatory phase- inc estradiol causes LH surge ->follicle rupture-> formation of corpus luteum
luteal secretory phase - progesterone from teh corpus luteum generates secretory endometrium
menstrual phase- without fertilization corpus luteum involutes, estrogen/progesterone declines,endometrium sloughs, and GnRH is released from hypohalamus again

Front 219

how long after menarche are menstrual cycles irregular

Back 219

1-2 years

Front 220

primary dysmenorrhea
1. pathogensis
2. clinical presentation
3. tx

Back 220

1. increased production of prostaglandings by endometrium --> excessive contractions + systemic effects ~ pain with menstrual flow
2. spasms, N/V, diarrhea, headache, fatigue
3. prostaglandin inhibitors (NSAIDs), OCP

Front 221

secondary dysmenorrhea
1. pathogensis
2. clinical presentation
3. tx

Back 221

1. pain with menstural flow secondary to endometriosis, PID, uterine fibroids, bicornate uteurs
2. same as primary
3. same as primary + treat underlying cause

Front 222

amenorrhea
1. primary amenorrhea
2. secondary amenorrhea
3. dx***
4. what does high vs low FSH+LH indicate

Back 222

1. no menstrual bleeding by age 16 with normal secondary sex characteristics, or age 14 with no secondary sex characteristics
2. absence of menses for three menstrual cycles or 6 months after regular menstrual cycles have occurred
3. PREGNANCY TEST FIRST***, TSH/T3, fasting prolactin**, FSH/LH
4. high indicates ovarian failure, low indicates hypothalmic/pituitary suppression

Front 223

amenorrhea (puberty onset, genetalia, FSH/LH)
1. turners syndrome (XO)
2. hypothalamic or pituitary failure before puberty
3. testicular femininization syndrome (46XY)
4. mayer rokitansky kuster hauser
5. PCOS
6. sheehans syndrome

Back 223

1. gonadal dysgenesis/ovarian failure, delayed onset puberty, normal genetalia, high FSH/LH
2. delayed onset puberty, normal genetalia, low FSH/LH
3. X-linked androgen receptor defect (androgen insensitivity) - normal female puberty, absent uterus/blind pouch vagina/ inguinal testes, **BILATERAL INGUINAL HERNIAS
elevated FSH/LH
4. congenital absense of vagina/uterus , normal pubertal development, NORMAL FSH/LH
5. normal puberty, normal genetalia, high LH:FSH raatio + obesity hirsuitism, acne
6. noraml puberty, normal genetalia, low FSH/LH

Front 224

why does prolactinoma cause amenorrhea

Back 224

prolactin inhibits HPG axis and dec GnRH

Front 225

1. dysfunctional uterine bleeding
2. polymenorrhea
3. menorrhagia
4. metorrhagia
5. menometorrhagia
6. oligomenorrhea

Back 225

1. frequent irregular menstrual periods, prolonged painless bleeding
2. regular intervals <21 days
3. prolonged or excessive uterine bleeding that occurs at regular intervals
4. uterine bleeding that occurs at irregular intervals
5. prologned excessive bleeding that occurs at irregular intervals
6. uterine bleeding that occurs at regular intervals >35 days

Front 226

DUB
1. pathogenesis
2. what other conditions can cause DUB
3. laboratory testing
4. treatment

Back 226

1. ANOVULATORY cycles--> excessivly thickened endometrium-->spontaneous prolonged uterine bleeding with weak uterine contractions
2. ectopic pregnancy, PID/cervicitis, vWD, leiomyoma/endometriosis, salicylates, OCP, steroids, IUDs, retained condoms/ tampons, trauma
3. CBC, pregnancy test, test for chlamydia, gonorhoea, bleeding time,
4. OCP/progestin only contraceptis for anemia, iron supplements, dilation and curretage

Front 227

1. is gynecomastia common in adolescnet males common?
2. ddx
3. tx

Back 227

1.yes very common, 60% of adolescent males
2. medications, testicular tumors, thryoid dz, liver dz
3. reassurance - usually resolves in 12-15 months

Front 228

testicular torsion
1. clinical presentation
2. dx
3. tx

Back 228

1. sudden onset scrotal, inguinal pain, swollen testicle, ABSENT CREMASTERIC reflex on the affected side, pain relief on elevation
2. hx/px, technetium 99m pertechnetate radionucleotide scan or doppler ultrasound (shows absent pulsations)
3. surgical detorsion WITHIN 5 HOURS

Front 229

testicular appendage torsion
1. clinical presentation
2. dx/tx

Back 229

1. acute or gradual onset of testicular pain and tenderness most noticable in the upper testicle, BLUE DOT SIGN
2. doppler ultrasound/radionucleotide scan is NORMAL rest and analgesia, resolves on its own

Front 230

testicular neoplasms
1. clinical presntation + cell type
2. dx

Back 230

1. germ cell tumors that presnt as firm, irregular painless nodules in the testicle
2. doppler ultrasound, serum tumor markers (AFP, B-HCG), find mets

Front 231

indirect inguinal hernia
1. pathogenesis
2. clinical presentation
3. treatment

Back 231

1. processus vaginalis fails to obliterate-->bowel extends through internal inguinal ring-
2. painless inguinal swelling + bowel sounds on auscuiltation of the scrotum
3. surgical repair, to prevent bowel incarceration

Front 232

hydrocele
1. pathogenesis
2. clinical presentation
3. tx

Back 232

1. collection of fluid in the tunica vaginalis
2. transillumination of scrotum reveals soft painless cystic mass
3. reassurance, or surgery if very large/painful

Front 233

varicocoele
1. pathogenesis
2. clnical presentation
3. tx

Back 233

1. tortuosity fo the vein sin the pampiniform plexus
2. bag of worms in the left half of the scrotum that increases with standing/valsalva
3. reassurance, if varicocoele is painful/distended or associated with small testicle refer to urology

Front 234

epidydymitis
1. pathogenesis
2. clinical presentation
3. dx
4. tx

Back 234

1. inflmamtion of the epididymis secondary to gonorrhea or chlamydia****
2. acut eonset scrotal pain/swelling with pain/tender epididymis
3. inc. wbc on urinalysis, positive urine culture, doppler ultrasound shows INCREASED FLOW, and INCREASED RADIONUCLEOTIDE SCAN
4. same as cervicitis

Front 235

how does puberty begin in males vs females

Back 235

males begins with testicular enlargment (next is pubic hair, facial and axilary hair is 18-24 months after pubic hair)

females begins with breast enalrgerment (next is pubic hair, menarche is 2-3 years after thelarche)

Front 236

lanugo

Back 236

thin hair that covers the skin of PRETERM infants

Front 237

what is a 2 on apgar scoring system

Back 237

>100bpm, good respirations + crying, active motion, coughing, sneezing, crying, completely pink color

Front 238

vernix caseosa

Back 238

thick white creamy material found in term/preterm infants, absent in post-term infants

Front 239

acrocyanosis
cutis marmorata

Back 239

cyanosis of the hands and feet frequent in first 48-72 hours,

motteling of the skin with venous prominence signs of vasomotor instability characteristic in some infants

Front 240

pallor indicates what conditions in a neonate

Back 240

sign of neonatal asphyxia, shock, sepsis, anemia

Front 241

when is jaundice pathological in a neonate, when is it not pathological

Back 241

abnormal if detected in first 24 hours
not assc. with serious disease if if seen during first few days after birth

Front 242

milia

Back 242

small cysts around pilosebaceious follicles seen on the face, and gingivae dont need to treat them

Front 243

mongolian spots

Back 243

dark blue hyperpigmented macules over the bosacral area and buttocks that are not pathological-- most commonly in hispanic, asian, and african american infants

Front 244

pustular melanosis

Back 244

transient benign rash -- small dry superficial vesicles over a dark macular base, leave a hyperpigmented region when they rupture -- more common in african american infants

Front 245

erythema toxicum neonatorum

Back 245

benign rash seen 72 hours after birth flea bite macules/pustules with surrounding erythema filled with EOSINOPHILS, 50% of preterm infants LESS FREQUENT IN PRETERM INFANTS

Front 246

nevus simplex

Back 246

MOST COMMON VASCULAR LESION OF INFANCY aka salmon patch aka telangiectatic nevus, PINK PATCH on nape of the neck (stork bite), upper eyelids, glabella, nasolabial region, TRANSIENT BENIGN, NUCHAL/OCCIPITAL salmon patches persist for life
**more prominent during crying

Front 247

nevus flammeus

Back 247

dilated capillary like vessels becomes darker with age, on face or trunk, if indistribution of V1 ~ assc STURGE WEBER - intracranial vascular malformations/calcifications

Front 248

strawberry hemangioma

Back 248

benign proliferative vascular tumors in 10% of infants, increased in size after birth and resolve by 18-24 months. ***NOTE MAY COMPOROMISE AIRWAY/VISION

Front 249

neonatal acne

Back 249

20% of newborns, week 1-2 of life, comedones + pustules/papules, no tx
~ comes from maternal hormone transmission

Front 250

microcephaly

Back 250

head circumference below 10th percentile ~ multiple etiologies - torch, malformations, chromosomal etc

Front 251

caput seccedaneum

Back 251

diffuse edema or swellign of scalp CROSSES THE SUTURES, USUALLY MIDLINE

Front 252

cephalohematoma

Back 252

subperiosteal hemorrhages, do not cross suture lines secondary to birth trauma confined to cranial sutures involving parietal/occipital bones

Front 253

craniotabes

Back 253

sort areas of the skull wiht ping pong ball feel, not related to rickets

Front 254

cause of abnormal red reflex

Back 254

cataracts, retinoblastoma, severe chorioretinitis

Front 255

choanal atresia

Back 255

occluded nasal passages, this is a problem in neonates because of obligate nose breathing-- clinical presentation = child that becomes cyanotic during feeding that improves with crying tx= PASS NG TUBE

Front 256

pierre robin syndrome

Back 256

micrognathia, cleft palate, glossoptosis

Front 257

syndromes associated with macroglossia

Back 257

beckwith wiedeman, hypothyroidism, mucopolysaccharidosis

Front 258

epstein pearls

Back 258

small white epidermoid mucoid cysts of the hard palate, usually disappear in a few weeks

Front 259

what cysts are seen on the lateral neck

Back 259

branchial cleft cysts
cystic hygromas

Front 260

what are the midline clefts/masses

Back 260

thyroglossal duct cyst
goiter (2' to maternal antithyroid medication, omphalocele, cleft lip/palate

Front 261

neonatal torticollis pathogensis

Back 261

asymmetric shortening of sternocleidomastoid - may be fixed in utero, postnatal hematoma, birth injury

Front 262

edema and webbing of the neck

Back 262

turner syndrome

Front 263

what risk factor increases clavicle fractures in neonates

Back 263

large birth weight

Front 264

accessory nipples

Back 264

present along anterior axillary/midclavicular lines, can later grow b.c they have glandular tissue

Front 265

poland syndrome

Back 265

chest asymmetrey from agenesis of ribs/pectoralis muscle (very serious)

Front 266

respiratory distress

Back 266

tachypnea (>60 breaths/min), deep respirations, cyanosis, expiratory grunting, sternal retractions,

Front 267

periodic breathing

Back 267

seen in PRETERM infants - apneic bursts that last less than 3-10 seconds- no significance

Front 268

cardiac exam in neonates
1. normal heart rate
2. decreased femoral pulses
3. increased femoral pulses

Back 268

1. 95-180 bpm
2. coarctation
3. patent ductus arteriosus

Front 269

what should be seen on examination fo the umbilicus
what does only one umbilical artery suggest

Back 269

1. two arteries, one vein, NO URACHUS
2. GENITAL RENAL ANOMALIES

Front 270

diastasis recti

Back 270

seperation of the left/right sides of the rectus abdominins at midline-- very common in african american infants -- DONT TREAT

Front 271

umbilical hernia
1. pathogensis
2. clinical presentation
3. treatment

Back 271

1. incomplete closure of the umbilical ring, soft
2. swelling beneath the skin around the umbilicus
3. only treat those that last greater than 4-5 years, or if the hernia is >2 cm

Front 272

persistant urachus pathogenesis + clinical presentation

Back 272

complete failure of urachal duct to close
fistula between the bladder and umbilicus that leaks urine when pressure is appleid over bladder

Front 273

1. meconium plug
2. meconium ileus
3. what disease is meconium ileus associated with
4. when is meconium usually passed

Back 273

1. obstruction of left colon by dehydrated meconium
2. occlusion of the distal ileum caused by thickened/dried meconium 2' defeciency of pancreatic enzymes leading to abnormally high protein content --> inspissated secretions
3. cystic fibrosis
4. normally passed within 24-48 hours

Front 274

what are the abdominal masses in the neonate

Back 274

hydropnephrosis (MCC), cystic kidnesy, ovarian cysts,

Front 275

what can cause a hypertrophied clitoris

Back 275

androgen excess associated with virilizing adrenal hyperplasia

Front 276

hydrometrocolpos

Back 276

imperforate hymen with retention of vaginal secretions

Front 277

hypospadias
+ is it assc. w/inc UTI

Back 277

1. urethral meatus located on ventral surface of penis
2. no not assc with increased with inc. UTI

Front 278

epispadias
+ is it assc. clinical findings

Back 278

urethral meatus located on dorsal surface of the penis,
2. bladder extrophy - bladder protrusion from the abdominal wall with exposure of the mucosa

Front 279

hydrocoele + what condition are they associated with

Back 279

accumulation of fluid through the tunica vaginalis into the testis , ~ ASSC with INDIRECT INGUINAL HERNIA

Front 280

cryptorchidism +assc conditions + major risk

Back 280

undescended testes , genitourinary malformations, hypospadias,

major risk is testicular cancer

Front 281

absence/hypoplasia of radius
1. clinical association

Back 281

1. TAR syndrome, fanconi anemia, holt oram syndrome

Front 282

holt oram syndrome

Back 282

absent radius bone in the arm, ASD/VSD, and first degree heart block

Front 283

edema of the feet + hypoplastic nails

Back 283

turner syndrome
noonan syndrome

Front 284

rocker bottom feet

Back 284

trisomy 18

Front 285

spina bifida
1. external findings
2. myelomeningocoele

Back 285

1. hair tufts, lipomas, dimples, in the lumbosacral area
2. hernial protrusion of the cord and meninges through a defect in the vertebral canal

Front 286

1. definition of prematurity
2. demographics

Back 286

1. birth before 37 weeks, seen in 7% of all births in the US
2. lower socioeconoomic populations, and lack of prenatal care

Front 287

1. definition of post-term
2. complications

Back 287

1. >42 weeks gestation
2. increased death from placental insufficiency, intrauterine asphyxia, meconium aspiration, polycythemia

Front 288

1. small for gestational age definition
2. LGA defninition
3. high birth weight

Back 288

1. <5th percentile for gestational age as a result of IUGR
2. >90th percentile for gestational age,
3. >4000 gram baby

*** LGA does not mean baby has a high birth weight (LGA may mean baby is less than 4000g)

Front 289

IUGR (gestational age + causes)
1. type 1
2. type 2
3. type 3

Back 289

1.conception - 24 weeks gestation interference with fetal growth -- trisomies, TORCH, maternal drugs, maternal db/hypertnesion
2. 24-32wks intrauterine malnutrition- uterine tumors/anomalies/multiple pregnancies, placental insufficnecy, small abnormal placenta
3. late intrauterine malnutrition after 32 weeks - infarct or fibrosis, maternal malnutrition,pregnancy induced hypertension, maternal hypoxemia

Front 290

causes of placental insufficiency

Back 290

renal failure, chronic essential hypertension, collagen vascular disease, pregnancy induced

Front 291

causes of maternal hypoxemia

Back 291

smoking, lung disease

Front 292

complications of small for gestational age infants**

Back 292

growth retardation, congenital anomaies*
asphyxia/MAS*
hypothermia*
hypoglycemia*
polycythemia
thrombocytopenia
hypocalcemia
meconium aspiration syndrome
intrauterine death
hypermagnesia (if mother treated with MgSO4)

Front 293

causes for increased weight/LGA

Back 293

matenral diabetes
beckwith wiedemann
praderwilli
nesidioblastosis (inc proliferation of pancreatic islet cells)

Front 294

what are the compications for LGA

Back 294

polycythemia, hypoglycemia, congenital malformations

Front 295

cyanosis
1. defninition
2. is it dangerous
3. causes

Back 295

1. blue discoloration of the skin/mucous membranes due to <3g/dL reduced Hgb in arterial blood, and >5g/dL Hgb in arterial blood
2. always an emergency
3. TOF, transposiotion, truncus, tricuspid atresia, TAPVR, itnraventricular
hemorrhage, polycythemia,
hypoglycemia, hypocalcemia, hypothyroidism, hypothermia

Front 296

cyanosis
1. steps for evaluation of cyanosis
2. 100% oxygen test results interpretation
3. treatment

Back 296

1. hx/px/CBC/electrolytes/chest radiograph
100% oxygen test
2. if 100% o2 test improves condition significantly >150mmHg, probably lung disease except in those with R-L shunts (<150mmHg increase)
if 100% o2 test does NOT improve condition (<15-20mmHg increase) it is probbably cyanotic heart disease except with increased pulmonary flow (truncus) the PaO2 will increase by >15-20mmHg
3. treat with O2 or intubation inf neccessary

Front 297

1. most common causes of respiratory distress in infants
2. clinical presentation

Back 297

RDS (hyaline membrane disease)
meconium aspiration
persistaent pulmonary hypertension of the newborn
2. tachypnea, dec. gas exchagne, costal retractions,grunting, stridor, flaring of the alae nasi, cyanosis

Front 298

RDS
1. pathogenesis
2. in utero assessment of fetal lung maturity
3. epidemiolgoical risk factors
4. clinical risk factors

Back 298

1.pulm surfactant produced 23-24 weeks, sufficient by 30-32 weeks ~ birth <32 weeks --> inc. surface tension due to dec. surfactant --> RDS
2. sample amnion --> lecithin to sphingomyelin ratio >2:1, presence of phsosphatidylglycerol = maturity
3. white male preterm infants
4. low L:S, prematurity**, maternal diabetes**, neonatal hypothermia/asphyxia, male sex, ceserean section w/o labor

Front 299

RDS
1. clinical presentation
2. dx
3. tx

Back 299

1. tachypnea, retractions, expiratory grunting/cyanosis,
2. CXR - shows atelectasis with inc. density in both lungs GROUND GLASS APPEARANCE AND HYALINE MEMBRNAES, AIR BRONCHOGRAMS (remaining patent airwas surrounded by inc. density of pulmonary fields
3. supplemental oxygen, CPAP, mechanial ventilation (for resp. acidosis), exogneous surfactant

Front 300

RDS
1. acute complications
2. chronic complications*
3. bronchopulmonary dysplasia dx

Back 300

1. intraventricular hemorrhage, pneumothorax, sepsis, R-L shunt across PDA (cyanosis)
2. bronchopulmonaryu dysplasia- progressive pathologic changes in the immature lung affecting parenchyma/airways
3. mechanical ventilation during first 2 weeks of life, need for O2 >28 days of life, respiratory compromise >28 days of life, CXR

Front 301

Persistant pulmonary hypertension
1. what age group infants does this effect
2. pathogensis
3. clinical presentation

Back 301

1. term, or post term
2. perinatal asphyxia/MAS -> inc. pulmonary vascular resistance (due to dec. O2) --> R-L shunt through foramen ovale/PDA
3. cyanosis, respoiratory failure, large dec. PaO2 in response to minimal stimuli, pre/post ductal PaO2 are abnormal

Front 302

PPH
1 dx
2. tx

Back 302

1. decreased pulmonary vascular markings on CXR, echocardiogram
2. oxygen b/c it is POTENT PULMONARY VASODILATOR, mechanical ventialtion, high frequency ventilation, extracorporeal membrane oxygenation (ECMO), inhaled NITRIC OXIDE

Front 303

what is meconium

Back 303

water, mucopoolysaccharides, desquamated skin, gastrointestinal epithelial cells, vernix, bile salts, amniotic fluid

Front 304

MAS
1. pathogenesis
2. clinical presentation of MAS

Back 304

1. post term baby/distress/hypoxia --> meconium stained amniotic fluid --> aspiration of meconium into the airways of the fetus--> distal airways and alveoli
2. respiratory distress, may progresst to hypoxemia/cyanosis

Front 305

MAS
1. dx
2. tx

Back 305

1. CXR shows increased lugn volume with PATCHY atelectasis/infiltration with diffuse areas of atelectasis, pneumothorax, pneumomediastinum
2. oxygen, succtioning the trachea***(first), ECMO, mechanical ventilation

Front 306

apnea of prematurity
1. clinical presentation

Back 306

1. respiratory pause without airflow common in prematurity -->bradycardia, cyanosis, o2 desaturation

Front 307

1. central apnea
2. apnea secondary to airway obstruction
3. mixed apnea

Back 307

1. complete cessation of chest wall movements without airflow
2. cx wall movments or respiratory effort without airflow
3. central + obstructive ~ most common type in infants

Front 308

what are the causes of apnea in preterm infants

Back 308

neonatal infections
lung disease
hypo/hyperthermia
hypoglycmeia
seizures
materanl drugs/withdrawl

Front 309

idiopathic apnea of prematurity
1. pathogeneissi
2. clinical presentation
3. tx

Back 309

1. apnea that is seen in up to 85% of infants <28 weeks gestation and 25% of infants 33-34 weeks gestation
2. occurs 24 hours after birth, resolves by post gestational age 38-44
3. caffeine, theophylline (respiratory stimulants), proprioceptive stimulation, ventillation, CPAP/mechanical ventillation

Front 310

neonatal jaundice
1. how high must bilirubin be to see jaundice
2. physiologic jaundice of the newborn
3. cause of PJN
- in preterm - the peak bilirubin is at 5-7 days and will decrease by 10-14 days

Back 310

1. >5mg/dl
2. indirect hyperbilirubinemia peaks @ day 3-4 (@5-16mg/dL) and resolves by the end of the first week of life, no treatment neccessary
3. inc. bilirubin load on hepatocytes, delayed maturation of the glucuronyltransferase enzyme

Front 311

1. indirect vs direct hyperbilirubinemia
2. which type is pathologic in neonates

Back 311

1. indirect <15% conjugated, direct >15% conjugated
2. direct hyperbilirubinemia

Front 312

breastfeeding jaundice

Back 312

occurs during first week, idue to suboptimal milk intake --> INC. TRANSIT TIME--> INC ENTEROHEPATIC CIRCULATION-->increased unconjugated bilirubin, weight loss, dehydration, dec. stool
*EXACERBATION OF PHYSIOLOGIC JAUNDICE

Front 313

breast milk jaundice
+ Tx

Back 313

occurs AFTER after the first week of life- due to breast milks high beta-glucuronidase/lipase content, and breast milk may also inhibit UDPGA
tx = stop breast feeding for 2-3 days then can start again and the jaundice will be gone

Front 314

DIFFERENTIAL DIAGNOSIS RESP DISTRESS PAGE 98
DIFFERENTIAL DIANGNOSIS BILIRUBINEMIA 103/104

Back 314

...

Front 315

when i jaundice suspicious for underlying pathology

Back 315

<24 hours old
bilirubin rises >5-8mg/dL in 24 hour period or rate >0.5mg/dl/hour rate

Front 316

dx for direct vs indirect hyperbilirubinemia

Back 316

indirect = CBC, reticulocyte count/smear, blood culture
direct = hepatic ultrasound, viral serologies, radio-scans of biliary tree

Front 317

tx. for hyperbilirubinemia of infants

Back 317

serial bilirubin labs
observation/reassurance (for PJN)
phototherpay
exchange transfusion

Front 318

kernicterus
1. pathogenesis
2. clinical presentation

Back 318

1. kernicterus (bilirubin encephalopathy)
very high hyperbilirubinemia--> pass through BBB --> damage to the basal ganglia, hippocapus, brainstem
2. choreoathetoid cerebral palsy, hearing loss, opisthotonus, seizures, oculomotor paralyisis

Front 319

1. what fraction of fetuses are exposed to drugs in utero
2. what obstetric complications
3. clinical presentation
3-10% mortality - perinatal asphyxia, congenital abnormalities, child abuse, SIDS

Back 319

1. 10-15%
2. abruptio placentae, precipitous delivery, preterm labor
3. jitteriness, hyperreflexia, irritability, tremulousness, feeding intolerance, excessive wakefullness

Front 320

tracheoesophageal fistula
1. clinical presentation
2. associated conditions
3. dx/tx

Back 320

1. choking/ aspiration pneumonia if feeding is attempted, polyhydraminos, increased oropharyngeal secretions
2. congenital heart disease, anorectal/skeletal/renal malformations, VACTERL assc
3. oral gastric tube + radiograph~~ surgical repair (closure of the fistula)

Front 321

diaphragmatic hernia
1. pathogenesis
2. clinical presentation + cxr
3. tx

Back 321

1. failure of the diaphragm to form between the fiftha and 8th weeks of gestation--> herniation + impaired lung dev
2. scaphoid abdomen (abdominal cont. in thorax), respiratory insufficinecy, bowel sounds in chest, mediastinal shift on X ray, bowel loops in thorax
3. *** first place NG tube then take XRAY ----- NO BAG AND MASK VENT (will compress lung), treat acidosis, hypoxemia, surgical reduction of the hernia + closure of defect

Front 322

1. omphalocoele
2. assocaited congenital anomalies (compare with gastroschesis)

Back 322

1. true hernia through the umbilical ring, covered with peritoneal sac,
2. ToF, ASD, beckwith wiedemann, trisomy 13

Front 323

1. gastroschisis + tx

Back 323

1. right paraumbilical fissure in anterior abdominal wall, NOT TRUE HERNIA, risk of bowel damage/ischemia ------ tx = sterile wrapping to reduce insensible water loss

Front 324

most common cause of intestinal obstruction

Back 324

intestinal atresia

Front 325

meconium ileus
1. pathogenesis
2. dx
3. tx

Back 325

1. accumulation of intestinal secretions + deficiency of pancreatic enzymes causes inc. viscosity of meconium --> occlusion of the distal ileum
2. abdominal radiogarph showing distension of intestine with no air fluid levels -- SOAP BUBBLES - from fine gas bubbles mixed meconium
3. enemas

Front 326

volvulus

Back 326

loop of intestine twist if attached to narrow band of mesentery --> intestinal gangrene

Front 327

hisrschprungs disease
1. pathogenesis
2. dx/tx

Back 327

1. lack of caudal migration of the ganglion cells from neural crest--> contraction of distal segment with proximal dilation
2. rectal biopsy showing lack of ganglion cells-- resection of affected segment or colostomy

Front 328

necrotizing enterocolitis
1. what type of infants most often get this
2. clinical presentation + x ray
3. treatment

Back 328

1. preterm neonates
2. abdominal distension/tenderness, bloody stools, abdominal erythema, metabolic acidosis
Plain film abdominal xray = PNEUMATOSIS INTESTINALIS (air in bowel wall), dilated bowel loops, venous portal gas
3. bowel rest, gastric decompression, no oral feeding, parenteral fluids/nutrition, resection via surgery

Front 329

hypoglycmeia
1. labs
2. why do infants of diabetic moms have hypoglycemia
3. nesidioblastosis

Back 329

1. glucose <40
2. inc glucose inc. insulin secretion --> transient hypoglycemia
3. islet cell hyperplasia --> persistant hypoglycemia

Front 330

what conditions cause decreased glucose production or substrate supply

Back 330

IUGR
dec. hepatic glycogen
aspyxia
sepsis
galactosemia/fructose intolerace/aminoacidopathies

Front 331

clinical presentation of hypogycemia

Back 331

diaphoresis, jitteriness, tachycardia, hypothermia, seizures, myocardial infarction

Front 332

maternal hyperglycemia
1. pathogenesis
2. clinical presentation
3. associated clinical disease

Back 332

1. inc. glucose --> fetal hyperinsulinemia --> inc. protein synthesis, inc. lipogeneiss, macrosomnia
2. visceromegaly (liver, adrenals, heart), increased skeletal length with small head/face, round facies
3. congenital heart disease, small left colon syndrome--> failure to pass meconium + abdominal distention

Front 333

polycythemia
1. pathogenesis
2. clinical presentation
3. tx

Back 333

1. hematocrit > 65% ~ inc. EPO from placental insuff, inc. RBC , hypoxemia, placental transfusion
2. plethora, poor perfusion, cyanosis, poor feeding, resp. distress, lethargy, jitteriness, seizures, renal vein thrombosis, NEC
3. partial exchange transfusion

Front 334

what is the most common abdominal mass in both male and female newborns

Back 334

hydronephrosis ****
others include ovarian cysts, wilms tumor, multicystic kidneys,

Front 335

what complications is omphalocoele vs gastroschisis assc with

Back 335

omph = congenital heart defects/trisomies 13/18
gastro = bowel obstruction (not assc w/ congenital malformations)

Front 336

what are the causes of primary pulmonary hypertension

Back 336

perinatal asphyxia
merconium aspiration
~ more common in term infants

Front 337

angelman vs prader willi

Back 337

angelmann - mother passes on abnormal chromosome 15
prader willi - father ""

Front 338

malformation
deformation
disruption

Back 338

1. intrinsically abnormal process forms abnormal tissue
2. mechanical forces exerted on normal tissue result in abnormal tissue (ex. amniotic bands)
3. normal tissue becomes abnormal after subject to destructive forces (Ex. ischemia)

Front 339

bladder extrophy pahtogenesis

Back 339

malformation: failure of infraumbilical mesenchyme to migrate to lower abdominal wall

Front 340

alpha feto protein
1. high levels
2. low levels

Back 340

1. neuroal tube defects
ventral abdominal wall defects
fetal demise
fetal skin defects/edema
multiple gestation preg
2. trisomy 21, 18, 13, and intrauterine grwoth retardation

Front 341

what are the three markers in triple screen

Back 341

AFP
unconjugated estriol
B-HCG

Front 342

down syndrome triple screen

Back 342

B HCG high
AFP low
estriol low

Front 343

trisomy 18/13 triple screen

Back 343

B HCG low
AFP low
estriol low

Front 344

1. chorionic villus sampling date and tissue collected
2. amniocentesis sampling date and tissue collected
3. percutaneous umbilical blood sampling

Back 344

1. villus tissue from trophoblast at 10-13 weeks,
2. amniotic fluid with sloughed fetal cells 16-18 weeks
3.obtain sample of fetal blood to assess for hematologic abnormalaiitesl , infections, fetal acidosis, genetic disorders

Front 345

marfans syndrome
1. pathogenesis
2. clinical presentation
3. what other dz has similar presentation

Back 345

1. auto dom fibrillin mutation chromosome 15
2. tall stature, dec. U:L SEGMENT RATIO arachnodactyly, pectus/scoliosis/kyophosis, upward subluxation of lens, aortic root dilation
3. homocystinuria

Front 346

how to treat aortic dilation of marphans to prevent complications

Back 346

beta blockers

Front 347

complications of marphans -

Back 347

endocarditis, retinal detachment, sudden cardiac death
aortic root dilation --> MVP, aortic dilation, aortic regurg, aortic dissection

Front 348

prader-willi syndrome
1. pathogenesis
2. clinical presentation
3. complications

Back 348

1. deletion paternal 15q11
2. failure to thrive (hypotonia/poor feeding)--> obesity/hyprtphagia, small hands/feet, hypotonia, MR, hypogonadism
3. FTT, obstructive sleep apnea (obesity), cardiac disease (obesity), type 2 diabetes

Front 349

angelman syndrome
1. pathogeneiss
2. clinical presentation
3. dx

Back 349

1. maternal 15q11 deletion -
2. jerky arm movements, small WIDE HEAD, blond hair/blue eyes, prognathia, tongue protrusion ataxia, inappropriate laughter, MR + speech delay
3. FISH probing

Front 350

noonans syndrome
1. pathogenesis
2. clinical presentation

Back 350

1. chromosome 12 deletion
2. short stature, shield chest,short neck, low hairline, PULMONARY VALVE STENEOSIS,

Front 351

digeorge
1. pathogenesis
2. clinical presentation

Back 351

1. del 22q11
2. CATCH22 - cardiac (VSD/TOF), abnormal facies (small chin, short palpebral fissures, ear anomalies)thymus (cell mediated immunity deficincy) and parathyroid hypoplasia, cleft palate, hypocalcemia (-->seizures)

Front 352

velocardiofacial syndrome
1. clinical presentation

Back 352

1. cleft palate, wide prominent nose, squared nasal root, short chin, fish shaped mouth, VSD, neonatal hypotonia, perseverative behavior

Front 353

ehlers danlos
1. pathogenesis
2. clincal presentation
3. complications

Back 353

1. mutatoin of type V collagen
2. hyperextensibility of joints, dislocation/scoliosis, velvety skin, with tissue paper thin scars, mitral prolapse, aortic root dilation, fragile blood vessels*, constipation, rectal prolapse, hernias
3. aortic disectiojn, GI bleeding

Front 354

osteogeneiss imperfecta
1. patho
2. clinical presentation

Back 354

1. type 1 collagen mutation
2. blue sclera, HEARING LOSS, fragile bones, genu valgum, scoliosis/kyphsis, osteoporosis, osteopenia, dentinogenesis imperfecta (II/III/IV), easy bruising

Front 355

VACTERL
1. clinical features

Back 355

1. vertebral defects, anal atresia, cardiac anomalies, TE fistula, limb defects (radial hypoplasia), syndactyly, polydactyly
2.

Front 356

CHARGE
1. clinical features

Back 356

1. colobomas, heart defects, choanal atresia, retardation, genital hypoplasia, ears (cup shaped, hearing loss)

Front 357

williams syndrome
1. patho
2. clinical presentaiton
3. dx

Back 357

1. del chromosome 7, elastin
2. COCKTAIL PARTY PERSONALITY, elfin facies, mental retardation, supravalvular aortic stenosis****, hypercalcemia, connective tissue
3. FISH probe

Front 358

cornelia de lange (brachmann de lange) syndrome
1. clinical pres

Back 358

1. single eyebrow, long curly eyelashes, microcephaly, infantile hypertonia, mental retardation, small hands and feet, cardiac defects, autistic features

Front 359

russell silver syndrome
1. clinical pres

Back 359

1. cafe au lait spots-- short stature, skeletal asymmetry, normal head size, small for gestational age, small triangular face, short stature, limb asymmetry

Front 360

pierre robin syndrome
1. clinical pres

Back 360

1. micrognathia, cleft lip/alate, protruding tongue, recurrent otitis, upper airway obstruction

Front 361

criduchat
1. pathogenesis
2. clinical presentation

Back 361

1. del 5
2. CATLIKE CRY - slow growth, microcephaly, metnal rettardation, hypertelorism, downslanting palpebral fissures

Front 362

trisomy 21
1. clinical features

Back 362

1. atlantoaxial instability***, leukemia, celiac disease, OSA, conductive hearing loss, hypothyroidism, cataracts, glaucoma,

~ epicanthal folds, upslanting palpebral fissures,protruding tongue, hypotonia, clniodactyly, palmary creases, duodenal atresia, hirschprungs

Front 363

trisomy 18 clinical presentation

Back 363

1. females > males,
hypertonia (with scissoring), small faces, clenched hands with overlapping digits, rocker bottom feet
~ death by first year

Front 364

trisomy 13 clinical presentation
1. clinical rpes

Back 364

1. holopros, microcephaly micropthalmia, cleft lip/palate,

Front 365

turner syndrome
1. pathogenesis
2. clinical presetantion

Back 365

1. XO paternal non dysjunciton
2. short, webed neck, shield chest, swelling of dorsum of hands and feet, ovarian dysgenesis/delayed puberty, COARCTATION OF THE AORTA

Front 366

fragile x
1. patho
2. clinical pres

Back 366

* most common inherited form of mental retardation
1.X LINKED trinucleotide CGG repeats with NTICIPATION
2. mild to severe MR, large ears, macrocephaly, large testes, behavioral finidngs

Front 367

klinefelters syndrome
1. pathogensis

Back 367

*most common cause of male infertility
1. XXY
2. tall starture long extremities
hypogonadism- delayed puberty
gynecomastia
antisocial behavior, shyness, aggression

Front 368

define
rhizomelia
mesomelia
acromelia
spondylodysplasias

Back 368

proximal long bone abnormalities - achondroplasia
medial long bone abnormalities
distal abnormalities
spine abnormalities

Front 369

achondroplasia
1. pathogenesis
2. clinical presentation
3. complications

Back 369

1. sporadic autosomal dominant FGFR3 mutation
2. megalencephaly, foramen magnum stenosis, frontal bossing, mid face hypoplasia, lumbar kyophosis, lumbar lordosis, rhizomelic shortening, tridnet hands, otitis media, conductive hearing loss
3. hydrocephalus from foramen magnum stenosis, head sweating, dilated facial veins, SIDS, OSA, bowed legs/back pain

Front 370

potter syndrome
1. patho + clinical pres

Back 370

1. amniotic fluid leak, bilateral renal agenesis, polycystic kidneys, intrauterine renal failure, renal agenesis, polycystic kidneys, severe oligohydraminos --> lung hypoplasia + fetal compression

Front 371

amniotic band syndorme

Back 371

1. rupture of amniotic sac, small strands of amnion wrap around the fetus

Front 372

fetal alcohol syndrome
1. clinical pres

Back 372

1. SGA, FTT, microcephaly, smooth philtrum****, smooth thin upper lip, mental retardation, ADD/ADHD, VSD

Front 373

fetal phenytoin syndrome
1. clinical presentation

Back 373

1. wide anterior fontanelle, thick hair, low nasal bridge with low hairline, hypoplasia of the distal phalanges and nails/ cardaic defects, cleft lip/palate

Front 374

fetal cigarette smoking

Back 374

SGA, polycythemia

Front 375

fetal cocaine

Back 375

IUGR, microcephaly, GU abnormalities

Front 376

fetal DES

Back 376

inc. risk of cervical carcinoma, GU abnormalities

Front 377

fetal isoretionoin

Back 377

CNS malforation, cardiac defects, microtia

Front 378

fetal propothiouracial

Back 378

hypothyroidism
goiter

Front 379

fetal thalidomide

Back 379

phocomelia

Front 380

fetal valproic acid

Back 380

narrow head, high forehead, mid face hypoplasia, NEURAL TUBE DEFECTS (spina bifida), cardiac defects, convex nails

Front 381

fetal warfarin

Back 381

hypoplastic nose with deep groove betwen nasal alae and nasal tip, stippling of the epiphyses, hypoplastic nails

Front 382

most common congenital anomaly of the CNS

Back 382

neural tube defects

Front 383

inborn errors of metabolism
1. when do they typically present
2. how does mitochondrial disorders differ form most IEMs

Back 383

1. severe neontatal illness at times of stress, chronic or progressive symptoms
2. slow progressive course,

Front 384

what other disease is IEM have a similar clinical presentation

Back 384

sepsis

Front 385

what labs should be ordered for suspected IEM

Back 385

metabolic acidosis and elevated serum ammonia

Front 386

how to treat IEM associated hyperammoniemia and acidosis

Back 386

hyperammoniema - sodium benzoate, sodium phenylacetate
neosporin and lactulose prevent bacterial ammonemia in the colon
acidosis - sodium bicarbonate
dialysis

Front 387

homocystinuria
1. pathogensis
2 clincal pres
3. dx
4.tx

Back 387

1. AR cystathione synthase deficiency
2. marphanoid body w/o arachnodactyly
DOWNWARD lens subluxation
hypercoagulability, THROMBOEMBOLISM*** of pulmonary/systemic arteries, and cerebral vasculature
mitral/aortic regurg (no dilation compare to marphans)
scoliosis
developmental delay
3. elevated methionine in the urine, by urinary cyanide nitroprusside test
4. methionine restricted diet, aspirin (thromboembolis)

Front 388

transient tyrosinemia of the newborn
1. patho
2. clincal pres
3. tx

Back 388

1. high proiten diet in first two weeks of life -->
2. poor feeding/letheragy
3. high serum tyrosine/phenylalanine
4. vitamin C, dec. protein intake

Front 389

cystinuria patho

Back 389

renal defect in reasbsorbtion of cystine, lysine, arginine, ornitihine --> renal stones (UTI/dysuria/urgency/freq)

Front 390

hartnup disease patho

Back 390

AR neutral amino acid defect --> dec. tryptophan --> dec. niacin--> ataxia, photosensitive rash, MR, emotional lability

Front 391

PKU
1. clinical pres
2. dx/tx

Back 391

1. mousy or musty odor, hypotonia, eczema, hypopigmentation,
2. inc. phenylalanine, inc. phenylpyruvate and phenylacetic acid and dec tyrosine in the urine~~ phe restricted diet

Front 392

maple syrup urine disease
1. clinical pres
2. dx/tx

Back 392

1. urine has maple syrup odor, hypoglycemia, severe acidosis, lethargy, hypotonia, coma
2. inc. serum/urine BCAA~~

Front 393

transient hyperammonemia of the newborn
1. patho + clinical pres

Back 393

1. immature urea cycle enzymes in premature infant~ 24-48 hours of life, respiratory distress, alkalosis, vomiting, lethargy, coma

Front 394

clinical presentaiton of hyperammonemia

Back 394

TOXIC TO BRAIN AND LIVER
poor feeding
hyperventilation
behavioral changes
seizures
ataxia
coma

Front 395

OTC def
1. patho
2. dx/tx

Back 395

1. increased ammonia in teh blood from dec formation of citrulline --> vomiting, lethargy, coma ~ females have cyclic vomiting and intermittent ataxia
2. elevated orotic acid, elevated ornithine, decreased citrulline-- low protein diet,

Front 396

tyrosinemia type 1
1. clinical presentatoin
2. dx/tx

Back 396

1.fumarylacetoacetate hydrolase --> elevated tyrosine --> rotten fish odor, episodic peripheral neuropathy, RENAL TUBULAR FUNCTION AND LIVER, hepatocellular carcinoma/cirrhosis
2. succinylacetone in urine, diet restriction of phenylalanine

Front 397

galactosemia
1. patho
2. clinical pres
3. dx/tx
4. complications

Back 397

1. defect in galactose 1 phosphate uridyl transerase -->
2. HEPATOMEGALY, HYPOGLYCEMIA
vomitting, diarrhea, FTT, oil droplet cataracts, RTA
3. non-glucose reducing substance in urine by clnistix
~ galactose restricted diet
4. ecoli sepsis***, female ovarian failure

Front 398

hereditary fructose intolerance
1. patho
2, sx

Back 398

1. fructose 1 phosphate aldolase B deficieny -
2. hypoglycemia, vomitting, diarrhea, FTT, seizures, avoiding fructose, sucrose, sorbitol

Front 399

von gierke
1. patho + clinical pres
2. tx
3. complications
4. lab findings

Back 399

1. glucose 6 phosphatase deficiency --> heaptomegaly, HYPOGLYCEMIA DURING FASTING**** metabolic acidosis, hypertriglyceridemia, enlarged kidneys
2. high carboydrate diet
3. hepatocellular carcinoma
4. hyponatremia,hypokalemia due to the fact that they are hyper lipidemic which dilutes serum ion concentrations

Front 400

pompe's disease
1. patho + clinical pres

Back 400

1. alpha glucoseidase deficiency --> musucla weakness, CARDIOMEGALY, flaccid weakness, poor feeding, hepatomegaly

Front 401

lab findings of fatty acid oxidation defects

Back 401

non-ketotic ypoglycemia, hyperammonemia, myopathy, cardiomyopathy

Front 402

medium chain acyl CoA dehydrogenase
1. pathogenesis + clinical pres
2. dx/tx

Back 402

1. dec. ability to breakdown fatty acid chains---> non ketotic hypoglycemia, hyperammonemia, myopahty, cardiomyoapahty
2. tandem mass spec showing inc. medium chain fatty acids,
~ tx = high carboydrate low fat diet, carnitine supplementation during acute episodes

Front 403

when to suspect mitochondrial disorders

Back 403

atypicl presentation
THREE OR MORE ORGAN SYSMTES

Front 404

kearns sayre syndrome
1. patho + clinical pres

Back 404

mitochondrial disorder ~ ophthalmoplegia, pigmentary degeneration of retina, hearing loss, heart block, neuro degeneration

Front 405

MELAS
1. patho + clinical pres

Back 405

mitochondrial disorder ~ nitochondrial encephalopathy, lactic acidosis, stroke like episodes

Front 406

tay sachs disasease
1. patho
2. clinical pres of infantile and adult onset
3. dx/tx

Back 406

1. AR hexaminidase A def --> accumulation of GM2 ganglioside
2. EARLY INFANCY-->hyperacusis, increased startle cherry red macula, macrocephaly, progressive blindness, severe developmental delay * NO HEPATOSPLENOMEGALY***--> death by age 4
> 2 years of life ataxia, dysarthria, choreoathetosis~ NO cherry red macula --> chronic debilitation
3. inc GM2 ganglioside, dec. HEX A activity

Front 407

gauchers disaease
1. patho + clinical pres

Back 407

1. AR glucocerebrosidase deficiency -- > glucocerebroside accumulation in spleen, liver, and erlenmeyer flask shaped distal femur, tissue macrophages --> leukopenia/anemia/thrombocytopenia from replacement of the marrow

Front 408

niemann pick disease
1. patho

Back 408

1.sphingomyelinase defeciency --> progressive neurodegeneration, ataxia, seizures heatosplenomegaly, cherry red macula ~ death by age 4 (like tay sachs)

Front 409

metachromatic leukodystrophy

Back 409

arylsulfatase A def --> accumulation of sulfatides --> ataxia, seizures, progressive mental retardation~ death by 10-20

Front 410

clinical presentation of mucopolysaccharidoses

Back 410

lysosomal storage disorders --> organomegaly, short stature, MR, DYSOSTOSIS MULTIPLEX - thickened cranium, J shaped sella, ovoid or beak like vertebrae, short/thickened clavicles, dolichocephaly

Front 411

hurler syndrome
1. patho + clinical pres
2.dx/tx

Back 411

1. AR alpha L iduronidase deficiecy - hepatosplenomegaly/kyophosis, dystosis multiplex progressively coarsened facial features, frontal bossing, prominent sagital/metopic sutures, wide nasal bride, hydrocephalous CORNEAL CLOUDING , stiff/contracted joints
2. dx = dermatan/heparin sulfates in urine + dec. alpha L iduronidase
tx = bone marrow transplant

Front 412

hunter syndrome
1. patho + clinical pres
2. dx/tx

Back 412

1. X linked deficiency iduronate sulfatase --> hepatosplenomegaly, dystosis multiplex NO CORNEAL CLOUDING,
2. dx= dermatan sulfatates
tx = none, death by age 15

Front 413

porphyria
1. patho + clinical pres
2. dx/tx

Back 413

1. drugs/hormone surges/poor nutrition --> skin photosensitivity, personality changes, emotional labiltiy, colicky abdominal pain.vomiting, autonomic instablity****, dark colored urine
2. increased serum prophobilinogen
IV glucose, correction of electrolytes, avoidance of fasting and precipitating drugs

Front 414

wilsons disease
1. patho + clincal pres
2. dx/tx

Back 414

1. AR defect in ceruloplasmin copper excretion--> copper depositoin in the brain, eyes, heart, liver*** kaiser fleishcer rings, dystonia, dysarthria, tremors, ataxia, seizures, hepatic dizease, fanconi syndrome, changes in behavior
2. dec. ceruloplasmin**, elevated serum/urine copper, liver biopsy shows copper
tx = avoid copper containing food chelation with oral penicillamine + zinc salts

Front 415

menkes kinky hair disease
1. patho + clinical pres
2. dx

Back 415

1. XLR abnormal copper transport -> low serum copper pale kinky friable hair, low serum copper, optic nerve atrophy, severe mental retardation
2. hair findings + low ceruloplasmin and copper

Front 416

what kind of formula will be asymptomatic in galactosemia

Back 416

soy formula

Front 417

hurlers/hunters disease clinical presentation

Back 417

coarse facial features, corneal clouding (not present in hunter's syndrome), dysostosis multiplex (thickened skull, abnormal vertebrae, abnormal ribs, and clavicle*, developmental delay

Front 418

which disease do the patients look similar to turners patients

Back 418

noonans ~~ looks similar and also has right sided heart lesions

Front 419

clinical presetaiot
1. PKU
2. tyrosinemia type 1
3. maple syrup urine disease

Back 419

1, developmental delay, hyperactivity, mousy/musty odor
2. peripheral neuropahty, renal and liver disease, rotton fish/cabbage odor
3. developmental delay

Front 420

gauchers clincal presentation

Back 420

erlenmeyer flask femur -

Front 421

1. short stature
2. normal variant short stature
3. pathologic short stature

Back 421

1. height less than two standard deviations below the mean
2. below the third percentile but normal growth velocity
3. child who is below third percentile but is growing with suboptimal growth velocity

Front 422

children who grow 2 inches per year between years 3 and puberty

Back 422

...

Front 423

what is calculation of male/female MPH

Back 423

male = fathers height + (mothers height + 5inches)/2
female = (fathers height -5) + mothers height /2

~ when children have completed their growth they are within 2-4 inches of this calculation

Front 424

two causes of normal variant short stature

Back 424

familial short stature
constitutional growth delay -- delayed puberty

Front 425

familial short stature

Back 425

>2 SD below the average, with short MPH
BONE AGE is normal with minimum of 2 inches of growth per year

Front 426

constitutional short stature

Back 426

>2 SD below average height with history of delayed puberty, delayed growth spurt and delayed bone age

Front 427

proportionate (pathologic) shrot stature
1. define
2. causes ~ pre/post natal

Back 427

1. height falls more than 3SD below the mean with abnormal growth velocity <2 in/year
2. PRE-- enviornmental exposures, chromosomal (down/turnor), genetic (russell silv, prader willi, viral infections (CMV/rubella), fetal alcohol, malnutrition
POST--malnutrition, psychosocial, organ disease

Front 428

disproportionate short stature
1. define

Back 428

1. short staure in patients who have short legswiht an increased U/L ratio
2. skeletal dysplasias, including rickets

Front 429

what should be tested for in pathologic short stature

Back 429

1. IGF1****, CBC, ESR, T4, serum electrolytes, serum creatinine, bicarbonate

**bone age ~~ AP film of left hand/wrist to assess epiphysis, AP and lateral skull to ***** the pituitary

Front 430

bone age = chronological age

Back 430

familial short stature
IUGR
turner sydnrome
skeletal dysplasias

Front 431

bone age<chronologic age

Back 431

constitutional short stature
hypothyroidism
hypercortisolism
growth hormone defeiciency
chornic diseases

Front 432

growth hormone deficincy (patho short stature)
1. pathogenesis
2. clinical pres
3. dx/imaging
4. tx

Back 432

1. brain tumors, craniopharyngioma, irradiation, trauma, congenital midline defects (ex. single central maxillary incisor or cleft palate)
2. delayed bone age (by Xray of left hand),
3. low IGF1, poor IGF1 response with L-dopa/glucagon/clonidine stimulation, MRI of the head,
4. dailyu injections of GROWTH HORMONE

Front 433

what kind of tumor must be considered in any child older than 5 years of age who is not growth > 2 inches/year

Back 433

craniopharyngioma ~~ because it will decrease the production of growth hormone

Front 434

hypothryoidism (pathologic short stature)
1. pathogenesis + clincial findings

Back 434

1. anti-TPO antibodies --> low TSH/T4

Front 435

hypercortisolism short stature

Back 435

iatrogenic cortisol use leads to poor growth and weight gain...

Front 436

tx for turner syndrome short stature

Back 436

growth hormone

Front 437

what prevents puberty in childhood
what changes at the onset of puberty

Back 437

hypothalamic sensitivity to sex steroids increases negative feedback
at the onset of puberty - decreased hypothalamic inhibition results in activation of HPG axis

Front 438

1. age of onset of female puuberty
2. thelarche
3. adrenarche
4. menarche

Back 438

1. 7-13 years of age
2. breast development (breast buds = FIRST SIGN)
3. pubic/axillary hair development from adrenal androgens
4. opset of menstruation, mean 12.5 years (9-15 range)

Front 439

1. age of onset male puberty +first sign

Back 439

1. 9-14, testicular enlargement (testucular length >2.5cm)

Front 440

1. precocious puberty in female
2. """ male

Back 440

1. breast development before age 7, or menarche before age 9
2. testicular changes/penile enlargment or pubic/axillary hair before age 9

Front 441

premature thelarche

Back 441

1. 2 years of life- transient activation of the HPGA axis --> palpable breast tissue

Front 442

premature adrenarche

Back 442

> 5 years of age, onset of pubic or axillary hair without development of breast tissue or enlarged testes, more common in girls

Front 443

1. isosexual precocious puberty or central precocious puberty (CPP)
2. cause in girls vs boys
3. diagnosis
4. what to do in boys with a positive CPP

Back 443

1. early hypothalamus activation --> early onset of gonadotropin mediated puberty~~~ looks like normal puberty just early
2. idiopathic in girsl, pathologic in boys (hydrocephalus, CNS infections, cerebral palsy, hamartomas, astrocytomas/glyomas, head trauma)
3. GnRH stimualtion test~~~ in someone with CPP the LH/FSH will dramatically rise where as in someone who is pre-pubertal there will be very little rise in LH/FSH
4. MRI of the head because this is almost always pathological in boys

Front 444

hypothyroidism associated precocious puberty

Back 444

...

Front 445

waht are the complications of phenytoin

Back 445

optic atrophy***most commonly
gingival hyperplasia ** common side effect
acute hepatic failure
lymphoma like syndromeraynaud phenomenon

Front 446

what kind of acid base disturbance from pyloric stenosis

Back 446

metabolic alkalosis

Front 447

erythema nodosum

Back 447

tender erythematous nodules associated with histoplasmosis, tuberculosis, cocci, and group A strep

Front 448

tourettes syndrome tx

Back 448

haloperidol

Front 449

urine findings
1. hurler syndrome
2. galactose
3. manoose
4. maple syrup urine smell
5. sweaty feet smell

Back 449

1. dermatan/heparan sulfage
2. galactosemia
3. mannosidosis
4. branched chain aminoacidemia (maple syrup urine disease
5. isovaleryl CoA dehydrogenase def

Front 450

Peripheral precocious puberty
1. pathogensis + clinical presentation
2. diagnostic test

Back 450

1. peripheral production of sex steroids that are not FSH/LH mediated. --->
Boys: gynecomastia or pubic hair with NO TESTICULAR ENLARGEMENT (because NO FSH***)
Girls: virilization or breast develpment
2. flat GnRH stimulation test because HPGA axis is not activated

Front 451

what are the causes of peripheral precocious puberty

Back 451

INDEPENDENT OF THE HPGA
exposure to exogenous sex steroids
gonadal tumors (leydig, B-HCG tumors,mccune albreight, virulizing ovarian tumors ex. granulosa cell)
adrenal tumors
non-classic CAH

Front 452

mccune albreight clinical presentation

Back 452

polyostotic fibrous dysplasia
cafe aulait spots
PPP/hyperthyroidism/hyperadrenalism

Front 453

testotoxicosis

Back 453

cause of PPP
~ testes enlarge independent of HPGA

Front 454

why does B-HCG tumors cause PPP

Back 454

because B-HCG has some LH like activity which causes the testes to enlarge and Leydig cells to produce androgesn

Front 455

definition of delayed puberty in boys/girls

Back 455

boys = no testicular enlargement by 14 years of age
girls = no breast tissue by 13 years of age, or no menarche by 14 years of age

Front 456

1, hypogonadotropic hypogonadism
2. labs
3. GnRH test

Back 456

inactivity of the hypothalamus and pituitary gland
Labs = low FSH, low LH, low testosterone, low estradiol, flat GNRH test

Front 457

1. hypergonadotrophic hypogonadism
2. labs

Back 457

1. end organ failure --> rising FSH/LH, low testosterone/estradiol

Front 458

constitutional delay of puberty

Back 458

immature hypothalamus ~ more common in boys
* often there is a family history ~ growth spurt late in college or highschool

Front 459

kallman syndrome

Back 459

isolated gonadotropin deficiency associated with anosmia

Front 460

laurence moon biedl syndrome

Back 460

obesity, retinitis pigmentosa, hypogonadism, polysyndactyly, mental retardation

Front 461

klinefelter syndrome/turner syndrome effect on HPG axis

Back 461

hypergonadotrophic hypogonadism

Front 462

what other diseases is autoimmune oophoritis associated with

Back 462

hashimotos thyroiditis, addisons disease,

Front 463

when do the gonads become differentiated

Back 463

week 9 SRY has turned the testes

Front 464

development of the male internal/external genetalia

Back 464

SRY gene causes differentiation into testes which produce AMH which causes involution of the mullerian structures and leydig cells produce testosterone which develops the wolfian system

external genetalial are formed by the conversion of testosterone to dihydrotestosterone by 5 alpha reductase ~ finished by 12 weeks

Front 465

mixed gonadal dysgenesis

Back 465

45XO/46XY mosaic - gonadal intersex (both male/female internal structures) ~ streak gonad on one side, testes and vas deferans on the other side

Front 466

true hermaphrodites

Back 466

46XX or 46XY, gonadal intersex, have both ovaries and testes tissue and ambiguous genetalia

Front 467

partial androgen insensitivity

Back 467

46XY --partial or incomplete peripheral androgen resistance results in defectie androgen binding in genital tissue ~~ hypospadias, cryptochoridsm,

Front 468

testicular femininzation syndrom

Back 468

46XY with no response to testosterone **EXTREME ANDROGEN INSENSITIVITY ~~ these individuals have phenotypic female external genetalia, with internal testes and no mullerian structures, no wolffian structures

Front 469

CAH

Back 469

21 hydroxylase deficiency MCC female pseudohermaphroditism

Front 470

clinical presentation of 11 beta hydroxylase deficinecy

Back 470

increased blood pressure with ambiguous genetalia in a genetic female

Front 471

primary adrenal insufficiency

Back 471

problem at the level of the adrenal gland

Front 472

clinical presentation of primary adrenal insufficieny

Back 472

cortisol def signs - hypotension, inc. pigmentation, weakness, anorexia
aldosterone def signs - hyponatremia, hyperkalemia*, FTT, salt craving

Front 473

diseases that cuase primary adrenal insufficiency

Back 473

addisons
CAH
adrenoleukodystrophy (X linked)

Front 474

secondary adrenal insufficinecy
1. define
2. electrolytes """
3. causes

Back 474

1. problem at the level of the hypothalmus or pituitary ( no CRH or ACTH)
2. serum potassium NORMAL ~ BECAUSE ALDOSTERONE AXIS IS INDEPENDENT OF HPG AXIS
3. craniopharyngioma, histiocytosis, IATROGENIC by glucocoticoid use*************

Front 475

acquired adrenal insufficiency pathogenssis

Back 475

addisons
chronic corticosteroid use

Front 476

CAH
1. pathogenesis + clinical presentation
2. dx

Back 476

1. AR defect in adrenal cortex --> inc. production of androgens--> anorexia, vomitting, diarrhea, dehydration at 5-15 days of life
2. elevated 17 hydroxyprogesterone

Front 477

21 hydroxylase def
1. pathogenesis
2. simple virilizing vs non-classic CAH3
3. lab dx of CAH
4. tx

Back 477

1. dec. 21 OH leads to inc. androgens
Girls have ambig genetalia, and electrolyte abnormalities
2. simple virilizing = no effect on aldosterone,
non-classic = late onset with no mineralocorticoid involvement
3. incrased 17 hydroxyprogesterone
4. cortisone suppresses ACTH production, flurocortisol suppresses mineralocorticoid production

Front 478

1. 11 B hydroxylase deficiency clinical presentation
2. lab diagnosis

Back 478

hypertensive and hyperkalemic (because 11-deoxycorticosterone has some mineralocorticoid activity) + ambig genetalia in females
2. elevated 11-deoxycortisol

Front 479

1. 3 B hydroxysteroid dehydrogenase deficiency clinical presentation
2. lab diagnosis

Back 479

1. severe salt wasting crisis, glucocorticoid deficiency, and ambiguous genetalia
2. elevated 17 hydroxyprogesterone

Front 480

addisons disease
1. pathogenesis

Back 480

1. AUTOIMMUNE DESTRUCTION - of the adrenal cortex by lymphocytic infiltration

Front 481

type 1 polyglandular syndomre

Back 481

hashimotos + type 1 diabetes + addisons

Front 482

type 2 polyglandular syndrome (schmidt syndrome)

Back 482

hypoparathyroidism, chronic mucocutaneous candidiasis, + addisons

Front 483

waterhouse friedrichsen syndrome

Back 483

acute adrenal hemorrhage, shock, DIC and MENINGOCOCAL SEPSIS***

Front 484

what test for adrenal insufficinecy

Back 484

ACTH stimulation test~~ if there is blunted response to ACTH there is primary adrenal indsufficeincy

Front 485

treatment for Adrenal crisis

Back 485

5% dextrose in normal saline to stabilize volume + glucose level, IV steroids to replace lost cortisol

Front 486

most common cause of hypercortisolism

Back 486

iatrogenic ~ ie. giving steroids to patients

Front 487

cushing 'syndrome'

Back 487

1. EXCESS GLUCOCORTICOIDS BY BENING/MALIGNANT ADRENAL TUMORS

Front 488

cushing 'disease'

Back 488

excess *********ACTH production by pituitary tumor

Front 489

diagnosis of cushings disease

Back 489

dexamethasone suppression test, if cannot suppress the adrenal there is an ACTH tumor
~~~ high dose dex suppresses pituitary tumor
~~~ high dose dex doesn't suppress ectopic ACTH producing tumor

Front 490

HLA haplotypes (chromosome 6) associated with Type 1 diabetes

Back 490

HLA DR3/DR4

Front 491

potential environmental triggers for diabetes

Back 491

viral infections (coxsackie and rubella), cows milk (maybe)

Front 492

type 1 diabetes melitus
1. immunoglobulins
2. clinical presentation
3. dx
4. tx

Back 492

1. ANTI-ISLET ANTIBOTIES
anti - insulin
aanti - glutamic acid decarboxylase
~~~ ATTACK THE ISLETS****
2. polyuria, polydipsia, nocturia
3. blood sugar >200mg/dL with polyuria, polydipsia, weight loss, nocturia.
4. various types of insulin, insulin pumps, glucagon (for rescue),

Front 493

what vaginal disease in girls predisposes to type 1 diabetes

Back 493

monilial vulvovaginitis

Front 494

adolescent type 1 DM pathogenesis

Back 494

1. sex steroids and growth hormone are antagoinistic to insulin action

Front 495

honeymoon period after starting treatment for diabetes

Back 495

transient recovery of residual islet function decreases insulin need ~ islets temporarily respond to carbohydrate load

Front 496

micro/macrovascular complications of diabetes

Back 496

1. micro = retinopathy, nephropathy, neuropathy
2. atherosclerosis, hypertension, heart disease, stroke

Front 497

what fraction of childhood diabetes is type 2 in children

Back 497

2-3 %

Front 498

type 2 DM
1. patho
2. clincial pres
3. tx

Back 498

1. STRONG HEREDITARY COMPONENET** peripheral insulin resistance ---> progressive insulin decline
2. DKA (not serious), NKHHS, obesity, acanthosis nigricans (inc. insulin-->inc. IGF-->melanocyte prolif)
3. oral hypoglycemics, insulin

Front 499

DKA
1. clinical definition
2. pathogenesis
3. clinical presentation
4. lab dx
5. tx

Back 499

1. hyperglycemia >300, ketonuria, bicarbonate <15, pH<7.3
2. insulin deficiency--> cells starved of glucose + hyperglycemia --> glucagon/cortisol/growth hormone/epinephrine-->ketogenesis (glucagon)
3. fruity breath vomitting, polyuria, polydipsia, dehydration, abdominal pain, coma (ketones)
4. anion gap metabolic acidsis, hyperglycemia, ketonemia HYPERKALEMIA
5. isotonic saline to correct dehydration, gradual decline in osmolality, potassium REPLETION b/c cellular K+ is depleted, insulin,

Front 500

pathogenesis of cerebral edema after treating DKA

Back 500

1. drop in serum glucose >100mg/dL/hour, fluid administration >4 L/m2/24 hours--> osmotic shift from hypotonic intravascular space to the brain --> BRAIN EDEMA

Front 501

how does T3/T4 circulate in the blood

Back 501

bound to thyroid binding globulin, and thyroid binding pre-albumin

Front 502

clinical presentation of hypothyroidism

Back 502

delayed bone age/suboptimal growth velocity (<5cm/year [2in])
goiter
myxedema (puffy/dry/orange skin)

Front 503

somogyi phenomenon

Back 503

too high evening insulin dose --> hypoglycemia in the morning--> epi/glucagon-->ketones + hyperglycemia ~~ TX BY LOWERING THE BEDTIME INSULIN

Front 504

most common metabolic disorder
most common cause of acquired hypothyroisism
most common cause of hyperthyroidism in childhood

Back 504

congenital hypothyroidism
hashimotos
graves

Front 505

thyroid dysgenesis

Back 505

absent thyroid gland, some have ectopic thyroid that can be found between tongue and mid chest

Front 506

thyroid dyshormonogenesis

Back 506

inborn errors of thyroid hormone synthesis~ a cause of congenital hypothyroidism

Front 507

pendred syndrome

Back 507

thyroid organification defect + sensorineural hearing loss ~~ most common cause of thyroid dyshormonogenesis

Front 508

PTU use during pregnancy effect on teh fetus

Back 508

transient hypothyroidism

Front 509

maternal autoimmune thyroid disease effect on the fetus

Back 509

matrnal anti-thyroid IgG cross placenta and destroy fetal thyroid with anti- TPO, anti-thyroglobulin, anti-TSH-R

Front 510

clinical presentation of congenital hypothyroidism

Back 510

asymptomatic at birth
* T3 essential for brain development
~ prolonged jaundice/poor feeding, large tongue/posterior fontanelles, protruding tongue, umbilical hernia, myxedema, mottled skin, hypothermia, PERMANENT mental retardation *CRETINISM

Front 511

chronic lymphocytic thyroiditis (hashimotos)
1. pathogenesis
2. clinical presentation
3. dx/tx

Back 511

1. lymphocytic infiltration of thyroid + anti -TPO/thyroglobulin antibodies--> fibrosis, atrophy/hyperplasia
2. transient HYPERTHYROIDISM then hypothyroidism, pebbly* goiter, short stature
3. neonatal screen (TSH), high TSH, low T4, antithyroid antibodies (anti-TPO)
tx = synthroid when patient becomes hypothyroid

Front 512

clinical presentation of hyperthyroidism

Back 512

exophthalmos, smooth* goiter, tachycardia/palpatations, flushing of skin, nervousness, fine tremors, fatigue, delayed menarche, gynecomastia, delayed puberty

Front 513

what skin manifestation indicates polyglandular autoimmune disease

Back 513

vitiligo or alopecia

Front 514

graves disease
1. pathogenesis
2. labs
3. tx

Back 514

1. anti-TSH-R IgG --> excess thyroid hormones
2. low TSH, high T3/T4
3. PTU (also inhibits peripheral conversion of T4-T3), methimazole, thyroidectomy, radioactivce iodine

Front 515

which hormones release calcium from the bones

Back 515

vitamin D/PTH

Front 516

PTH effects

Back 516

releases Ca2+/phos from the bones, reabsorbing calcium/HCO3- at kidneys
secretion of phosphate secretion at the kidneys
stimulates 1-alpha-hydroxylase at kidney to inc. conversion of 25- 1,25 OH vitamin D

Front 517

1,25 vitamin D effect

Back 517

inc. reabsorbtion of phos/Ca2+ at kidney
inc. release of Ca/phos from bones
***inc. reabsorbtion of Ca through the intestines

Front 518

1. hypocalcemia lab def
2. clinical features of hypocalcemia

Back 518

1. <8 mg/dl or <2.5 mg/dl
2. tetany, carpopedal spasm, laryngospasm, paresthesias, seizures, long QT

Front 519

pseudohypocalcemia

Back 519

low serum albumin in nephrotic syndrome looks like low total calcium levels

Front 520

early neonatal hypocalcemia

Back 520

1. <4 days old, prematuity, IUGR, asphyxia, maternal diabetes, hypomagnesemia

Front 521

late neonatal hypoparathyroidism

Back 521

>4 days of age, due to maternal hyperparathyroidsim, temporary

Front 522

hyperphosphatemic hypocalcemia
1. pathogenesis

Back 522

1. uremia, renal failure, excess phosphate intake--> elevated phosphate/calcium binding and deposition

Front 523

childhood hypoparathyroidism

Back 523

ring chromosome 16 or 18, autoimmune,digeorge

Front 524

pseudohypoparathyroidism (Albrights hereditary osteodystrophy)

Back 524

end organ (bone/kidney) unresponsiveness --> short stature, short metacarpals, elevated PTH, delayed bone age, increased bone density, 4-5 digit brachydactly, obseity, subcapcuslar cataracts, periventricular/perivasclar/basal ganglia calcifications

Front 525

hypomagnesemia related hypocalcemia

Back 525

low magnesium interferes with PTH release

Front 526

labs which should be ordered to evaluate hypocalcemia

Back 526

ca, phos, mg, EKG (look for long QT), PTH level, vitamin D level (if BOTH Ca/phos are low), radiograph of wrists and knees

Front 527

how to evalulate for rickets

Back 527

radiographs of wrists/knees

Front 528

treatment for hypocalcemia

Back 528

~ treat when Ca2+ <8 mg/dl
oral (mild)
intravenous calcium gluconate (symptomatic)
1,25 vitamin D

Front 529

rickets
1. pathogenesis
2. risk factors
3. clinical presentation
4. dx + XR

Back 529

1. vitamin D def--> inadequate mineralization of bones with NORMAL bone matrix
2. breast feeding with no sun, phenytoin, phenobarbital, renal/hepatic failure, fad diets, celiac disease, cystic fibrosis,
3. first 2 years/adolescence, knobby wrists, knees, ribs, bowing of weight bearing bones, short stature, rachitic rosary, craniotabes, frontal bossing, delayed suture closure
4. low phosphorus, low/nrml Ca, inc. Alk phos, inc. PTH, HYPOCALCURIA
wrist radiographs show WIDENED, FRAYED, CUPPPED metaphysis, widened space between epiphysis and metaphysis

Front 530

Vitamin D dependent rickets
1. pathogenesis
2. labs

Back 530

1. AR mutation of 1 alpha hydroxylase
2. high PTH, low vitamin D, low ca/phos, inc. alk phos

Front 531

vitamin D resistant rickets
1. pathogenesis
2. labs
3. clinical pres **

Back 531

1. XLD disorder, renal tubular phosphate leakage
2. normal calcium, low phosphorus,
3. bowing BUT NO TETANY***~ compare to vitamin D dependent rickets and secondary rickets

Front 532

diabetes inspidus
1. pathophysiology
2. central vs nephrogenic
3. labs
4. dx

Back 532

1. defective/deficienct effect of ADH leads to inability to concentrate urine
2. central = no ADH, nephrogenic =ADH resistance
3. NORMAL ELECTROLYTES IF THIRST IS INTACT. urine inappropriatly dilute, urine SG <1.018
4. water deprivation test- rising serum osmolality with persistantly low urine osmolality, and good response to DDAVP = central DI
if no response = nephrogenic DI

Front 533

central DI causes
central DI tx

Back 533

1. autoimmune, trauma, craniopharyngioma, glioma, germinoma, langerhans cell histiocytosis, sarcoid/TB, vascular, genetic
2. DDAVP

Front 534

1. hypoglycemia definition
2. clinical presetnation

Back 534

1. <40mg/dL in serum
2. lethargy, myoclonic jerks, cyanosis, apnea, seizures

Front 535

transient neonatal hypoglycemia
1. causes

Back 535

NOT ENOUGH SUBSTRATE: prematurity, perinatal asphyxia, fetal distress, SGA, LGA (ie. all of these conditions have low glycogen stores)
INAPPROPRIATE HYPERINSULIN: maternal diabetes

Front 536

persistent neonatal hypoglycemia
1. causes

Back 536

islet cell hyperplasia (nesidioblastosis)
beckwith widemann (visceromegaly, hemihypertrophy, macroglossia, umbilical hernias, distinctive ear creases) von gierke, galactosemia, maple syrup urine disease, tyrosinemia,
growth hormone deficiency
congenital hypopituitarism

Front 537

most common cause of hypoglycemis in children age 1-6 yeasr

Back 537

ketotic hypoglycemia

Front 538

ketotic hypoglycemia
1. clinical pres

Back 538

1. children age 1-6 yrs, hypoglycemiain the morning, + ketonnuria, low insulin, due to inability to adapt to fasting state

Front 539

what conditions cause short stature with delayed bone age

Back 539

constitutional growth delay, hypothyroidism, hypercortisolism

**NOT in genetic short stature, IUGR, turners, skeletal dysplasias, growth retardation ~~~ all of these the bone age is the same as the chronological age

Front 540

1. sudden change of mental status while treating a patient with DKA
2. electrolyte changes in DKA

Back 540

1. cerebral edema
drop in serum glucose>100mg/dl/hour, excess fluid administration,
2. hypoglycemia, hypocalcemia (osmotic loss/phosphorus usage, hypokalemia(causes arrythmias),

Front 541

parinaud oculoglandular syndrome
1. patho

Back 541

1. atypical bartonella, tularemia, tuberculosis, syphilis --> conjunctiva --> pre-auricular lymphoadenopathy + conjunctivitis

Front 542

werdnig hoffman disease
1. patho + clinical pres

Back 542

1. AR destruction of anterior horn cells -->rapid motor weakness that begins in infancy--> weakness/paralysis, hyporeflexia, muslce fasciculations

~~ slower version = kugelberg welander

Front 543

acute cerebellar ataxia
1. patho + clinical pres

Back 543

1. viral illness (cox/vari/echo) -->autoimmune response ---> cerebellar ataxia (nystagmus, vomitting, truncal ataxia)

Front 544

Apert syndrome
1. clinical presentation

Back 544

1. AD craniosynostosis + syndactyly of the fingers/toes

Front 545

describe the arthritis of rheumatic fever

Back 545

migratory polyarthritis effecting mostly the LARGE JOINTS, pain and swelling of one joint subcides as it begins in another joint
*Fever/carditis may or may not be present at this time
SPECC - subcutaneous nodules, polyarthreitis, erythema nodosum, chorea, carditis

Front 546

TAPVR
1. patho + clinincal pres

Back 546

1. pulmonary veins drain to the right atrium, then some oxygenated blood makes it through to the foramen ovale/PDA to the body --> CYANOSIS in infant + DIFFUSE RETICULAR PATTERN OF LUNG FIELDS on radiograph

Front 547

where are pediatric brain tumors located

Back 547

infratentorial, and midline

Front 548

intussucception
1. age of onset + clinical findings
2. tx

Back 548

1. second half of first year of life, teloscoping of ileum into the colon --> fever, palpable sausage shaped mass, + CURRANT JELLY STOOL
2. barium enema

Front 549

1. risk factors for brain abscess in a child

Back 549

right to left intracardiac shunts/cyanotic heart disease, otitis media, mastitis,

Front 550

why does right to left cardiac shunt/congenital heart disease lead to increased risk of brain abscess/infection

Back 550

blood that passes through the shunt avoids the lungs and its macrophage filtering mechanism

Front 551

what is the treatment for pertussis

Back 551

azithromycin (used to be erythromycin) within 2 weeks of onset of coughing.... after this it is a toxin mediated disease

Front 552

1. three types of juvenile idiopathic arthritis
2. what does ANA positivity increase risk for in childe with juvenille idiopathic arthritis

Back 552

1. systemic, pauciarticular, polyarticular
2. uveitis

Front 553

hand/foot syndrome + dactylitis******

Back 553

classic presentation of sickle cell disease, symmetric swelling of the hands and feet

Front 554

which tumor of childhood mets to bones most often

Back 554

NEUROBLASTOMA****

Front 555

chronic non-specific diarrhea

Back 555

toddlers diarrhea-- diarrhea with normal growth and physical examination-- no treatment neccessary

Front 556

clinical findings of PDA

Back 556

wide pulse pressure (accentuated peripheral pulses), and continuous murmor

Front 557

what endocrine disorder should be associated with a child who has amidline defect ~ cleft palate, hypoglycemia, and microphallus

Back 557

...congenital hypopituitarism

Front 558

stains for:
1. acid fast baccili
2. fungus
3. stool WBCs
4. herpes family, RSV, adeno, influenza A/B
5.trichamonas vaginaliss
6. treponema pallidum
7. cocci/ m.tuberculosis
8. EBV, CMV, VZV, HIV, toxo, bartonella, myocplasma

Back 558

1. ziehl- neelsen
2. silver stains
3. wright stain
4. fluorescent antibody
5. direct observation
6. dark field
7. intradermal skin testing
8. antibody testing

Front 559

what are the markers of systemic inflammation/infection

Back 559

CRP (Rises fast) and ESR (rises slow)

Front 560

what is fever

Back 560

rectal temp >100.4

Front 561

what temperature should an infant with fever be evalulated for meningitis, pneumonia, sepsis, bone/joint infections

Back 561

<28 days ANY FEVER
>28 days fever >39 (102.2)

Front 562

clinical features of infection in young infants

Back 562

non-specific = fever, dec. appetitie, irritability, cough, rhinorrhea, vomitting, diarrhea

Front 563

pathogens causing sepsis/meningitis + tx
1. 0-1 month
2. 1-3 months
3. 3mo - 3yr
4. 3yr-adult

Back 563

1. GBS, ecoli, listeria---amp + genta + cefotax + acylcovir (if cutaneous vesicles/apnea/seizures)
2. GBS, pneumo, listeria--- amp + cefotax + vanc
3. pneumo, h.flu, neisseria --- cefotax + vanc
4. penumo, neisseria --- cefotax + vanc

Front 564

1. labs which should be ordered in suspected sepsis/meningitis
2. lab results that indicate low risk
3. who should be hospitlaized

Back 564

1.CBC + blood culture, urinalysis + urine culture, CXR
2. WBC <15k
bands <1.5k
normal urinalysis <10WBCs
<5 WBCs
normal
3. all infants <28 days of age
infants 29-3 months with toxic apppearance, meningitis, pneuonia, pyelonephritis, skin/soft tissue who do not meet low risk criteria

Front 565

Fever in 3-36 month child
1. sepsis/meningitis bacterial etiologies 3-36 months
What to do when:
A. child appears toxic
B. nontoxic appearaing and temp <102.2
C. nontoxic appearing and temp >39 (102.2)

Back 565

1. s. pneumo, h.flu type B, neisseria
A. hospitalization with complete sepsis evaluation
B. no labs required, observe closely at home
C. urine culture for males <6mo and females <2mo
blood culture if WBCs>15k
chest radiograph if respiratory distress, rales, or tachypnea
stool culture if blood and mucus in teh stool or if >5WBCs
empiric antibiotics for children whose WBCs>15k

Front 566

FUO - fever of unknown origin
1. definition
2. cause
3. lab studies
4. radiologic studies

1/4 resolve spontaneously

Back 566

1. fever that lasts longer than 8 days to 3 weeks, or when prior history, physical and lab evals have failed to lead to a diagnosis
2. common illness with atypical presentation~ big table 7-2
3. CBC/blood culture, ESR or CRP, ASO, serum transaminases, urinalysis/urine culture, ANA, stool culture, tuberculosis test, HIV test
4. CXR, echocardiography, bone scan, CT and MRI (depending on the clinical clinical pres)

Front 567

pseudotumor cerebri

Back 567

increased intracranial pressure with normal with normal brain anatomy (mimics a tumor) caused by imparied reabsorbtion of CSF---- symptom = headache, complication is optic atrophy/blindness
tx = serial spinal taps, acetazolomide, steroids

Front 568

what treatment for children born to HBsAg positive mothers

Back 568

HBIG and and HBsAg recombinant vaccine in the first day of life

Front 569

what are the common complications of maternal diabetes on an infant

Back 569

cardiac malformations (TGA, VSD)
lumbosacral agenesis
neural tube/renal defects
duodental atresia/small left colon
holoprosencephaly/anencephaly

Front 570

aniridia in an infant is associated with what syndomre

Back 570

wilms tumor, aniridia, genitourinary nomalies, retardation , HEMIHYPERTROPHY
WAGR complex

Front 571

bacterial meningitis in infants/young children
1. most common age
2.risk factors
3. clinical presentation

Back 571

1. 1st month of life
2. young age, immunodeficiency, complement deficiency, ventriculoperitoneal shunt, basilar skull fracture
3. MINIMAL OR ABSENT FEVER, BULGING FONTANELLE****, non-specific signs/symptoms (fussiness, poor feeding, irritabilty, lethargy, respiratory distress

Front 572

clinical presentation of bacterial meningities in an older child

Back 572

fever and signs of meningeal irritation
altered LOC, nuchal rigidity, kernigs (painful flexion of the leg with the hip at 90'/brudzinskis sign (pressure on the cheek causes flexion of arms and hips)
seizures, photophobia, emesis, headache

Front 573

lumbar puncture results for bacterial meningitis

Back 573

WBC>5k with predominance of neutrophils
hypoglycorrhachia CSF:serum <0.40
increased protein
positive gram stain and culture

Front 574

labs + imaging which must be tested for a suspected case of bacterial menintgitis

Back 574

lumbar pucture with culture
blood culture
CT with contrast (to find brain abscess)

Front 575

what is the role of corticosteroids given with the first dose of antibiotics for suspected meningitis

Back 575

reduces the incidence of hearing loss in HIB meningitis

Front 576

bacterial meningitis CSF: WBC, protein, glucose, gram stain

Back 576

WBC 100-50k, high protein, low glucose positive gram stain

Front 577

viral meningitis: CSF: WBC, protein, glucose, gram stain

Back 577

WBC 10-1000 monos + lymphs, HSV shows RBCs
protein normal, glucose normal, enterovirus/HSV identified by PCR

Front 578

tuberculosis meningitis: CSF: WBC, protein, glucose, gram stain, CT FINDINGS

Back 578

WBC 10-500 lymphos
protein very high, glucose low
PCR may be positive, smear usually negatie
CT FINDINGS = ENHANCEMENT OF THE BASILAR CISTERNS

Front 579

fungal meningitis:CSF: WBC, protein, glucose, gram stain

Back 579

WBC 25-500 lymphos
protine - normal to high
glucose low
culture positive, india ink for cryptococcus

Front 580

parameningeal focus (brain abscess) CSF findings

Back 580

WBC 10-200, PMNs/monos
protein high
glucose normal
culture negative

Front 581

bacterial meningitis complications

Back 581

death 5-50% (gram neg>s.pneumo>HIB>neisseria)
HEARING LOSS
GLOBAL BRAIN INJURY
SIADH, seizures, hydrocephalus, cranial nerve palsy, learning disability

Front 582

aseptic meningitis

Back 582

inflammation of the meninges with CSF lymphocytic pleocytosis, no protein, normal glucose

Front 583

1. clinical presentation of aseptic meningitis
2. clinical presentation of tuberculosis meningitis

Back 583

1. mild fever, headache, emesis, altered level of consciousnss, seizures
2. initially lethargy or irritability, but then progresses to cranial nerve defecits, LOC, paraplegia,death

Front 584

viral meningitis dx

Back 584

culture takes 10-14 days,
PCR for EBV CMV HSV enteroviruses

Front 585

TB meningitis brain imaging results

Back 585

basilar enhancement

Front 586

1. viral causes of meningitis
2. bacterial aseptic meningitis causes
3. fungal ""
4. parasitic """

Back 586

1. enteroviruses, LCM, HSV, EBV, CMV, VZV, arbo, influenza
2. tuberculosis, borrelia burgdorferia, treponema pallidum
3. cocci, crypto, histo
4. t. solium, toxo gondii

Front 587

tx. for viral and TB meningitis

Back 587

viral is self limiited
TB- 4 drug TB treatment + corticosteroids

Front 588

1. causes of viral URI
2. clinical presentation of viral URI
3. persistnet fever/symptoms >10 days indicates what
4. tx

Back 588

1. rhinovirus, parainfluenza, corona, RSV
2.low grade fever,rhinorrhea, cough, sore throat lasts 7-10 days
3. bacterial superinfections
4. adequate hydration, mucolytic, cough suppressant, decongestants, NO ANTIBIOTICS

Front 589

sinusitis pathogenesis
1. development of the sinuses

Back 589

1. ethmoid/maxillary - 3rd/4th month of gestation, sphenoid between 3 and 5 years of age, and frontal sinuses between 7 and 10 years of age

Front 590

sinusitis
1. acute perisitant
2. acute severe
3. subacute
4. chronic

Back 590

1. nasal discharge AND****cough 10-30 days, headache, malodorous breath
2. HIGH FEVER with purulent discharge >3-5 days
3. same as acute perisistant but 30-90 days
4. same as acute persistant but >90 days

Front 591

sinusitis
1. pathogens
2. treatment

Back 591

1. pnuemo, h.flu, moraxella, ~ except chronic which may be s.aureus/anaerobes *** OFTEN SECONDARY TO PRECEDING VIRAL URI******
2. amoxicillin, or amoxicillin/clavuloanate, or second gen cephalosporin
~~ except chronic which is broad spectrum oral, CT image, and IV abx

Front 592

pharyngitis
1. viral causes
2. bacterial causes
3. is it possible to tell what type of organism is causing the pharyngitis based on clinical presentation

Back 592

1, cox, EBV, CMV
2. s. pyogenes, arcanobacterium hemolyticum, c.diptheriae
3. NO NO NO

Front 593

1. EBV pharyngitis clinical presentation + tx
2. coxsackie pharyngitis clinical presentation

Back 593

1. enlarged POSTERIOR CERVICAL NODES, malaise, hepatosplenomegaly
~~ Tx = corticosteroids
2. painful ulcers on posterior pharynx and mouth (herpangina), and blisters on palms and soles

Front 594

GABHS pharyngitis
1. clinical presentation
2. dx/tx

Back 594

1. NO URI symptoms, exudates on the tonsils, petichiae on the tonsils, strawberry tongue, and large tender ANTERIOR CERVICAL lymph nodes, fever, scarlatiniform rash
2. rapid strep test, or culture, penicillin VK, single dose of IM benzathine penicillin, or oral macrolides

Front 595

1. diptheria clinical presentation
2. tx

Back 595

gray adherent tonsillary membrnae , low grade fever
--> cardiac failure and neurologic complications
2. macrolide or IV penicillin + antitoxin

Front 596

acute otitis media
1. AOM vs OME
2. pathogens
3. clinical pres
4. dx/tx

Back 596

1. AOM is an acute infection of the middle ear, OME is just fluid in the middle ear without infection
2. s.pneuo, NTHI, m.catarrhalis
3. fever, ear pain, dec. hearing +/- pus drainage if tympanic membrane perforates
4. PNEUMATIC OTOSCOPY-- TYPMPANIC MEMBRANE DOES NOT MOVE INDICATING FLUID IN THE MIDDLE EAR -- erythema/loss of tympanic membrane landmarks are less reliable
tx = amoxicillin, or amox-clav, or cephalosporin or macroides

Front 597

otitis externa
1. pathogensis
2. pathogens
3. cliniccal presentation

Back 597

1. disruption of protective mechanisms: cerumen, swimming, excessive moisture
2. pseudomonas, staph, candida
3. pain/itching/drainage
4. dx - erythema of external auditory canal tenderness while moving tragus,
tx- acetic acid wash, topical antibiotics/topical corticosteroids
~ if perforated use both oral and topical abx

Front 598

cervical lymphadenitis
1. causes
2. reactive lymphadenitis
3. clinical presentation
4. tx

Back 598

1. s.aureus (MCC UNILATERAL), s.pyogenes, MAC, m.tuburculosis, B.henselaek, EBV (BILATERAL), CMV, HIV, kawasaki disease, t.gondii, branchial cleft cyst, cystic hygroma
2. response to infections of the pharynx, teeth, soft tissues of head/neck
3. mobile, tender, with erythematous skin over the node, single or multiple nodes
4. cephalosporin or anti-staphylococcal penicillin (Dicloxacillin or cephalexin or clindamycin) + incision/drainage

Front 599

parotitis
1. viral pathognes + clinical presentation
2. bacterial pathogens + clinical presentation

Back 599

1. MUMPS, EBV, CMV, HIV, influenza~ BILATERAL PAROTITIS
2. s.aureus, s.pyogenes, M.tubuerculosis - unilateral parotid involvement

Front 600

mumps clinical presentation + complications

Back 600

parotitis****
complications = meningoencephalitis (during or after parotitis), orchitis (post pubertal), epididymitis, pancreatitis, abscess/osteomyelitis of the jaw

Front 601

impetigo
1. pathogenesis
2. clinical presentation
3. tx

Back 601

1. superficial skin infection with s.aureus or s.pyogenes
2. honey colored crusted or bullous lesions on the face/nares VERY CONTAGIOUS
3.mupirocin (topical) or oral dicloxacillin, cephalexin, clindamycin

Front 602

complications of strep impetigo

Back 602

post strep glomerulonephritis
staph scalded skin syndrome
bacteremia
NOT RHEUMATIC FEVER****

Front 603

erysipelas
1. pathogenesis
2. complications

Back 603

1. group A beta hemoloytic strep --> dermal lymphatics --> TENDER ERYTHEMATOUS SKIN WITH A DISTINCT BORDER~ usually effects the face and scalp
2. post strep glomerulonephritis,necrotizing fasciitis, bacteremia

Front 604

cellulitis
1. pathogenesis
2. clinical presentation

Back 604

1. skin infection in the dermis caused by group A strep and S.aureus
2. erythema,warmth, tenderness, indistinct border (compare to erysipelas)
3. first gen cephalosproins, anti-staph penicillins

Front 605

buccal cellulitis
1. clnical presentation
2. tx

Back 605

1. bluish discoloration of the cheek in unimmunized child caused by HIB
2. IV 3rd gen cephalosporins (cefotax/cefuroxime), lumbar punctrue to check for meningitis

Front 606

perianal cellulitis
1. clincal presentation.
2. treatment

Back 606

1. erythema of the anus caused by group A strep
2. cefalexin (first get cepho)

Front 607

necrotizing fasciitis
1. pathogenesis
2. clinical presentation
3. tx

Back 607

1. deep cellulitis that is potentially fatal--
2. crepitus and hemorrhagic bullae
3. IV antibiotics and surgicla debridement

Front 608

staph scalded skin syndrome
1. pathogenesis
2. clinical presentation

Back 608

1. s.aureus exfoliative toxin
2. fever, tender skin, bullae, sloughing of sheets of skin, Nikolsky sign (extension of bullae when pressure applied to the skin), tends to be in flexor surfaces

Front 609

scarlet fever
1. pathogenesis/clinical presentation

Back 609

1. group A strep produces erythrogenic toxin --> fever/chills/malaise/exudative pharyngitis--> blanching erythematous scarlatiniform (sandpaper like) rash
Pastias lines - petechiae at skin creases form a linear distribution
--> desquamation when the infection resolves

Front 610

scarlet fever
1. dx/tx
2. complications***

Back 610

1. throat culture or rapid strep test
tx = penicillin, IM penicillin, or macrolides
2. rheumatic fever, post strep glomeruloneprhitis, post streptococcal arthritis, pediatric autoimmune neuropsychiatric disorders associated iwth streptococcal infection (OCD, tic disorders after strep infection)

Front 611

which post strep infection complications are not prevented by antibiotics

Back 611

post-strep glomerulonephritis
post strep arthritis

Front 612

toxic shock syndrome
1. pathogenesis/clinical presentation
2. tx

Back 612

1. S.aureus (sometiems s.pyogenes) --> exotoxins + toxic shock syndrome toxin --> >101 fever, hypotension, diffuse erythroderma (sunburn like rash), desquamation
+ multisystem involvement (>3)--- gastrointestinal, myalgias, hyperemia, thrombocytopenia***
2. anti-staph antibiotics, IVIG , treat the shock/nidus of infection

Front 613

classic cause of toxic shock syndrome

Back 613

retained tampons

Front 614

rotavirus
1. pathogenesis + clinical presentation
2. dx/tx

Back 614

1. fecal oral in winter--> 1-3 day incubation --> vomiting, diarrhea, dehydration (self limited)
2. stool ELISA, tx= hydration

Front 615

norwalk viurs
1. pathogenesis + clinical presetnation

Back 615

1. fecal oral outbreaks in closed populations --> vomitting (self limited in 48-72 hours)
~ often associated with daycare, cruise ships etc

Front 616

lab studies to order for diarrhea

Back 616

CBC, serum electrolytes, stool guaiac, WBCs, ova/parasite, ELISA - for rota, giardia, c.diff

Front 617

ETEC
1. clinical presentation
2. dx/tx

Back 617

1. non-invasive watery diarrhea-- MCC travelers disarrhea
2. absent stool WBCs, culture
tx = quinolones or sulfonamides, hydration

Front 618

EPEC
1. clinical presentation
2. dx/tx

Back 618

1. non-invasive watery diarrhea in preschoolers
2. absent stool WBCs stool culture
tx- quinolones or sulfonamides, hydration

Front 619

EHEC
1. clinical pres
2. dx/tx

Back 619

1. bloody diarrhea, HUS (0157 H7)
2. WBCs are PRESENT in the stool, culture
tx = NO NO NO ABX IF HUS IS PRESENT****THIS ENHANCES ENDOTOXIN RELEASE

Front 620

shigella sonnei
1. clinical pres
2. dx/tx

Back 620

1. bloody diarrhea, seizures in children (fron neruotoxin)
2. stool WBCs, stool culture is diagnostic,
tx = third generation cephalosporin, fluoroquinolones

Front 621

salmonella
1. clinical pres
2. dx/tx

Back 621

1. fecal oral poultry/milk/eggs/lizards/turtles ---> bloody/non-bloody diarrhea + rose spots on the abdomen
2. +/- stool WBCs, culture is diagnostic
tx = NOT INDICATED FOR GASTROENTERITIS IN IMMUNOCOMPETENT HOSTS BECAUSE IT INCERESES CHRONIC CARRIER RATE
3rd gen cephalosporins

Front 622

capmpylobacter ejuni
1. clinical pres
2. dx/tx

Back 622

1. poultry exosure --> bloody diarrhea
2. stool WBCs present + stool culture
tx = erytrhomycin

Front 623

yersinia enterocolitica
1. clinical pres
2. dx/tx

Back 623

1. diarrhea + mesenteric adenitis (mimics appendicitis)
2. stool culture or mesenteric node grwos organism
tx = cephalosporins

Front 624

c. difficle
1. clinical pres
2. dx/tx

Back 624

1. post Abx use --> pseudomembranous colitis
2. toxin in the stool + endoscopy shows pseudomembranes
tx = metranididazole or vancomycin

Front 625

v. cholerae
1. clinical pres
2. dx/tx

Back 625

1. massive watery diarrhea
2. history of living in endemic area or travel, culture, serology
tx = fluid replacement

Front 626

acid base disturbance in diarrhea

Back 626

non-anion gap hyperchloremic metabolic acidosis
~~ due to loss of bicarbonate

Front 627

HIV
1. pathogenesis

Back 627

1. in utero, intrapartum, post partum
transmission from mother to chlid is ~5% by using anti-retrovirals durign pregnancy

Also: sexual contact, blood products, sharing needles

Front 628

what factors increase risk of vertical transmission of HIV

Back 628

high viral load
chorioamnionitis
maternal genital infections
premature birth
prolonged rupture of memrbanes

Front 629

what factors decrease risk of vertical transmission of HIV

Back 629

undetectable viral load
cesarean section
post exposure prophylaxis of the infant + mother being adherent to treatment

Front 630

Early symptoms of HIV in an infant

Back 630

FTT, thrombocytopenia, recurrent infections (otitis, pneumonia, sinusiits), lymphadenopathy, parotitis, recurrent thrush, loss of dev. milestones, zoster/varicella

Front 631

HIV in infant
1. is an antibody titer that is positive for the first 2 years indicative of disease
2. dx of HIV in infant

Back 631

1. NO THESE ARE MATERNAL ANTIBDOES
2. HIV DNA PCR, if negative at 4 months

Front 632

prophylactic treatment for infants born to HIV positive mothers

Back 632

zidovudine for 6 weeks post exposure
TMPSMX for PCP
NO BREASTFEEDING
urine CMV culture

Front 633

treatment of HIV positive children

Back 633

antiretrovirals
prophylaxis for oppurtunistic infections
immunizations (EXCEPT LIVE VARICELLA)
MONITOR VIRAL LOAD
ANNUAL OPHTHALMOLOGIC EXAMINATION to assess CMV retinitis

Front 634

PCP pneumonia in an HIV+ infant
(most common oppurtunistic infection in HIV infected children)
1. clinical pres
2. tx

Back 634

1. fever, HYPOXIA, interstitial infiltrates
2. TMPSMX prophylaxis + TMPSMX, pentamidine, or atovaquone (ubiquinone analog)

Front 635

MAC clinical presetnation

Back 635

often seen in HIV positive patients with CD4<50
fever, weight loss, night sweats, abdominal pain, bone marrow suppression, inc. AST/ALT

Front 636

what fungal infections seen in HIV positive patients

Back 636

candidal thrush/esophagitis
cryptococcus pneumonia/meningitis
histo, cocci, aspergillus

Front 637

what viral infections seen in HIV positive patients

Back 637

CMV retininitis/esophagitis/colitis
HSV, VZV

Front 638

what parasitic infections seen in HIV positive patients

Back 638

cryptosporidium, toxoplasmosis, isospora

Front 639

what kind of neoplasm common in HIV positive patients

Back 639

B cell lymphoma caused by EBV

Front 640

infectious mononucleosis
1. pathogenesis + clinical pres
2. dx
3. tx

Back 640

1. EBV (most commonly), also less commonly toxo,CMV,HIV in saliva --> infection of B lymphocytes--> fever, malaise/fatigue, pharyngitis(exudative), POSTERIOR CERVICAL LYMPHADENOPAHTY, hepatosplenomegaly***, macular or scarlatiniform rash (rare)
2. monospot test identifying heterophile antibodies (agglutinates sheep RBC)
IgG anti VCA, EA and EBNA
IgM anti VCA with no* EBNA = acute infection
atypical lympohcytes on smear, neutropenia, thrombocytopenia, inc. AST/ALT
3. supportive, corticosteroids for sever pharyngitis

Front 641

1. what is the cause of heterophile negative infectious mononucleosis
2. how to diagnose EBV in children <4 years of age

Back 641

CMV, toxo, HIV, also monospot test is less sensitive in chidlren less than 4 years of age
2. EBV antibody titers anti- VCA, EA, EBNA
EBNA = late antibody
IgM anti VCA = early antibody

Front 642

EBV mononucleosis complications

Back 642

cranial nerve palsies
encephalitis
upper airway obstruction (from pharyngitis)
splenic rupture** (no heavy lifting/contact sports)
nasopharyngeal carcinoma
burkitts lymphoma
lymphoproliferative disease

Front 643

patient with exudative pharyngitis is treated with amoxicillin for suspected group A strep infection 1 week later develop pruritic macularpapular rash. Why?

Back 643

did not have group A strep
they had EBV mononoculeosis which has this reaction when treated with amoxicillin

Front 644

measels (rubeola)
1. clinical presentation
2. complications
3. tx

Back 644

1. 12 day incubation --> cough, conjunctivitis, coryza (THREE Cs ****), fever >101 Koplik spots (gray papules on erythematous base) --> erythematous maculopapular rash that STARTS ON NECK/EARS THEN EXTENDS TO CHEST/UPPER EXTREMITIES IN FIRST 24 HOURS THEN LOWER LIMBS BY 48 HOURS--> rash resolves by day 4-7
2. bacterial pneumonia (hemorrhagic), encephalomyelitis, otitis media, laryngotracheitis,
3. vitamin A, immunoglobulin for post exposure prophylaxis

Front 645

rubella (togavirus)
1. clinical presentation in children
2. complications

Back 645

1. 2-3 week incubation period --> astymptomatic or fever <101,mild URI, suboccipital/ posterior auricular/cervical lymphadenopathy**, non-pruruitic, maculopapular and confluent exanthem with same progression as measels (head to toe/extremities)
2. meningoencephalitis, polyarteritis**

Front 646

congenital rubella syndrome (CRS)

Back 646

first trimester rubella infection --> 30-50% CRS
blueberry muffin rash (purpura)
congenital cataracts
PDA, microcephaly
sensorinerual hearing loss
meningoencephalitis
thrombocytopenia, hepatosplenomegaly, jaundice, purpura
Late = mental retardation, type 1 diabetes, hypertension, autoimmune thyroid disease

Front 647

aspergillosis
1. invasive disease clinical pres + tx
2. allergic bronchopulmonary aspergillosis clinical pres _ tx

Back 647

1. immunocompromised pts --> invasive disease tx = ampho B, resect aspergilloma
2. pts with chronic lung disease (CF etc) --> wheezing, eosinophilia, pulmonary infiltrates ****HIGH levels of aspergillous IgE -- tx = corticosteroids + antifungals

Front 648

candida
1. disease in immunocompetent
2. disease in immunocompromised

Back 648

1. mild superficial infections, oral thrush, diaper dermatitis, vulvovaginal candidiasis
2. fungemia, meningitis, osteomyelitis, endophthalmitis

Front 649

coccidioides immitus
1. pathogenesis + clinical presentation
2. tx

Back 649

1. fungus found in SW united states and mexico--> asymptomatic/mild pneumonia, **africans, filipinos, pregnant, neonates, immunocompromized --> disseminated meningitis, severe pneumonia, osteomyelitis
2. mild diseaes- no tx, CNS cryptococcal infection includes systemic antifungal

Front 650

cryptococcus
1. pathogenesis + clinical presentation
2. tx

Back 650

1. yeast in soil --> inhalation--> asymptomatic in immunocompetent,
immunocompromised: CNS meningitis, disseminated infection of bones/joints/skin
2. systemic antifungal

Front 651

entamoeba histolytica
1. pathogenesis + clinical presentation
2. clinical presentation
3. dx/tx

Back 651

1. ingestion of cysts in contaminated food or water --> invasion of colonic mucosa--> mild colitis or sever dysentary (young children/pregnant/immunocomp) cramping, abdominal pain, tenesmus, diarrhea with blood or mucus, weight loss, hepatomegaly, chest pain/right shoulder pain, respiratory distress
2., intestinal perforation/hemorrhage, strictures, ameboma (local inflammatory mass), ABSCESS IN THE LIVER**** (also brain/lung/other)
3. trophozoiites/cysts in stool, colonoscopy/biopsy, CT scan of liver
tx = metronidazole, iodoquinol (luminal amebicide)

Front 652

giardia lamblia
1. pathogenesis + clinical presentation
2. dx/tx

Back 652

1. fecal oral ingestion of cysts from contaminated water in U.S./daycare centers--> 1-2 wk incubation-->small bowel --> voluminous watery, FOUL SMELLING stool, bloating, flatulence, weight loss
2. stool examination for cysts/trophozoites
tx = etronidazole, furazolidone

Front 653

malaria
1. pathogenesis
2. dx/tx

Back 653

1. plasmodium falciparum(also vivax, malariae, ovale) ~ bite from anophales mosquito --> flu like prodrome, chills, fever, vomiting, headache, abdominal pain every 48-72 hours as a result of RBC rupture and parasitemia
~ hemolytic anemia, splenomegaly, jaundic, hypoglycemia, cerebral malaria, renal failure/shock
2. giemsa stained peripheral blood- to identify plasmodium
tx = chloroquine, quinine, quinidine gluconate, mefloquine, doxycycline

Front 654

prevention of malaria

Back 654

DEET repllants, bed nets, protective clothing
chemoprophylaxis with chloroquine/mefloquine/doxycycline/atovaquone/proguanil hydrochloride

Front 655

toxoplasmosis
1. pathogenesis + clinical presentation

Back 655

1. direct contact with cat feces, eating undercooked meat, fruts/vegetables, transplacental, blood products/transplant -->
Immunocompetent : asymptomatic, or MONONUCLEOSIS LIKE ILLNESS
Immunocompromised/PREGNANT: encephalitis, focal brain lesions/seizures, disseminated
2. PCR, serology, finding organisms in CSF, blood
tx = sulfadiazine and pyrimethamine

Front 656

congenital toxocplasmosis clinical presentation

Back 656

hydrocephalus, intracranial calcifications, chorioretinitis

Front 657

1. what groups are most at risk for helminth infections
2. diagnosis of helminth infections

Back 657

1. imigrants, travelers, homeless
2 three stool examinations for ova and parasites, cellulose tape test for enterobius

Front 658

cystercercosis
1. pathogenesis + clinical presentation
2. dx/tx

Back 658

1. mexico/central america fecal oral taenia solium eggs --> encyst in muscle, subcutaneous tissue, subcutaneous nodules, 4th ventricle, meninges, spine, eyes --> SEIZURES (most common presenting symptom), hydrocephalus, stroke
2. ova and parasite stool, head CTshowing parenchymal cysts/calcifications(non-viable)
tx = anti-convulsant therapy

Front 659

enterobius vermicularis
1. pathogenesis + clinical presentation
2. tx

Back 659

1. fecal oral transmission of eggs --> anal/vulvar pruruitus/insomnia, anorexia, enuresis, teeth grinding
2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

Front 660

ascaris lumbricoides
1. pathogenesis + clinical presentation
2. tx

Back 660

1. fecal oral egg ingestion --> loefflers syndrome = transietn pneumonitis, fever, cough, wheezing, eosinophilia, small bowel obstruction
2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

Front 661

trichuris trichiuria
1. pathogenesis + clinical presentation
2. tx

Back 661

1. ingestion of eggs --> abdominal pain, tenesmus, bloody diarrhea, RECTAL PROLAPSE
2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

Front 662

necator americanus/ancylostoyma duodenale (hookworm)
1. pathogenesis + clinical presentation
2. tx

Back 662

1. human feces contaminated soil --> percutaneous infection through bare foot migrate to lungs coughed up and swallowed --> rash/pruritus at site of penetration, IRON DEFICINECY ANEMIA with fatigue, abdominal pain, eosinophilia, pica, pallor, FTT
2. tx = mebendazole, albendazole or pyrantel pamoate (for the whole family)

Front 663

strongyloides stercoralis
1. pathogenesis + clinical presentation
2. tx

Back 663

1. same lifecylce as hookworm --> transient pruritic papules at site of penetration--> loefflers pneumonitis, GI symptoms, eosinophilia
2. ivermectin, thiabetdazole, albendazole

Front 664

cutaneous larva migrans
1. pathogenesis + clinical presentation
2. tx

Back 664

1. intradermal migration of dog or cat hookworms after contact with feces-> migrating serpiginous erythematous tracks on teh skin
2. self limited, or ivermectin, thiabendazole, albendazole

Front 665

toxicara canis cati
1. pathogenesis + clinical presentation
2. tx

Back 665

1. pica children, ingestion of eggs --> generalized visceral larval migrans ~eosinophilia, leukocytosis, hepatomegaly, malaise, anemia, cough, myocarditis, OCULAR LARVA MIGRANS (retinal granulomas/endophthalmitis)
2. albendazole, mebendazole, steroids for ocular involvement

Front 666

rocky mountain spotted fever
1. pathogenesis + clinical presentation
2. dx/tx

Back 666

1.SOUTH EASTERN tick bite transmits rickettsia rickettsii gram negative intracellular coccobacillus -->
2. petechial rash BEGINS ON THE FLEXOR SURFACES OF EXTREMITIES AND MOVES CAUDAL AND CENTRIPETAL DIRECTION - includes the palms/soles
myalgias, hepatosplenomegaly/jaundice, CNS symptoms like headache, coma, seizure, hypotension
2. dx = thrombocytopenia, hyponatremia, elevated AST/ALT
tx = IV doxycycline and supportive care

Front 667

ehrlichia chaffeensis
1. pathogenesis + clinical presentation
2. tx

Back 667

1. tick bite --> same symptoms as rickettsia but no rash - fever, headache, myalgias, lymphadenopathy
2. dx = serology and PCR,
tx = doxycycline and supportive

Front 668

cat scratch disease
1. pathogenesis + clinical presentation
2. Parinauds oculoglandular syndrome
3. other complications
2. dxtx

Back 668

1. bartonella henselae -->initail scratch forms papule along line of scratch--> REGIONAL LYMPHADENOPAHTY (AXILLARY/CERVICAL/INGUINAL)~ nodes are warm tender, suppurative (10%), fever occurs in 1/3 of patients
2. conjunctivitis and PREAURICULAR LYMPHOADENITIS if the infection is in the eye.
3. osteomyelitis, encephalitis, hepatitis, pneumonia, hepatic/spleen lesions
4. IgM to b.henselae, azithromycin/TMPSMX/ciprofloxacin for systemic disease

Front 669

myocobacterium tuberculosis
1. pathogenesis + clinical presentation
2. cxr/dx

Back 669

1. inhalation of small airbone droplets --> fevers, chills weight loss, cough, night sweats, cervical lymphadenitis (most common extrapulmonary TB in children), meningitis, ileitis, bone involvmenet (potts diseaes), dissemination or miliary disease
2. hilar/mediastinal lymphadenopathy, ghon complex, pleural effusion/lobar involvement, cavitary disease, PPD test (will be + 2-12 weeks after exposure),
positive culture for M.tuberculosis, positive staining for acid fast bacilli,caseating granulomas from biopsy speciment

Front 670

mycobacterium tuberculosis
1. exposure vs latent vs disease.
2. epidemiology

Back 670

1. exposure = someone who has contact with pulmonary TB,
latent tuberculosis infection = asymptomatic with positve PPD, and +/- chest radiograph
disease = signs and symptoms with +/- positive chest radiograph
2. immigrants, homeless, institutions, correctional facilities, immunodeficiency conditions

Front 671

what age group is tuberculosis not contageous

Back 671

children <12 years old who do not cough much and have small pulmonary lesions

Front 672

PPD results when to call it a postive
size of induration (not erythema)
1. how long after exposure does it take for a PPD to turn positive
2. what pts is >5mm positive
3. what pts is >10mm positive
4. what pts is >15mm positive

Back 672

1. 2-12 weeks
2. children who have close contact with individuals, + chest radiograph, or immunocompromised
3. children <4 years of age, chronic medical conditions, or live in endemic, or work in institution, or healthcare
4. children older than 4 years with no risk factors

Front 673

treatment of m.tuberculosis

Back 673

latent tuberculossi - isoniazid for 9 months + B6 to prevent neurologic complications of INH

TB disease - 2 moths of INH rifampin/pyrazinamide then 4 months of INH and rifampin

Front 674

1. define CHF
2. three major physiologic responses that occur as a result of congestive heart failure

Back 674

1. inadequate oxygen delivery by the myocardium to meet metabolic demands o the body
2. hypoperfusion of end organs --> inc. contractility and heart rate
hypoperfusion --> kidneys retain salt annd water through RAAS
SNS --> inc catecholamins which increase heart rate/contractility

Front 675

which congenital heart diseases cause increased pulmonary flow

Back 675

ASD/VSD/PDA
transposition of the great arteries
truncus arteriosus, TAPVC

Front 676

what congenital heart diesease cause obstruction of flow

Back 676

aortic/pulmonic/mitral stenosis, coarctation of the aorta, interrupted aortic arch, hypoplastic left heart syndrome

Front 677

what is a common cause of acquired CHF in older children/adolescents

Back 677

viral myocarditis (coxsackie B)

Front 678

what kind of CHF caused by anemia

Back 678

high output CHF

Front 679

clincal presentation of CHF

Back 679

tachypnea, cough, wheezing, rales
CXR = pulmonary edema
tachycardia, sweating, pale or ashen skin, decreased urine output, enlarged cardiac silouette
FTT, poor feeding (infants), exercise intolerance

Front 680

clinical presentation of systemic venous congestion (right heart failure)

Back 680

hepatomegaly, peripheral edema

Front 681

treatment for CHF (3 drug classes + mechanisms

Back 681

*cardiac glycosides - increased efficeincy of myocardial contractions and dec. tachycardia
*loop diuretics - decreased intravascular volume, decreases afterload/ventricular dilation
*ionotropes - dobutamine/dopamine
phosphodiesterase inhibitors- amrinone, milrinone- inc. contractility

Front 682

what interventional strategies for heart failure in children/infants

Back 682

interventional catheterization (baloon valvuloplasty)
surgical repair

Front 683

what is an "innocent murmur"

Back 683

murmors from turbulent flow and NOT structural heart disease~~ seen in 50% of children

Front 684

stills murmur: age, location, characteristic

Back 684

INNOCENT MURMUR
AGE 2-7,
systolic vibratory/buzzing - found at the mid left sternal border, loudest when supine/exercise
grade 1-3

Front 685

pulmonic systolic murmur: age, location, characteristic

Back 685

INNOCENT MURMUR
any age
systolic blowing high pitched, found at upper left sternal border loudest when supine/exercise
grade 1-2

Front 686

venous hum: age, location, characteristic

Back 686

any age but usually school age

continuous murmor heard in the neck and below the clavicles, heard only when sitting or standing, disappears when supine, changes with compression of the jugular vein or with movment of the neck

Front 687

ASD ostium primum
1. features
2. what other heart defect associated
3. which genetic disease associtaed with

Back 687

1. lower portion of the atrial septum,
2. +/-cleft or deviation in the anterior mitral valve leaflet also causes mitral regurg (MCC congenital heart defect in downs syndrome
3. DOWNS SYNDROME~ most common congenital heart disease in these patients

Front 688

ASD ostium secundum (most common)
1. features

Back 688

1. MIDDLE PORTION OF THE ATRIAL SEPTUM

Front 689

sinus venosus
1. features

Back 689

1. defect high in the septum near right atrium/SVC junction, right pulmonary veins drain into the atrium/SVC instead of the left atrium

Front 690

1. physical exam findings of acyanotic congenital heart disease
2. complications

Back 690

1. *increased right ventricular mpulse
*systolic ejection murmur (through pulmonary artery) at the mid/upper left sternalborder
*fixed split S2 - due to excessive pulmonary blood flow
2. right heart failure, pulmonary hypertension, atrial dysrhythmias,

Front 691

treatment of acyanotic congenital heart disease

Back 691

open heart surgery

Front 692

ASD
1. physical exam findings
2. ECG fidnings
3. CXR

Back 692

1. systolic ejection murmor, mid-left sternal border and ULSB, fixed split S2, diastolic rumble,
2. RAD, RVH, RAE
3. right atrial/ventricular enlargment, inc. pulmnoary vasculature markings

Front 693

VSD
1. physical exam findings
2. ECG fidnings
3. CXR

Back 693

1. high pitched holosystolic murmur at LLSB, diastolic rumble due to inc. pulmonary flow
2. LVH +/- RVH if there is pulmonary hypertension
3. cardiomegaly, and increased pulmonary vascular markings

Front 694

PDA
1. physical exam findings
2. ECG fidnings
3. CXR

Back 694

1. continuous machinelike murmor at the uppler left sternal border with STRONG PERIPHERAL PULSES, WIDENED PULSE PRESSURE
2. LVH, RVH if pulmonary hypertension
3. cardiomegaly with increased PVM

Front 695

coarctation of the aorta
1. physical exam findings
2. ECG fidnings
3. CXR

Back 695

1. elevated BP in right arm, reduced BP in legs, delayed femoral pulses (brachiofemoral delay), left upper back bruit***~ also associated with bicuspid valve aortic stenosis***
2. normal or LVH
3. RIB NOTCHING (dilation of collateral chest wall vasculature), normal heart size

Front 696

aortic stenosis
1. physical exam findings
2. ECG fidnings
3. CXR

Back 696

1. ejection click, systolic ejeection murmor that radiates to URSB/apex/suprasternal notch/carotids, thrill at the URSB and suprasternal notch
2. noraml or LVH
3. nromal or mild cardiomegaly, prominent ascending aorta from post stenotic dilation

Front 697

pulmonary stenosis
1. physical exam findings
2. ECG fidnings
3. CXR

Back 697

1. ejection click, systolic ejection murmur at ULSB
2. RVH
3. prominant main pulmonary artery (post stenotic dilation)

Front 698

what are the four types of VSD

Back 698

inlet, trabecular, membranous, outlet ( supracristal)

Front 699

why does pulmonary hypertension occur in VSD

Back 699

incrased blood flow from left to right

Front 700

what two factors in VSD determine how much blood goes from left to right

Back 700

degree of pulmonary vascular hypertension
size of the VSD

Front 701

1. what is the relationship between the size of the VSD and the intensity of the murmur
2. what kind of murmur from a moderate size VSD
3.what kind of murmur + symptoms from a large VSD

Back 701

smaller size of VSD causes a more intense murmur
2. holosystolic, but because 2x the ammount of blood goes to lungs --> mitral valve there is also a diastolic murmur of mitral turbulence at the apex
3. shorter/lower pitch systolic murmur, with a mitral filling rumble at the apex ~~ present with signs of CHF

Front 702

how does S2, mitral filling rumble change in a VSD in which the PVR is beginning to increase

Back 702

S2 gets louder due to inc. pulmonary pressures
mitral filling rumble decreases because of diminished pulmonary blood flow

Front 703

is PVR reversable if VSD is closed + what happens if PVR exceeds SVR

Back 703

NO~~ if PVR>SVR there is shunt reversal and this is called EISENMENGER SYNDROME

Front 704

what patients with VSD should get surgical closure

Back 704

heart failure refractory to medical treatment
large VSD with pulmonary hypertension
small to moderate VSDs (at age 2-6)

Front 705

what group of infants have a high incidence of PDA

Back 705

PRETERM

Front 706

symptoms of large PDA

Back 706

CHF due to increased pulmonary blood flow

Front 707

treatment of PDA

Back 707

indomethacin

Front 708

how does blood get to the body of an infant with severe coarctation of the aorta

Back 708

blood from the pulmonary artery goes past the coarctation through the ductus arteriosus (this is a right to left shunt)

Front 709

what is the clinical presentation of a patient with a severe coarctation

Back 709

normal appearing infant that develops cyanosis and CHF symptoms as the PDA closes -->weak pulses in all extremities, and loss of the murmur

Front 710

what other heart anomaly is associated with coarctation of the aorta

Back 710

bicuspid aortic valve***

Front 711

treatment for coarctation of the aorta

Back 711

prostaglandin E keeps the PDA open, inotropic medications keep cardiac output sufficeint to perfuse the kidneys, surgery, balloon angioplasty for recurrent COARCTATION IS COMMON************ IN THESE PTS)

Front 712

clinical presentation of sever aortic stenosis in neonate and older children

Back 712

neonate: normal appearing neonate at birth becomes cyanotic at 12-24 hours and develops CHF as the ductus arteriosus closes
older children: exercise intolerance, chest pain, syncope, sudden death

Front 713

treatment for aortic stenosis

Back 713

balloon valuloplasty
palliative valvuloplasty, aortic valve replacement(using pts own pulmonary valve = Ross procedure) or using prosthetic valve

Front 714

pathogenesis + clinical presentation of severe pulmonary stenosis

Back 714

inc. PVR ==> shunting of blood through the patent forament ovale leading to right to left shunt and cyanosis

Front 715

what is the cause of peripheral "acrocyanosis"

Back 715

peripheral vasocontstriction from cold temperature

Front 716

what are the causes of central cyanosis (non cardiac and cardiac)

Back 716

non cardiac: pulmonary, sepsis, hypoglycemia, polycythemia, neuromuscular disease, impaired chest wall
cardiac: the 5Ts

Front 717

what is the diagnostic test to test for suspected congenital heart disease

Back 717

100% oxygen challenge test- PaO2 does not rise following administration of 100% oxygen
~~~ then do an echocardiogram

Front 718

tetralogy of fallot
1. physical examination
2. ECG
3. CXR
4. 4 anatomic components +
5. mechanism of blood flow
6. tx

Back 718

1. systolic ejection murmur of pulmonary stenosis*, increased right ventricular impulse(RVH)
2. RVH
3. boot shaped heart, decreaed pulmonary vascular markings, right aortic arch
4. VSD, overriding aorta, pulmonary stenosis, RVH~~~
5. Mechanism: pulmonary outflow track obstruction causes a right to left shunting of blood across the VSD
6. squatting position,
IV fluid bolus,
oxygen,
sedation (dec. agitation),
beta blocker (dec. contractility / facilitate pulmonary flow
sodium HCO3- (tx. acidemia)
transfusion

Front 719

transposition of the great arteries
1. physical examination
2. ECG
3. CXR
4. pathophysiology
5. tx

Back 719

1. NO MURMUR (quiet precordium), single S2 (no split)
2. normal or RVH
3. small heart with narrow mediastinum**** (egg on a string), increased pulmonary vascular markings
4. systemic and pulmonary circuits are in parallel rather than series~~ OBLIGATE patent foramne ovale, ASD or VSD (determines the degree of cyanosis
5. arterial switch operation

Front 720

tricusipid atresia
1. physical examination (w and w/o VSD)
2. ECG
3. CXR
4.mechanism
5. when is tricuspid atresia PDA and not PDA dependent

Back 720

1. single S2 (only one valve) if no VSD, systolic murmur of VSD if present, NO murmur if VSD is not present
2. LAD***, LVH***, RAE,
3. small heart, decreased pulmonary vascular markings
4. plate of tissue where the tricuspid valve should be with an obligate ASD or PFO~~ blood flows from the right atrium to the left atrium to left ventricle to aorta/pulmonary artery through VSD
5. if VSD is not present, this is a PDA dependent lesion, if VSD is present this lesion does not require PDA

Front 721

truncus arteriosus
1. physical examination
2. ECG
3. CXR
4. tx

Back 721

1. single S2, systolic ejection murmor @ left sternal border (inc. flow across truncal valve), diastolic murmor at the apex (from excess flow across mitral valve)
2. combined ventricular hypertrophy
3. enlarged heart, increased pulmonary vascular markings, right aortic arch
4. surgical repair

Front 722

TAPVR
1. physical examination
2. ECG
3. CXR
4. pathophysiology
5. tx

Back 722

1. pulmonary ejection murmur (inreased pulmonary flow) along left sternal border
2. RVH/RAE,
3. enlarged heart with supracardiac drainage, snowman appearance,
if there is obstruction small heart, and pulmonary edema
4. systemic and pumlmonary blood mixes in the right atrium becuase pulmonary veins drain into the systemic circulation
5. pulmonary veins surgically put onto the left atrium

Front 723

what are the congenital heart diseases that cuase decreased pulmonary flow

Back 723

TOF
pulmonary atresia
tricuspid atresia

Front 724

1. what determines the severity of TOF
2. what actions increase right to left shunting in TOF
3. what actions increase flow through the RVOT in TOF

Back 724

1. degree of RVOT obstruction (pulmonary stenosis)
2. dec SVR = exercise, vasodilation, volume depletion
inc. RVOT resistance = crying, tachycaria
3. inc. SVR = volume infusion, systemic hypertension, valsalva, bradycardia

Front 725

1. when after birth does the onset of cyanosis occur in TOF
2. treatment for tetralogy of fallot

Back 725

1. at birth as soon as the PDA closes~ now blood cannot flow through the PDA to the lungs to be oxygenated (left to right shunt)
2. surgical repair at 4-8 months, modified blalock-taussig shunt (gore tex graft between subclavian and pulmonary artery)

Front 726

1. what is a tet spell
2. how to compensate during tet spell

Back 726

1. trigger* such as crying (inc. RVOT resistance) --> dec. oxygenation of blood --> sudden cyanosis and decreased murmur intensity --> altered consciousness + hyperpnea
2. squatting in the knee/chest position to increase venous return to the heart and increase SVR

Front 727

treatment for tricuspid atresia procedure

Back 727

fontan procedure- flow from the inferior vena cava is directed into the pulmonary arteries by means of extracardiac conduit Glenn shunt - SVC anastomosed is placed
**goal is systemic venous return to the pulmonary artery

Front 728

truncus arteriosus pathophysiology

Back 728

excessive blood flow to the lungs leads to CHF

Front 729

most common acquired hear diesease in children in the UNITED STATES VS WORLD

Back 729

United states = kawasaki
world = rheumatic fever

Front 730

endocarditis
1. underlying conditions
2. infectious organisms
3 pathogensis

Back 730

1. structural abnormalities, and post surgery (50%)
2. alpha hemolytic strep viridans, and staphylococcus are most common bacterial ~~~ also gram negatives (rare) and fungal(rare)
3. bacteria introduced into the blood and infect cardiac endothelium --> fibrin/platelets adhere forming a vegetation --> DISTAL MANIFESTATIONS

Front 731

1. diagnosis of infectious endocarditis
2. treatment
3. what pts need prophylaxis for infectious endocarditis

Back 731

1. blood culture 3x, ESR (usually elevated) unless there is polycythemia, rheumatoid factor,echocardiography identifies vegetations
2. intravenous antimicrobial therapy 4-6 weeks
3. all structural disease except secundum ASD, post operative cardiac surgery indefinatly if hemodynamic residua remain (otherwise stop at 6 months)

Front 732

clinical features of infectious endocarditis

Back 732

fever, non-specicifc
new murmur
hematuria (embolism or glomerulonephritis)
splinter hemorrhages
retinal hemorrhages
osler nodes (swollen tender lesions on palms/soles)
janeway lesions - small hemorrhageic lesions on the palms/soles
roths spots - round or oval white spots on the retina

Front 733

pericarditis
1 casues
2. viral causes
3. bacterial causes (purulent) + complications
4. pathophysiology

Back 733

1. infection, collagen vascular disease, uremia, postpericardiotomy syndrome (autoimmune reaction after the pericardium has been opened [+viral])
2. MOST COMMON CAUSE *** coxsackie, echo, adeno, influenza, parainfluenza, EBV
3. staph aureus, and strep pneumoiae --> constrictive pericarditis**
4. inflammation of hte pericardium -->exudate/transudate-->impaired venous return/cardiac filling --> CARDIAC TAMONADE

Front 734

1. Clinical presentation of pericarditis
2. physical exam findings
3. diagnosis
4. ECG findings
5. CXR findings
6. tx

Back 734

fever, dyspnea,
CHEST PAIN MOST INTENSE WHILE SUPINE,RELIEVED WHILE UPRIGHT
2. pericardial friction rub, distant heart sounds, PULSUS PARADOXUS, hepatomegaly
3. any child with fever/dyspnea + recent history of heart surgery, dx with PERICARDIOCENTESIS, ESR, echocardiogram
4. diffuse ST elevation and low voltage QRS complexes
5. large heart shadow
6. antibiotics, anti inflammatory ages such as aspirin or steroids, drainage

Front 735

myocardidits (inflammation of the myocardium itself)
** major cause of sudden death in athletes
1. etiologies
2. clnical presentation
3. dx
4. tx

Back 735

1. coxsachie, c.diphtheria, s.pyogenes, s.aureus, m.tuberculosis, candida, cryptococcus, t.cruzi (chagas*), SLE, rheumatic fever, sarcoidosis, kawasaki disease
2. viral or flu like illness --> dyspnea and malaise ~~ resting tachycardia, muffled heart sounds, gallop heart rhythum, tachypnea, hepatomegaly, pulmonary rales
3. ESR/CRP, CK-MB, ECG (T/ST wave changes), echocardiogram shows global ventricular dysfunction, endomyocardial biopsy
4. ionotropes, diuretics, IVIG, cardiac transplant~~ mortality 10-20%

Front 736

dilated cardiomyopathy
1. causes
2. clinical presentation
3. dx/tx

Back 736

1. viral (coxsackie B + same as myocarditis), mitochondrial abnormalities, carnitine deficiency, nutritional deficiency in selenium and thiamine, hypocalcemia, anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) ,doxorubicin, alcohol
2. CHF signs and symptoms
3. viral serologies, serum carnitine, ECG (low voltage + ST/T wave changes), evidence of infarction, echocardiogram (shows dilated left ventricle)
tx = manage CHF/nutritional deficiencies, repair of ALCAPA, cardiac transplant

Front 737

hypertropic cardiomyopaty
1. pathognesis
2. what infants have transient septal hypertrophy
3. clinical presentaiton
4. what manuevers increase the murmur of HCM

Back 737

1. AUTOSOMAL DOMINANT LVH, specifically asymmetric septal hypertrophy (idiopathic hypertrophic subaortic stenosis) w/o cardiac disease --> poor left ventricular filling + dyanmic left ventricular outflow tract obstruction --> myocardial ischemia
2. infants born to diabetic mothers
3. sudden cardiac death (MCC in athletes), syncope, chest pain, exercise, harsh SYSTLIC EJECTION MURMUR AT THE APEX
4. valsalva and standing these reduce LV volume and increase the outflow tract obstruction

Front 738

dynamic LVOT obstruction in hypertrophic cardiomyopathy mechanism

Back 738

1.. anterior mitral leaflet is swept into the subaortic region during systole

Front 739

dx. hypertrophic cardiomyopathy
tx. hypertrophic cardiomyopahty

Back 739

1. ECG: LVH, ST/T wave changes, LAD, deep and wide Q waves, in the inferior and lateral leads, Echocardiogram shows hypertrophy
2. B blockers, CCBs decrease LVOT obstruction
surgical myomectomy
antiarrythmics (to prevent ventricular arrythmias)
dual chamber pacing

Front 740

restrictive cardiomyopathy
1. causes
2. clinical presentation
3. treatment

Back 740

1. amyloid, fabrys, gauchers, hemosiderosis, hemochromatosis
2. exercise inolerance, weakness, dyspnea + edema, hepatomeegaly, elevated CENTRAL VENOUS PRESSURE
3. diretics, beta blockers, CCBs

Front 741

supraventricular tachycardia
1. AV re-entrant tachycardia
2. AV node* re-entrant tachycardia
3. wolff parkinson white syndrome

Back 741

1. retrograde conduction through accessory pathway leads to SVT
2. circuit moment in the AV node
3. bypass tract through the atria and ventricles --> sudden death

Front 742

clinical presentation of SVT
dx of SVT
tx of SVT

Back 742

1. palpitations, chest pain, dyspnea, altered level of consciousness, CHF symptoms (esp. in neonates)
2. ECG, will see delta wave for WPW (slurredupstroke of QRS) with short PR interval
3. vagal manuevars (valsalva), icepack on face*, carotid massage, invert the child,orbital pressure
IV adenosine
synchornized cardioversion
digoxin/propranolol
radiofrequency catheter ablation to destroy accessory paths

Front 743

heart block
1. fist degree
2. second degree
3. third degree
4. causes of heart block

Back 743

1. prolonged PR interval
2. type 1 = wenckeback- progressive prolonged PR ==> dropped beats
type 2 = failed AV conduction without progressive PR prolongation
3. complete block -- no conduction between the atria and ventricles
4. SLE mother, post surgical AV block (especially after closing VSD), bacterial endocaridits

Front 744

what mother condition leads to a neonate with third degree heart block

Back 744

SLE

Front 745

long QT syndrome
1. what is the major risk factor with this disease
2. causes
3. drug causes
4. clinical presentation

Back 745

1. progression to torsade de pointes
2. 50% AR Jervel lange nielsen* syndrome associated with congential deafness, or Romano Ward* not associated with congenital deafness, idipathic
3. phenothyazines, tricyclics, erythromycin, terfenadine
4. syncope, seizure, palpitations, sudden cardiac arrest

Front 746

long QT
1. diagnosis
2. treatment

Back 746

1. QTC>0.44 seconds
2. beta blockers, cardiac pacing, left stellate ganglionectomy, automatic implantable defibrillator

Front 747

page 252 has whole ddx for chest pain

Back 747

...

Front 748

non-cardiac causes of chest pain

Back 748

asthma, esophagitis, costochondritis, anxiety, pneumothroax, pneumonia, sickle cell chest syndrome peptic ulcer

Front 749

do WPW, kawasaki disease, and cardiac dyarrythmias need prophylaxis for infectious endocarditis

Back 749

NO

~<6months status post heart sturgery, all congneital heart defects except secundum ASD

Front 750

development of the pulmonary system
1. when does bronchial tree develop by
2. when are the air sacs/pulmonary vasculature developed enough to support life
3. when does 90% of alveolar development occur

Back 750

1. by 16 weeks
2. 26-28 weeks
3. after birth

Front 751

1. obstructive lung defects
2. restrictive lung defects

Back 751

1. decreased airflow - asthma, bronchiolitis, foreign bodies, COPD
2. decreased ability to inc. lung volume - pulmonary edema, scoliosis, pulmonary fibrosis, respiratory muscle weakness

Front 752

1. inspiratory stridor
2. expiratory wheezing
3. crackles or rales

Back 752

1. extrathoracic obstruuction - ex. croup, laryngomalacia
2. intrathroactic obstruction ex. asthma/bronchiolitis
3. parenchymal disease ex. pneumonia/pulmonary edema

Front 753

what blood gas values does oxygenation vs ventilation refer to***

Back 753

oxygenation = SaO2
ventilation = PCO2

Front 754

what does pulse oximetry measure

Back 754

oxygen saturation SaO2

Front 755

epiglottitis
1. pathogenesis + clinical presetation
2. lab findings + CXR
3. tx

Back 755

1. HITB, group A Beta hemolytic strep, s.pneumo, staph ---> inflammation/edema of the epiglottis/aryenoids, aryepiglottic folds --> high fever, muffled speech, dysphagia/drooling, tripod positioning, complete airway obstruction/respiratory arrest, CHERRY red epiglottis (seen during intubation)
2. left shift leukocytosis, blood culture, CXR appears like thumbprint on lateral neck radiograph
3. EMERGENCY, nasotracheal intubation, minimize stimulation, RIFAMPIN for HITB, 3rd gen cepahlosporins

Front 756

what other conditions can present like epiglottitis

Back 756

croup, bacterial tracheitits, retropharyngeal abscess

Front 757

supraglottic disorders extrathoracic
1. what are they
2. describe the assc: stridor/cough/voice/dysphagia+drooling/fever/toxicity/posture

Back 757

1. epiglottitis, retropharyngeal abscess
2. quiet/none/muffled/present/high fever/toxic apperarance/neck extended+tripod position

Front 758

subglottic disorders
1. what are they
2. describe the assc: stridor/cough/voice/dysphagia+drooling/fever/toxicity/posture

Back 758

1. croup and tracheitis
2. loud/barking/hoarse voice/no dysphagia/drooling/low fever in croup, high fever in tracheitis/toxicity only with tracheitis/normal posture

Front 759

laryngotracheobronchitis (croup)
1. definition
2. pathogenesis + clinical pres
3. CXR
4. age group effected + time of year

Back 759

1. inflmmation/edema of the SUBGLOTTIC larynx, trachea, bronchi
2. Parainfluenza (MCC), RSV, rhinovirus, adeno, influenza A/B, myocoplasma -->URI --> inspiratory stridor (worse at night), barking cough/hoarse voice, lasts 3-7 days,
3. AP film shows STEEPLE SIGN OF SUBGLOTTIC NARROWING
4. age 3 months = 3 months to 3 years fall and winter

Front 760

spasmodic croup

Back 760

acute onset of stridor croup at night, resolves without treatment

Front 761

laryngotracheobronchitis (croup)
1. treatment

Back 761

1. COOL NIGHT AIR/MIST **,
systemic corticosteroids for stridor,
epinephrine aerosols (vasoconstricts subglottic tissues),
Beta agonists for wheezing
hospitalization

Front 762

bacterial tracheitis
1. pathogenesis + clinical pres

Back 762

1. staph aureus (MCC), streptococcus, NTHI --> ACUTE infalmmmation fo the trachea--> toxicity, high fever, mucous and pus in the trachea

Front 763

bronhiolitis
1. pathogenesis
2. CXR
3. complications
4. tx

Back 763

1. RSV (MCC), parainfluenza, adeno, rhino, infleunza, mycoplasma --> URI (fever/congestion/cough) --> LRI: inflammation of the bronchiols/bronchiolar obstruction - tachypnea, fine rales, wheezing, palpable SPLEEN AND LIVER (due to lung hyperinflation), hypoxemia, apnea
2. hyperinflation with air trapping, patchy infiltrates , atelectasis
3. APNEA, respiratory failrure/death, bacterial superinfection
4. suctioning, hydration, nebulized bronchodilators/epinephrine, ribovirin, steroids, PALIVIZUMAB - RSV monoclonal antibody given prophylactically to pts. with risk factors

Front 764

1. most common lower respiratory tract infecction in the first two years of life
2. risk factors for bronchiolitis

Back 764

1. bronchiolitis
2. day care, siblings, tabacco smoke exposure, lack of breast feeding, chronic lung disease, prematurity, immunodeficiency

Front 765

Pneumonia
1. etiology 0-3 months
2. 3 months to 5 years
3. >6 years

Back 765

1. TORCH, GBS, gram negative rods, listeria, RSV chlamydia, ureaplasma, mycoplasma, PCP
2. adeno, influenza a/b, parainfluenza, RSV, s.pneumo, s.aureus, HITB
3. adeno, influenza a/b, parainfluenza, s.pneumo

Front 766

pneumonia
1. clinical presentation
2. dx + CXR

Back 766

1. URI sx (nasal congest/rhinorrhea/fever/cough) --> tachypnea, wheezing, rales, respiratory distress
2.WBC <20k with lymphocyte predominance
CXR = interstitial infiltrates

Front 767

chlamydia trochomatis* pneumonia
1. clinical presenation
2. dx +CXR
3. tx

Back 767

1. AFEBRILE*** PNEUMONIA in chldren age 1-3 months of age STACCATO TYPE COUGH, dyspnea, tachypnea, wheezing, history of conjunctivitis
2. EOSINOPHILIA****
CXR = interstitial infiltrates
3. erythromcyin or azithromycin

Front 768

myocoplasma pnemoniae pneumonia
1. clinical presentation
2. dx + CXR
3. tx

Back 768

1. low grade fever, chills, non-productive cough, pharyngitis, malaise, headache, widespread rales on physical exam **EXAMINATION FINDINGS ARE WORSE THAN THE HISTORY
2. positive COLD AGGLUTININS, serum IgM titers for mycoplasma
CXR shows bilateral diffuse infiltrates
3. erythromycin or azithromycin

Front 769

pertussis
1. pathogenesis + clinical presentation
2.dx -tx

Back 769

1. usually UNVACCINATED --> bordetella pertussis exposre --> 10 day incubation--> CATARRHAL STAGE (2 wks ) URI, rhinorrhea, nasal congestion, low grade fever --> PAROXYSMAL STAGE (4 wks) cough + whoop+ post tussive emesis, cyanosis, apndea, choking--> CONVALESCENT PHASE recovery phase and cough becomes less frequent
2. clinical features + CULTURE ON REGAN LOWE OR BORDET GENGOU MEDIUM, LYMPHOCYTOSI, fluroescent antibody tests
tx = azithro/erythro, respiratory isolatoin

Front 770

asthma
1. pathogenesis + clinical presentation
2. what fraction of asthma patients are in remission by puberty
3. dx + CXR

Back 770

1. 95% PRESENT BY 5 YEARS OF AGE chronic inflammatory --> bronchiolar smooth muscle constriction, airway mucosal edema, mucous plugging, inflammatory mediator production + HYPERRESPONSIVENESS TO STIMULI --> recurrent wheezing, cough, dyspnea, chest tightness ** symptoms are reversible spontaneously or with treatment,
2. 35%
3. therapeutic bronchodilator trial
CXR shows hyperinflation, peribronchial thickening, , PFTs show dec. expiratory flow

Front 771

1. what are the predisposing risk factors for asthma
2. what are the asthma "triggers"

Back 771

1. atopy, family history of asthma, tobacco smoke exposure, infection, diet, pollution
2. respiratory infections, cold air, emotions, allergens, GI reflux, pollutants

Front 772

acute wheezing ddx pg 269
asthma severity/management chart

Back 772

...

Front 773

asthma
1. assessment of disease
2.tx

Back 773

1. peak expiratory flow rates, and keeping journal of the values
2. AVOID TRIGGERS, beta adrenergic agonists, systemic corticosteroids, cromyln/nedocromil sodium (prophylaxis), anticholinergics (atropine/ipratropium), leukotrienne modifiers (montelukast/zafirleukast), methylxanthines (theophylline)

Front 774

cystic fibrosis
1. pathogenesis
2. clinical presentation
3. pulmonary function test findings

Back 774

1. AR CFTR mutation (del DF508 chromosome 7) --> sodium/choloride transport dysfunction--> abnormal mucus production
2. progressive pulmonary insufficiency, pancreatic insufficinecy, high sweat electrolites, meconium illeus at birth, steatorrhea, FTT, chronic cough, dyspnea, lung hyperinflation, crackles, wheezing, digital clubbing/hypoxemia
3. decreased respiratory flow rates , decreased lung volume s(LATE)

Front 775

cystic fibrosis
1. frequency of disease/carrier
2. length of survival

Back 775

1. 1/2500 caucasians effected, 1/20 carrier in caucasians
2. average 31 years

Front 776

cystic fibrosis
1. sinopulmonary disease (infectious agents+clinical presentation)
2. GI disease
3. nutritional deficiencies
4. metabolic abnormalities
5, reproductive abnormalities

Back 776

1. staph aureus, pseudomonas, burkholderia cepacia ~~ chronic cough/wheeze/sputum, nasal polys/sinusitis, bronchiectasis, hemoptysis pulmoary infiltrates, hyperinfaltion
2. meconium ileus, pancreatic insufficinecy/pancreatitis, chronic hepatits, rectal prolapse
3. hypoproteinemia, edema, ADEK deficinecies
4. hyponatremia, hypochloremia, hypokalemic metaboic alkalosis
5. obstructive azoospermia
`

Front 777

cystic fibrosis
1. dx/tx

Back 777

1. >1 phenotypic features, increased immunoreactive trypsinogen on newborn screen, sweat chloride test (>60mmol/L), CF mutations, nasal epithelium ion transport abnormalitiy
TX = antibiotics, bronchodilators, ADEK supplementatoin, oxygen, anti-inflammatory therapy, lung transplant, psychological support

Front 778

chronic lung disease (bronchopulmonary dysplasia)
1. causes
2. pathogenesis + clinical presentation

Back 778

1. *** RDS in premature infants (MCC) acute lung injury(mechanical ventilation, meconium aspiration, infection), secondary lung injury (oxidants/proteases) follows acute lung injury
2. lung damage (all of the above) --> abnormal healing/parenchymal remodeling --> diminished oxygenation, hypercarbia, intermittent episodes of tachypnea/weakness, frequent respiratory tract infections-- BOTH OBSTRUCTIVE AND RESTRICTIVE LUNG DISEASE

Front 779

chronic lung disease
1. CXR
2. tx

Back 779

1. hyperinflation, atelectasiss, linear/cystic chest densities
2. supplemental oxygen, bronchodilators, caloric intake,

Front 780

1. what age does aspiration occur at highest frequency
2. clinical presentation of laryngotracheal foreign bodies
3. bronchial foreign bodies clinical presentation
4. is CXR diagnostic for aspiration

Back 780

1. 3months - 5 years
2. stridor, cough, hoareness
3. asymmetric auscultation findings, partial ball valve, wheezing, persistent pneumonia
4. only 15% of the time

Front 781

1. apnea of infancy
2. short central apnea
3. two general causes of apnea
4. causes of obstructive apnea

Back 781

1. unexplained cessation of breathing >20sec
2. <15seconds of apnea normal at all ages
3. central (no respiratory effort), or obstructive where there is respiratory effort
4. craniofacial abnromalities

Front 782

apnea or prematurity

Back 782

cessation of breathing > 20seconds in premature infant as a result of immature respiratory center control

Front 783

periodic breathing

Back 783

three or more respiratory pauses at least 3 seconds each with less than 20 seconds in between

Front 784

1. SIDS
2. risk factors + age of SIDS

Back 784

1. unexplained death in child <1 year of age
2. age 2-4 months ----- prone sleeping, soft bedding, over bundling, prematurity, low birth weight, recent illness, no breastfeeding, maternal smoking/drug abuse, infection

Front 785

most common cause of pneuonia in older children/adolsecents

Back 785

chlamidia pneumoniae and mycoplasma pneumoniae

Front 786

1. what is the most common cause of cardiac arrest in a child
2. what should be done for a child in cardiac arrest

Back 786

1. lack of oxygen supply -- choking, suffocation, near drowning, lung disease etc
2. CPR

Front 787

ABCs of CPR

Back 787

airway
breathing
circulation

Front 788

what is the most common cause of airway obstruction + how to fix this in a patient during CPR

Back 788

the tongue-- fix using the head tilt method, or the jaw thrust method if suspect cervical spine injury

Front 789

1. how is breathing evalulated during CPR
2. what to do if the patient isnt breathing

Back 789

1. look listen, feel method = look for chest movement, listen for exhaled air, feel for exhaled airflow
2. rescue breathing

Front 790

1. when should chest compressions begin
2. where is pulse assessed in an infant vs a child

Back 790

1. if need is determined after two rescue breaths~~~ if the patient is in ASYSTOLE or BRADYCARDIA
2. brachial artery of infants, carotid artery of children

Front 791

define shock

Back 791

1. inadequate delivery of oxygen/metabolic substrates to meet metabolic demands of tissue +/- decreased blood pressure

Front 792

1. compensated shock
2. decompensated shock
3. irreversible shock

Back 792

1. normal bloo dpressure, adequate tissue perfusion but maldistribution to essential organs
2. hypotension, low cardiac output, inadequate tissue perfusion
3. cell death refrractory to medical treatment

Front 793

1. hypovolemic shock
2. what % of volume loss leads to inability of endogenous catecholamines to maintain blood pressure

Back 793

dec. circulating volume ex. hemorrhage/dehydration
2. 25% ~~ now called decompensated shock

Front 794

septic shock
1. pathogenesis
2. two stages

Back 794

1. infalmmatory response to an organism
2. hyperdynamic stage- bounding pulses*, normal or high cardiac output, warm extremities, wide pulse pressure
decompnesated stage - impaired mental status, cool extremities, diminished pulses

Front 795

1. distributive shock
2. anaphylactic shock
3. neurogenic shock

Back 795

1. distal pooling of blood/fluid extravasation-- caused by anaphylaxis, neurogenic, meds, toxins
2. acute angioedema of upper airway, bronchospasm, pulmonary edema, urticaria, hypotension (from extravasation)
3. 2' to spinal cord transection-- loss of sympathetic cardiovascular tone --> hypotension from pooling of blood in the vascular bed

Front 796

cardiogenic shock

Back 796

1. CO is limited due to cardiac dysfunction: dysrhytmias, congenital heart disease, cardiac dysfunction after surgery ~~ presents as CHF

Front 797

1. physical exam findings of shock
2. historical features of shock

Back 797

1. +/- abnormal blood pressure (normal early), tachycardia**, tachypnea**, altered metnal status, capillary refil is slow, cool/mottled* extremities, bounding peripheral pulses (early)
2.vomiting/diarrhea, trauma/hemorrhage, febrile illness*, CHF sx, exposure to allergen, spine injury

Front 798

treatment of shock

Back 798

supplemental oxygen
early endotracheal intubation
20ml/kg fluid resuscitation with normal saline or ringers lactate
innotropes (dobutamine) after intravascular volume is restored
broad spectrum abx (septic shock)
broad pectrum ABX, blood products

Front 799

1. what is the most common cause of death in children >1 year
2. why are injuries more common in a child

Back 799

1. motor vehicle accidents ~ trauma
2. becaue head is larger % of total body mass in a child , shorter neck w/ greater weight
pliable rib cage leads to more spleen/liver,ligaments stronger than growth plates --> growth plate injuries

Front 800

1. what is the primary survey* when a child comes to the emergency room
2. what else is done in the emergency room when a child first arrives

Back 800

ABCDE
airway,
breathing and ventilation (on 100%
O2),
circulation (control of hemorrhage)
disability (GCS),
exposure/environmental (undressed for examine then warmed to prevent hypothermia)
2. ECG monitor (dysarrythmias= cardiac injury), pulseless activity (tamponade, pneumonthorax, hypovolemia
urinary catheter/NG tube
Radiographs: CXR, cervical spine, pelvis, CT,

Front 801

GCS
Eye movement scale

Back 801

1- no response
2- response to pain
3- response to voice
4. - spontaneous movement

Front 802

GCS
best motor response

Back 802

1. no response
2. decerebrate (abnormal extension)
3. decorticate (abnormal flexion)
4. flexion withdrawl
5. localizes pain
6. obeys command

Front 803

GCS
best verbal response

Back 803

1. no response
2. incomprehensible (grunts for non-verbal child)
3. inappropriate words (crys/screams inappropriately)
4. cries
5. cries normally, smiles, coos

Front 804

what is the secondary survey in pediatric emergency

Back 804

full history and physical and head to toe examinations

Front 805

head trauma
1. what kind of response is common after head trauma
2. why are infants more tolerable of intracranial masses/hemorrhage

Back 805

1. seizure
2. they have open fontanells and cranial sutures which allows blood to accumulate in sub galeal (between the scalp and galea aponeurosa) and epidural spaces

Front 806

1. epidural hematoma
2. dx
3. tx

Back 806

1. bleeding between inner table of the skull and the dura --- due to tearing of the MIDDLE MENINGEAL ARTERY
2. CT shows LENS SHAPED DENSITY***
3. immediate drainage

Front 807

1. subdural hematoma
2. dx
3. tx

Back 807

1. tearing of the BRIDGING MENINGEAL VEINS --> blood beneath the dura, more common than epidural bleed
2. head CT shows CRESCENT shaped density in subdural space that crosses suture lines
3. surgical drainage

Front 808

1. intracerebral hematoma

Back 808

1. bleeding in the brain parnechyma ~ frontal/temporal lobes OPPOSITE (contracoup) to the side of injury

Front 809

increased ICP
1. clinical presentation
2. complications*
3. tx

Back 809

1. HEADACHE (EARLY FIRST SIGN), PUPILLARY CHANGE, ALTERED MENTAL STATUS
2. transtentorial or uncal herniation into the infratentorial compartment --> bradycaria, blown pupil, contralateral hemiparesis, cushings triad (bradycardia, hypertension, irrecgular breathing)
3. hyperventilation with 100% oxygen, elevation of the head to 30-45 degrees to help with venous drainage
diuretics
neurosurgery

Front 810

symptoms ~~~ what are the signs
1. headache
2. vomitting
3. stiff neck
4. double visison
5. transient loss of vision
6. episodic severe headache
7. gait disturbance
8. dulled intellect
9. irritability

Back 810

1. papilledema
2. cranial nerve palsies
3. stiff neck
4. head tilt
5. retinal hemorrhage
6. macewen's sign (skull percussion hyperresonsnace)
7. obtundation
8. unconsiousness
9. progressive hemiparesis

Front 811

what make spinal cord injury difficult to diagnose

Back 811

spinal cord injury without radiographic abnormality

Front 812

1. why are children more prone to chest trauma
2. tension pneumothorax clinical presentation
3. tx for tension pneumothorax

Back 812

1. their chest is soft and pliable
2. decreased breath sounds, hyperresonance to percussion, displaced trachea, distended neck veins
3. rapid chest decompression by needle thoracotomy

Front 813

duodenal hematoma

Back 813

1. BICYCLE HANDLEBAR INJURY --> right upper quadrant injury --> abdominal pain, vomiting, bowel obstruction on radiographic evaluation

Front 814

lap belt injury

Back 814

from MVA
chance fracture (flexion disruption of lumbar spine)
liver/spleen laceration, kidney damage
bowel perforation

Front 815

burns
1. scalding injury
**consider child abuse

Back 815

1. from hot liquids

Front 816

1. first degree burn
2. second degree burn (two subtypes)
3. third degree burn

Back 816

1. epidermis only, red blanching, painful skin, HEALS WITHOUT SCARRING (ex. sunburn)
2. entire epidermis and part of the dermis
A. superficial partial thickness = outer dermis-- blister but no scar
deep partial thickness -= entire epidermis and lower dermis pale white may blister, heal with scarring
3. full thickness burn: epidermis, dermis, subcutaneous tissue, DRY WHITE LEATHERY, skin grafts are needed, INSENSITIVE TO PAIN

Front 817

burn surface area (BSA)

Back 817

Lund-browder classifcation
arms = 9%
legs = 18%
anterior trunk = 18%
posterior trunk = 18%
head and neck = 9%

Or can estimate burn area with palm size ~ one childs palm = 1% of body area

Front 818

1. emergency treatment of burns on presentation
2. fist degree burn treamtnet
3. second degree burn treatment
4. third degree burn treatment

Back 818

1. ABCs- endotracheal tubation for suspected inhaled hot gases, 100% oxygen/pulse OX, intravenous access + fluid ressusitation (due to rapid loss of lost through burned skin)
2. moisturizer/analgesics
3. analgesiscs, debridement of dead skin and popped bullae, dont pop unruptured bullae.
4. skin graft, hydrotherpy, escharotomy (surgical removal of constricting scar tissue)

Front 819

when does a patient with burn need hospitalization

Back 819

1. partial thickness burns > 10% BSA
full thickness burns >2% BSA
inhalation
perineum, hands, feet, burn overylying joints
non accidental trauma

Front 820

near drowning
1. definition
2. what classic causes

Back 820

1. survival after asphyxia while submerged in a liquid
2. child falls in pool or water containers (toilet/bucket), adolescents assocated with drugs/alcohol ingestion

Front 821

near drowning
1. mechanism (2)

Back 821

1. wet drowning-asphyxia from aspirating liquid
dry drowning - laryngospasm
--> denaturing surfactant, alveolar instability/collapse, pulmonary edema--> decreased compliance, increased resistance, increased pulmnoary artery pressure, decreased gas exchange

Front 822

1. clinical presentation of near drowning
2. what determines the degree of neurological defecit in a near drowning
3. hematologic sequelae of near drowing

~ renal failure can also occur

Back 822

1. absent/irregular respirations, cough up pink/frothy material, rales/rhonchi/wheezes
**pneumonia often develops within 24 hours from normal flora
slow deterioration of pulmonary function
2. length/severtiy of hypoxia, pt. may be alert, agitative, combative, or comatose
3. hemolysis/DIC

Front 823

treatment of a patient who has nearly drowned

Back 823

ABCs
cervical spine immobilization, remove wet clothing
intubation/mechanical ventilation with positive end expiratory pressures for respiratory failure
rewarming of body core (warm saline lavage, gastric lavage,

Front 824

1. which are risk factors for a poor prognosis for near drowning
2. why in general do children have better near drowning results than adults

Back 824

1. children < 3 years of age, submersion time >5 minutes, ressusitation delay>10 minutes, CPR required, abnormal neurologic examination ro seizures, pH < 7
2. primitive dive reflex shunts blood to vital organs

Front 825

what are the risk factors for child abuse that increase the frequency of abuse

**SUSPECTED CHILD ABUSE MUST BE REPORTED

Back 825

<4 years of age
<1 year of age
mental retardation
chronic illness

Front 826

what risk factors increase the probability that a person will abuse a child

Back 826

low self esteem, social isolation, depression, history of substance abuse
history of child abuse
history of mental illness
history of violent temperament
single parenthood, unemployment, poverty, marital conflict

Front 827

bruises
1. red/blue, blue/purple, green, yellow/brown bruise ages
*inflicted bruises tend to be on protected areas: face, neck, back, chest, abdomen, buttocks, genetalia
*human bite marks
2. what feature of burns leads to suspicion of abuse

Back 827

1. 0-3 days, 3-5 days, 5-8 days, 8-14 days
2. clear line of demarcation, stocking/glove distribution (submersion),
Accidental burns tend to be more splashlike and irregular

Front 828

1. what kind of bone fractures characteristic of abuse

Back 828

1. metaphyseal fractures (bucket handle or corner fractures) from torsional force or violent shaking
posterior/first ribs, sternum, scapula, vertebral spinous processes
*MULTIPLE fractures at different stages of healing

Front 829

shaken baby syndrome / shaken impact syndrome clinical findings

Back 829

retinal hemorrhages, subdural hematomas, metaphyseal fractures, brain injury

Front 830

what visceral injuries cause death in child abuse

Back 830

injury to the GI tract, liver, and spleen

Front 831

1. what features during interview with parents is suspicious for abuse
2. diagnosis of abuse

Back 831

1. implausable histories, histories that change or are inconsistent
2. dilated ophthalmological exam, bone skeletal survey to find old fractures

Front 832

sexual abuse
1. how do patients who are victims to sexual abuse typically present
2. treatment of suspected sexual abuse

Back 832

1. with non-specific complaints including abdominal and urogenital, sexual behavior
2. safety of the child, child protective services, pregnancy prevention, empiric antibiotics for STDs

Front 833

SIDS
1. peak incidence + clinical presentation
2. treatment
3. autopsy findings

Back 833

1. 2-4 months, child is dead after being put to sleep at night
2. resuscitation on all patients
3. intrathoracic petechiae, puulmonary congestion ,small airway inflammation, hypoxia

Front 834

poisonings
1. what age do most poisonings occur
2.when/why do most poisonings occur

Back 834

1. children <6 years of age
2. accidental when the caregiver is distracted (usually ingestions, somtimes inhaled, cutaneous, skin/eyes, intravenous

Front 835

what are the general non-specific signs/symptoms of poisoning
*calculate potential poison dose as WORST CASE****

Back 835

seizures, vomiting/diarrhea, dysrhythmias, altered mental status, abnormal behaviors, shock, trauma, metabolic acidosis

Front 836

poisoning scents from pt
1. bitter almond
2. garlic
3. acetone
4. wintergreen
5. moth balls

Back 836

1. cyanide
2. arsenic/organophosphates
3. salicylates, isopropyl alcohol
4. methyl salicylate
5. camphor

Front 837

poisoning skin findings from pt
1. cherry red color
2. sweaty
3. dry skin
4. urticaria
5. gray skin

Back 837

1. carbon monoxide/cyanide
2. organophosphates/sympathomimetics
3. anticholinergics
4. allergic reaction
5. methemoglbinemia

Front 838

poisoning eye findings
1. miosis
2. mydriasis
3. nystagmus
4. retinal hemorrhages

Back 838

1. opiates, organophosphates, phencyclidine, phenothiazines
2. amphetamines, cocaine, TCA, atropine
3. dilantin (phenytoin), phencyclidein
4. carbon monoxide/methanol

Front 839

poisonings that can cause fever

Back 839

cocaine, TCA, phencyclidine, salicylates, thyroixine, anticholinergics, amphetamines, theophylline

Front 840

lab studies for poisoning

Back 840

screening- serum glucse, serum/urine toxicology screen, electrolytes/anion gap

Front 841

poisoning----
1. what are the causes of anion gap metabolic acidosis
2. what are the radioopaque substances that can be seen on abdominal Xray

Back 841

AMUDPILES (AG>16)
1. alcohol, methanol, uremia, DKA, paraldehyde, iron/isoniazid, lactic acidosis, ethylene glycol, salicylates
2. CHIPE - chloral hydrate, calcium, heavy metals, iodine/iron, phenotyiazines, enteric coated tablets

Front 842

treatment for poisonings
1. if patient has altered mental status
**CALL POISON CONTROL FOR HELP***
2. syrup of ipecac
3. gastric lavage

Back 842

1. dextrose + naloxone
2. syrup of ipecac induces emesis, only useful in first 30 minutes--- does not improve clinical outcome......
3. large bore orogastric tube suction within one hour after ingestion --- variable results as to clinical outcome

Front 843

poisoning
1. activated charcoal + which toxins is it ineffective for

Back 843

1. binds toxins, minimizes their absorbtion --- iron, lithium, alcohols, ethylene glycol, iodine, potassium, arsenic, ferrous sulfate, strong bases, cyandide, strong bases
**contraindicated in caustic injections because it will interfere with endoscopy

Front 844

poisoning
1. what is whole bowel irrigation

Back 844

1. rapid complete emptying of the GI tract using polyethylene glycol + electrolyte solution

Front 845

poisoning
1. acetaminophen mechanism of damage
2. 30minutes - 24 hours
3. 24-72 hours
4. 72-96 hours
5. 4 days - 2 weeks

Back 845

1. depletes glutothione--> accumulation of toxic intermiates causes death of hepatocytes and hepatocellular necrosis
2. asymtomatic + N/V + diarrhea
3. hepatic transaminases rise
4. hepatic necrosis (-->transplant), jaundice, hypoglycemia, lactiic acidosis, hepatic encephalopathy, coagulopathy, renal fialure
5. resolution, progressive liver damage, death

Front 846

treatment of acetaminophen poisoning

Back 846

gastric lavage, activated charcoal,
serum acetaminophen 2-4 hours after ingestion plot on matthew rumack monogram to determine hepatitis potential
*N-ACETYLCYSTINE 140mg/kg loading dose then 70mg/kg every 4 hours for 17 doses

Front 847

salicylates poisoning
1. pathogenesis
2. clinical presentation
3. lab findings

Back 847

1. metabolic acidosis from the weak acid, but it STIMULATES THE RESPIRATORY CENTER -> respiratory alkalosis
+ UNCOUPLE OXIDATIVE PHOSPHORYLATION --> lactic acidosis and enhancing ketosis
2. fever, diaphoresis, tinnitus, vomiting, headache, lethargy, restlessness, coma, seizures
3. respiratory alkalosis with an anion gap metabolic acidosis, hyperglycemia-->hypoglycemia, hypokalemia

Front 848

salicylate poisoning treatment

Back 848

gastric lavage, activated charcoal
serumsalicylate 6 hours after injection and plot on a done nomograph, alkalinization of the urine with sodium bicarbonate, dialysis

Front 849

iron poisoning parthenogenesis + tx

Back 849

kid eats ferrous sulfate tablets, or prenatal vitamins--> GI tract damage -->hemorrhage, hepatic injury/necrosis, pooling of blood and fluids in GI tract (+hematemesis)/pleural/peritoneal cavity (Third spacing), interference with ox phos causes lactic acidosis + tx = ipecac and deferoxamine

Front 850

toxins/antidotes
1. acetaminophen
2. anticholinergics
3. benzodiazepines
4. black widow spider venom
5. carbon dioxide
6. coral snake
7. cyanide

Back 850

1. n-acetyl cystine
2. physostigmine
3. flumazenil
4. antivenin lactrodectus mactans
5.oxygen
6. antivenin micuris fulvius
7.amyl nitrate, sodium nitritie, sodium thiosulfate, hydroxocobalamin

Front 851

toxins/antidotes
1. digitalis glycosides
2. heavy metals (mercury, maganese, copper, gold, nickel, zinc, lead, arsenic)
3. inducers of dystonia
4. inducers of methemoglobinemia
5. iron
6. isoniazid

Back 851

1. digoxin Fab fragments
2. D-penicillapmine, dimercaprol, DMSA, EDTA
3. diphenhydramine, benztropine
4. methylene blue
5. deferoxamine
6. B6

Front 852

toxins/antidotes
1. methanol/ethylene glycole
2. narcotics
3. organophosphate/carbamate pesticides
4. pit viper snake bite
5. beta blockers/calcium channel blockers
6., sulfonylureas/oral hypoglycemics

Back 852

1. ethanol, fomepizole
2. naloxone
3. atropine, pralidoxime
4. antivnin, crotalidae polyvalent Fab fragments
5. glucagon
6. octreotide, glucagon

Front 853

treatment of iron poisoning

Back 853

gastric lavage
ACTIVATED CHARCOAL DOESNT HELP
treat hypovolemia, blood loss, shock
intravenous deferoxamine if iron >500ug/dL or >300ug/dl + acidosis, hyperglycemia, or leukocytosis

Front 854

how to know if deferoxamine is the right treatment for iron poisoning if serum iron is not known

Back 854

do a test dose, if the urine turns a pink color, then the test is positive and deferoxamine should be continued

Front 855

stages of iron toxicity
1. 1-6 hours
2. 6-12 hours
3. 12-36 hours
4. 2-6 weeks

Back 855

1. abdominal pain, vomiting, diarrhea, GI BLEEDING, shock from bleeding, fever, leukocytosis
2. resolution of stage 1 symptoms
3. metabolic acidosis, circulatory collapse, hepatic/renal failure, DIC, neurologic deterioration
4. late sequelae, pyloric or intestinal scarring with stenosis

Front 856

1. clinical presentation of lead poisoning
2. hematologic findings of lead poisoning
3. radiologic findings of lead poisoning

Back 856

1. irritability, seizures, listlessness, colicky pain, decreased consciousness/encephalopaty,
2. peripheral smear shows MICROCYTIC ANEIMA WITH BASOPHILIC STIPPLING
3. dense metaphyseal bands on abdominal radiographs
lead lines on the knees and wrists

Front 857

1. lab diagnosis of lead poisoning
2. treatment of lead poisoning

Back 857

elevated serum lead level
elevated erythrocyte protoporphyrin
2. dimercaprol, british anti-lewisite, calcium disidium ethylenediaminetetraacetic acid

Front 858

1. pathogenesis of acid ingestion
2. pathogenesis of alkali ingestion
3. clinical presentation

Back 858

1. acids cause coagulative necrosis + superficial damage
2. liquifactive necrosis and deep penetrating damage
3. immediate burning -->dysphageia, salivation, chest pain, OBSTRUCTIVE AIRWAY EDEMA, gastric perforation, esophageal perforation with mediatinitis

Front 859

treatment of ingestion of acid or alkali

Back 859

DONT NEUTRALIZE (this will generate an exothermic reaction
NO IPECAC, GASTIC LAVAGE, OR ACTIVATED CHARCOAL
endoscopy,
do not treat for household bleach ingestion

Front 860

carbon monoxide poisoning
1. parthenogenesis
2. clinical presentation
3. perminant damage from CO poisoning

Back 860

1. CO displaces oxygen from hemoglobin forming CARBOXYHEMOGLOBIN which does not carry oxygen--> left shifted oxygen dissociation curve ,
2. CHERRY RED SKIN (due to inc. venous O2 content), retinal hemorrhages, tachycardia, tachypnea, syncope, slurred speech, cyanosis
3. memory loss, personality change, deafness, seizures

Front 861

1. treatment for carbon monoxide poisoning
2. when should patient with carbon monoxide poisoning be hospitalized

Back 861

hyperbaric oxygen
2. CO -Hb >25%, neurologic symptoms, metabolic acidosis or ECG changes

Front 862

1. what secondary infections associated with dog bites
2. tx

Back 862

1. staph, pasturella, strep
2. irrigation, suturing for wounds <12 hours old on body, <24 hours old for the face
amoxicillin + clavulonic acid , tetanus prophylaxis

Front 863

1. cat bites secondary infetion

Back 863

1. paturella multocida, bartonella henselae (Cat scratch disease)

Front 864

1. where is a human bite most likely located during a fist fight + pathogenesis
2. pathogens in human bites

Back 864

1. MCP joint --> avascular facial layers -->deep infection/tendinitis
2. mixed bacterial: s. viridans, s.aureus, anarobes (bacteroides, peptostreptococcus, eikenella)

Front 865

black widow spider
1. pathogenesis + clinical presentation
2. treatment

Back 865

1. black spider with red or orange hourglass marking--> female bites delivers POTENT NEUROTOXIN --> HYPERTENSION + MUSCEL CRAMPS + headaches,"", dizziness, N/V, anxiety, sweating
2. local care, benzodiazepines for muscle cramping, meperideine, lacrodectus anti-venin for sever envenomation

Front 866

brown recluse spider (fiddleback)
1. pathogenesis + clinical presentation
2. treatment

Back 866

1. brown violin shaped marking --> bite --> painful itchy papule-->necrotic/deep lesion --> +/- fever, chills, weakness, vomiting, DIC, hemoysis, renal failure
2. no antivenin available, treat ulcer with steroids, skin grafting, dapsone, hyperbaric oxygen

Front 867

pit viper snakes
1. pathogenesis + clinical presentation
2. treatment

Back 867

1. 95% of snake bites, rattlesnake, cottonmouth, copperhead-> proteolytic enzyme venom --> swelling and eccymosis at site of bite --> paresthesias of scalp, periorbital fasiculations, weakness, diaphoresis, dizziness, METALLIC TASETE IN MOUTH, coagulopathy, thrombocytopenia, hypotenision, shock
2. crotalidae polyvaent immune Fab

Front 868

coral snakes
1. pathogenesis + clinical presentation
2. treatment

Back 868

1. RENEXT TO YELLOW, KILLA FELLOW, --> neurotoxic venum --> SEVERE SX = paresthesisas, vomiting, diplopia, weakness, fasiculations, confusion, respiratory depression
2. antivenin, local wound care, supportive care

Front 869

what is the initial sign of cerebral herniation

Back 869

bradycardia***

Front 870

what should be done if there is no antivenin available for a patient bit by a poisonous snake

Back 870

immobilization, tetanus prophylaxis, local wound care and transportation to a facility that has anti-venin

Front 871

how much protein is required in the diet of an infant vs adult

Back 871

infant 2.2g/kg
0.8 g/kg

Front 872

what fraction of calories should come from fats

Back 872

<30%

Front 873

vitamin deficiency
1. vitamin A
2. vitamin D
3. vitamin E

Back 873

1. night blindness, xerophthalmia,
2. rickets/osteomalacia, dental caries, hypocalcemia/hypophosphatemia
3.anemia/hemolysis, neurologic defecits, altered prostaglandin synthesis

Front 874

vitamin deficiency
1. vitamin K
2.vitamin B1
3. vitamin B6
4. vitamin B12

Back 874

1. coagulopathy, prolonged PT/PTT, abnormal bone matrix synthesis
2. beri beri- cardiac failure, peripheral neuropathy, hoarsenes/aphonia, wernickes encephalopathy
3. peripheral neuropathy, microcytic anemia, dermatitis, chelosis, glossitis
4. megaloblastic anemia, demyelination, methylmalonic acidemia

Front 875

vitamin deficiency
1. vitamin C
2. folic acid
3. niacin
4. zinc

Back 875

1. scurvy- hematologic abnormalities, edema, spongy welling of the gums, poor wound healing, impaired collagen synthesis
2. megaloblastic anemia, neutropenia, impaired growth, diarrhea
3. pellagra - diarrhea, dermatitis, dementia, glossitis, stomatitis
4. skin leasons, poor wound healing, immune dysfunction, diarrhea, growth failure

Front 876

marasmus
1. pathogenesis + clinical pres

Back 876

1. near starvation from PROTEIN AND NON-PROTEIN DEFICIENCY --> loss of muscle/body fat

Front 877

kwashiorkor
1. pathogenesis + clinical pres

Back 877

1. diet that consists of only starches --> PROTEIN DEFICIENCY --> GENERALIZED EDEMA , abdominal distension, skin pigmentation changes, thin sparse hair

Front 878

what is malabsorbtion

Back 878

inadequate absorbtion of nutrients that is characterized by abdominal distension and impaired growth

Front 879

digestion

Back 879

process of breaking down foods using mechanical breakdown, digestive enzymes and bile acids

Front 880

absorbtion

Back 880

uses intestinal mucosal surface and villous brush border for transport of macronutirent monomers and micronutrients

Front 881

1. carbohydrate malabsorbtion pathogenesis
2. lab diagnosis

Back 881

1. congenital ENZYME DEFECT/MUCOSAL ATROPHY --> undigested sugars are osmotic force that cause water to enter the intestine increasing stool volume/peristalsis and decreasing transit time ==> colonic bacteria proliferation forming H2/CO2/acids --> diarrhea
2. reducing substance (sugars) by positive CLINITEST reaction, stool pH<5.6

Front 882

1. protein malabsorbtion pathogenesis
2. lab diagnosis

Back 882

1. enterokkinase def/protein losing enteropathies/crohns/colitis --> dietary loss of proteins
2. fecal alpha 1 antitrypsin levels used to document enteric protein loss

Front 883

1. lipid malabsorbtion pathogenesis
2. lipid malabsorbtion diagnosis

Back 883

1. dec lipase/exocrine pancreatic insufficiency/intestinal mucosal atrophy/bile acid deficiency /abetalipoproteinemia --> steatorrhea and dec. absorption of ADEK
2. fecal fat, pH, reducing substances, alpha 1 antitrypsin

Front 884

1. what studies for suspected malabsorbtion
2. CBC findings in B12 or folate malabsorbtion
3. """ abetalipoproteinemia
4. """" schwachann diamond syndrome
5. low serum albumin

Back 884

1. fecal fat, carbohydrates (CLINITEST reducing sugar), alpha 1 antitrypsin, CBC
2. macrocytic anemia
3. acanthocytosis
4. neutropenia
5. protein losing enteropathies

Front 885

schwachman-diamond syndrome

Back 885

autosomal recessive pancreatic insufficiency (->chronic diarrhea) disease, FTT, short stature, cyclic neutropenia

Front 886

protein intolerance
1. pathogenesis + clinical presentation
2. enteropathy
3. enterocolitis
4. dx/tx

Back 886

1. cows milk protein (MCC), soy, egg protein--> diarrhea/vomiting, colicky pain, ENTEROPATHY, ENTEROCOLITIS
2. progressive diarrhea, vomiting, irritability, abdominal pain --> anemia, protein loss, FTT
3. acute diarrhea, rectal bleeding, mucus, distension, irritability, hypoproteinemia, FTT
4. withdrawing the food, avoid the food

Front 887

celiac disease
1. age of onset
2. pathogenesis + clinical pres
3. diagnosis
4. consequences of not maintaining a gluten free diet

Back 887

1. 6 months - 2 years
2. wheat, barley, rye, and oats contaminated with wheat
--> autoimmune intolerance to gluten in the proximal small intenstine --> diarrhea, vomiting, bLoating, anorexia, FTT, large foul smelling stools, DERMATITIS HERPETIFORMIS**, HYPOCHROMIC MICROCYTIC ANEMIA
3. small bowel biopsy, IgA anti-endoymysial, IgA anti transglutaminase, IgG anti gliadin
4. growth failure, delayed sexual maturity

Front 888

short bowel syndrome
1. pathogenesis
2. clinical presentation
3.treatment

Back 888

1. congenital - gastroschisis, volvulus, intestinal atresia
necrotizing enterocolitis surgery, rohns disease, tumors, radiation --> carbohydrate/fat malabsortbion, dehydration, hyponatremia, hypokalemia, B12/bile acid malabsorbtion
2. diarrhea, malabsorbtion, failure to thirve
3. parenteral nutrition, but encourage enteral feedings so that intestine/biliary function develops, small bowel transplantation

Front 889

complications of short bowel syndrome

Back 889

TPN cholestasis, intestinal bacterial overgrowth, nutritional deficiencies, poor bone mineralization, renal stones, secretory diarrhea

Front 890

GERD

Back 890

pathological state in which reflux causes GI or pulmonary symptoms

Front 891

when does the lower esophageal spincter normally relax

Back 891

when food is being pushed by peristalitic contraction down the esophagus

Front 892

GERD
1. pathogenesis in children
2. clinical presentation in infants
3. sandifer syndrome

Back 892

1. inappropriate transient lower esophageeal sphincter relaxation OR gastric emptying delay --> prolonged contact of esophageal mucosa with gastric contents --> INFLAMMATION
2. emesis, FTT, sandifer syndrome, feeding refusal (irritation of eating), constant hunger* (buffering action of milk)
3. torticollis with arching of the back due to painful esophagitis

Front 893

physiologic reflux
1. presentation
*compare this to GERD in children

Back 893

1."happy spitters" 60% of all infants have episodes of spitting up/vomitting related to overfeeding THIS IS BENIGN EMESIS, but parents take their kids to the doctor because they are worried

Front 894

GERD clinical presentation in older children

Back 894

mid epigastric pain that is relieved with food or antiacids,
exacerbated by fatty foods, caffeine, and supine position

Front 895

complications of GERD
1. upper/lower airway
2. gastrointestinal

Back 895

1. upper and lower airway disease exacerbation
bronchopulmonary constriction
aspiration
chronic laryngitis, hoarseness, wheezing, vocal cord nodules, subglottic stenosis
2. barretts esophagus(stratified squamous --> columnar-->esophageal adenocarcinoma

Front 896

GERD
1. diagnosis

Back 896

1. *barium upper gastrointestinal study shows ASPIRATION - reflux of the barium to the oropharynx is just due to transient lower esophageal sphinger realxation during the study and is not indicative of GERD
*scintigraphy - technetium 99m mixed with food measrues rate of gastric emptying, and radioactive material in the lungs indicates aspiration
*pH probe measurement - measure NUMBER OF TOTAL ASPIRATION EPISODES
*endoscopy/biopsy
* bronchoscopy with lavage to detect aspiration

Front 897

GERD
1. treatment

Back 897

1. upright positioning during sleepand after feedings, thicken feeds H2 blockers, PPIs, metoclopromide (MOTILITY AGENT)
surgery - nissen fundoplication - wrap the fundus of the stomach around the esophagus leading to decreased transient lower esophageal sphincter relaxation
+ gastrostomy to assist with the adaptation to the smaller gastric size after fundoplication

Front 898

hypertrophic pyloric stensois
1. epidemiology
- may be associated with duodenal atresia, tracheoesophageal fistula, trisomy 18, cornelia de lange syndrome
2. pathogenesis
3. clinical presentation
4. treatment

Back 898

1. UNKNOWN ETIOLOGY first born male children 4:1 female to male
2. thickening of pyloric muslce --> obstruction + PROJECTIVLE VOMITTING
3. nonbilous milky fluid vomiting, projectile vomiting, irritability, hunger, jaundice, dehydration
- palpable olive, visible peristalsis, hypochloremic- hypokalemic-metabolic acidosis (from vomitting), ultrasound shows thiceked pylorou, UGI shows STRING SIGN (narrowed pyloric channel)
4. tx. dehydration, partial pyloromyotomy

Front 899

malrotation/midgut volvulous
1. epidemiology
2. associatted conditions
3. pathogenesis
4. clinical presentation + CXR

Back 899

1. 2:1 male predominance
2. heterotaxy, small bowel atresia, hirschprung, intussusception
3. @ 10 weeks when the gut returns back to the abdominal cavity through the midgut~ this processes is interrupted --> LADDS BANDS that compress the duodenum, and voluvulus at the small bowel near the SMA
4. bilous vomitting in an apprantly healthy infant, +/- peritoneal signs on physical exam, bowel ischemia-->shock/ cardiovascular collapse + CURLY Q ABDOMINAL RADIOGRAPH

Front 900

malrotation/midgut volvulous
1. diagnosis
2. treatment

Back 900

1. radiograph shows proxial intestinal distension/obstruction, upper intestinal contrast imaging shows abnormal ligament of treitz, and duodenal obstruction and jejunum to the right of midline
2. resection of nonviable intestine, fluid resusitation, antibiotics

Front 901

duodenal atresia
1. pathogenesis
-associated with down syndrome, more common in males
2. clinical presentation
3. diagnosis
4. treatment

Back 901

1. failure of the duodenum to recanalize at 8-10 weeks gestation,
2. polyhydraminos, scaphoid abdomen with epigastric distension, feeding intolerance, vomitting, NO BILIOUS EMESIS
3. abdominal radiograph shows DOUBLE BUBBLE
4. hydration/electrolytes, duodenoduodenostomy

Front 902

jejunal atresia
1. pathogenesis + clinical presentation
2.dx/tx

Back 902

1. mesenteric vascular accident during fetal life--> obstruction/reabsorbtion of the damaged jejunum--> billlous emesis and abdominal distension
2. air fluid levels and contrast studies show atresia, "TRIPLE BUBBLE ON CXR
tx = NG suctioning, and surgical resection/anastomosis of the atretic small bowel

Front 903

intussusception
*most common in males 5-9 months of age
1. pathogenesis
2. dx/tx

Back 903

1. lead point (meckels diverticulum, polyp, intestinal dupication, peyers patch, lymphoma) --> telescoping of distal ileum into the colon --> BOWEL WALL EDEMA AND HORMORRHAGE/ISCHEMIA/INFARCTION
2. sudden onset episodic crampy or colicky abdominal pain, infant draws legs toward chest, vomitting/lethargy, CURRANT JELLY STOOLS, SAUSAGE SHAPED PALPABLE MASS in the right upper quadrant
3. contrast enema both to diagnose (COIL SPRING SIGN), and will also reduce the intussception
- if this doesnt work can use operative redution

Front 904

pg 295 for acute abdominal pain ddx

Back 904

...

Front 905

abdominal exam findings for
1. intestinal obstruction
2. peritonitis
3. what labs should be ordered for acute abdomen

Back 905

1. high pitched bowel sounds,abdominal distension, tenderness, peristalis
2. DIMINISHED OR ABSENT BOWEL SOUNDS, abdominial rigitidty, involuntary guarding, REBOUND TENDERNESS
3. CBC, urinalysis, metabolic panel, hepatic function tests, amylase/lipase, pregnancy/STD tests

Front 906

appendicitis
- most often age 8-12 years of age
1. pathogenesis
2. clinical presentation
3. dx

Back 906

1. fecolith or lymphoid tissue cuases appendiceal distension/ischemia
2. T10 periumbilical pain + vomitting --> right lower quadrant pain @ MCBURNEYS POINT
3. leukocytosis with PMN prodominance, abdominal ultrasound/CT- TX appendectomy, antibiotics, irrigation of the peritoneal cavity if perforation occurs

Front 907

acute pancreatitis
*uncommon in children
1. pathogenesis
2. clinical presentation
3. dx
4. complications

Back 907

1. iDIOPATHIC (25%) TRAUMA (MCC), INFECTION, congenital , cystic fibrosis, SLE --> pancreatic duct obstruction or infection-->premature activation of proteolytic enzymes leads to autodigestion of the pancreas
2. periumbilical or epigastric pain that RADIATES TO THE BACK, fever, anorexia, N/V, abdominal distension, GRAY TURNER SIGN ( blue colored flanks), CULLEN SIGN ( blue discoloration of the periumbilical area), hypotension, tachycardia
3. serum amylase, serum lipase (more specific + elevated longer), leukocytosis, hyperglycemia, hypocalcemia (precipitates) elevated transaminases, coagulopathy, abdominal pancreatitsi
4. PSEUDOCYSTS, ARDS, renal failure, shock, GI bleeding
5. supportive, TPN, antibiotics, surgery to remove necrotic tissue

Front 908

cholecystitis
*uncommon in children
1. what children have a higher risk for acute cholecystitis
2. pathogenesis
3. causes of acalculous cholecystitis

Back 908

1. sickle cell disease, cystic fibrosis, TPN therapy
2. obstruction of cystic duct--> inc. pressure/secretio of enzymes/prostaglandings --> transmural inflammation/infection /necrosis/perforation
3. salmonella, shigella, e.coli infection, or after trauma, burns, vasculitis

Front 909

cholecystitis
1. clinical presentation
2. dx.tx

Back 909

1. diffuse right upper quadrant pain**, fever, anorexia, jaundice, MURPHYS SIGN*** (RUQ palpation causes pain/guarding/dec. respiratory effort)
2. abdominal ultrasound shows stones + thickened gallbladeder wall
tx= fluid resuscitation, parenteral antibiotics, cholecystectomy if peritonitis is occuring

Front 910

chronic abdominal pain
-organic= caused by disorder
-inorganic= functional (more common in females)
1. causes of organic CAP

Back 910

1. constipation, PUD, carbohydrate intolerance, IBD, pancreatitis, parasites, pyelonephritis, hydronephrosis, malrotation, hernia

Front 911

nonorganic (functional) abdominal pain
1. epigastric pain
2. periumbilical pain
3. infraumbilical pain

Back 911

1. belching, bloating, N/V, early satiety, (equiv to non-ulcer dyspepsia)
2. classic pres - varied in character (dull/sharp etc), confers SECONDARY GAIN, does not interfere with sleep/pleasure...
3. abdominal cramping bloating,alterations in stool (equiv to irritable bowel syndrome)

Front 912

nonorganic abdominal pain
1. causes/risk factors (psychosocial/family history)
2. what past medical history of the child inc. the risk
**the healthier the child appears, the more likely that the pain is functional and not organic

Back 912

1. personality, birth order, life stressors, change in shcool/nanny, family stressors, familial alchoholism, ADHD, family members with functional pain syndromes
2. pregnancy problems, intrapartum problems, Cesarean section, enuresis, nightmares

Front 913

1. laboratory evaluation of organic/non-organic abdominal pain
2. treatment
*poor prognosis, only 50% of children will have resolution of symptoms

Back 913

1. CBC, electrolytes, liver function, fecal occult blood, ova/parasites, ESR/CRP, h.pylori screening, lactose breath test
2/ normalization of childs activities, education of family, counseling MEDICATIONS ARE INEFFECTIVE

Front 914

constipation
encopresis
*encopresis usually in males

Back 914

1. dec. defecation, that is difficuly, and causes abdominal discomfort with dry hard stools
2.developmentally inappropriate release of stool: liquid stool leaks around hard retained stool mass and is released through a distended anorectal canal

Front 915

frequency of stools at
1 week of life
1 year
4 years
~ adults defecate 3x/day to 3x/week

Back 915

4x/day, 2x/day, 1x/day

Front 916

does breastfed or formula fed infant defecate more

Back 916

BREASTFED UP UNTIL 4 MONTHS THEN IT IS THE SAME

Front 917

functional fecal retention (constipation)
MCC constipation in childhood
1. pathogenesis
2. clinical presentation

Back 917

1. traumatic events (hard stool, painful diarrhea, diaper rash, abuse) --> retained stool --> increaesd hardness/mass--> constipation + complications
2. anorectal distension, encopresis, fecal halitosis, abdominal pain/distension, pelvic complications, psychosocial problems

Front 918

organic causes of constipation

Back 918

hirschprungs disease, neuroenteric dysfunction, low fiber feeding, anatomic, dehydration, celiac, hypothyroidism, cystic fibrosis, botulism, lead toxicity

Front 919

what history suggests organic constipation

Back 919

delayed meconium passage, onset of constipation in infancy, history of pelvic surgery, encopresis before 3 years of age, inability to toilet trian

Front 920

what history suggests FFR (inorganic constipation)

Back 920

sentinal event after which bowel change occured - change in psychosocial environement, inappropriate toilet trianing, abuse

Front 921

ulcerative colitis
1. pathogenesis
2. clinical presentation
3. complications

Back 921

1. mucosally limited and effects only the colon, starts at rectup and progresses (ulcerative proctatitis --> pancolitis)
2. rectal bleeding, nocturnal stool, cramping, tenesmus, weight loss, anemia, fever, leukocytosis/hypoalbuminemia (severe)
3. toxic megacolon --> fever, abdominal distension, septic shock, colon cancer

Front 922

crohns disease
1. pathogenesis
2. clinical presentation

Back 922

1. eccentric segmental transmural skin lesions, FISTULAS, sinus tracts, crypt abscess, most commonly effects TERMINAL ILEUM, but can effect anywhere from mouth to anus
2. abdominal pain, post prandial cramping, diarrhea, anorexia iron/B12/zinc/folate deficiency, PERIANAL DISEASE- skin tags, fissures, fistulas, abscesses

Front 923

extra intestinal manifestations
1. ulcerative colitis
2. crohns disease

Back 923

1. uveitis, arthropathy, pyodermagangrenosum, sclerosing cholangitis
2. FTT, delayed sexual development, oral aphthous ulcers, erythema nodosum, arthritis, renal stones, strictures, fistulas, abscesses

Front 924

serologic testing
1. ulcerative cholitis
2. crohns disease

Back 924

1. anti-neutrophil cytoplasmic antibody
2. anti- saccharomyces cerevisiae antibody

Front 925

lab diagnosis/radiological diagnosis of crohns/ulcerative colitis

Back 925

CBC shows leukocytosis/anemia, ESR is elevated, albumin/serum transaminases assess nutritional status and liver disease, serum antibody tests (ANCA, or anti-saccharomyces cerevisiae)
UGI study, colonoscopy with biopsys

Front 926

treatment of crohns disease/ulcerative colitis

Back 926

sulfasalazine (maintenance), corticosteroids(acute exacerbations), immunosuppresants, metronidazole (perianal crohns)
total proctocolectomy - ulcerative colitis
recurrence after bowel resection is high for crohns disease
TPN during flare ups

Front 927

hematemisis vs hematochezia
vs. melena

Back 927

fresh red blood from the mouth (upper GI bleeding) vs the rectum (lower GI bleeding)
melena = dark tarry stools upper GI bleed proximal to ligament of treitz

Front 928

stool guiac test
+ what causes false positive vs false negative

Back 928

guiac is a colorless dye that changes color from peroxidase activity of hemoglobin in the presence of hydrogen peroxide

false positives= ingested iron, red meats, beets, cantaloupe, broccoli, cauliflower
false negative = large doses of vitamin C

Front 929

what are the causes of upper GI bleeding in a child

Back 929

swallowing maternal blood during delivery, or epistaxis, gastric ulcers (stress, burns, H.pylori), mechanical injyr (mallory weiss tears, foreign body/caustic ingestion, varicies (rare)

Front 930

laboratory studies for upper GI bleeding

Back 930

hemoglobin, platelet counts, coagulation studies, serum transalminases, BUN (ELEVATED WITH UPPER GI BLEED),

Front 931

treatment of upper GI bleeding

Back 931

1. intravenous access with two large boor peripheral lines + fluid bolus of 20mg/kg***** of normal saline, octreotide(vasoconstrictor), H2/PPI for ulcers, endoscopic therpay, arteriographic embolization (for vascular malformations)

Front 932

lower GI bleeding
1.necrotizing enterocolitis
2.juvenille polyps
3. allergic colitis
4. infectious enterocolitis
- meckels diverticulum
-hemolytic uremic syndrome
-henoch schonlein purpura
-inflammatory bowel disease

Back 932

1. typically in newborns - condsider in any newborn with rectal bleeding, feeding intolerance, or abdominal distension
2. MCC lower GI bleeding, painless, intermittent, streaky
3. sensitization to protein antigens in cows milk, soy milk, or breast milk
4. salmonella, shigella, campylobacter, yersinia, E.coli

Front 933

ddx for lower GI bleed page 307

Back 933

...

Front 934

meckels diverticulum
1. pathogenesis + clinical presentation
2. diagnosis + tx

Back 934

1. outpouching of the terminal ileum remnant of the vitelline duct, contains ectopic gastric mucosa that produces acid,---> ulceration and painless lower GI bleeding/melena (or lead point for intussuception)
2. nuclear medicine scan*** shows ectopic gastric mucosa
tx = surgical resection

Front 935

hemolytic uremic syndrome
1. pathogenesis
2. lab findings

Back 935

1. vasculitis characterized by MAHA, thrombocytopenia, acute renal failure + intestinal ulceration and infarction of the bowel, cortical necrosis of the kidney
2. hemoglobinuria, proteinuria, elevated BUN/creatinine

Front 936

henoch schonlein purpura
- usually younger than 10 years of age
1. pathogenesis
2. labs

Back 936

1. viral syndrome or URI or GABHS-->IgA mediated vasculitis presents with palpable purpuric rash on the buttocks and lower extremities, edema of hands/feet/scrotum/scalp
large joint arthralgias of knee/ankle (early)
glomerulonephritis (gross hematuria/nephrotic/chronic renal insufficiency)
intussusception, bowel perforation/bleed, colicky GI pain (early)
2. normal platelet count, elevated IgA, normal platelets

Front 937

1. what is AST a marker for
2. what is ALT a marker for
3. what is LDH a marker for
4. what is ALP a marker for
5. what is GGTP/5NT a marker for

Back 937

1. liver, skeletal muscle, RBCs, cardiac tissue
2. VERY SPECIFIC LIVER MARKER****
3. Liver, and HEPATOCELLULAR NECROSIS
4. BILIARY DISEASE, BONE (rapid growth in kids), KIDNEY, INTESTINAL DISEASE, TRAUMA
5. biliary disease (5NT is more specific)

Front 938

1. what is the source of unconjugated bilirubin, how is it converted to conjugated bilirubin
2. what conditions decrese bilirubin excretion

Back 938

1. increased heme load (polycythemia,hematoma,hemolysis) --> UDP glucuronyl transferase--> conjugated bilirubin --> excretion into the biliary system
2. hepatitis, liver fialure, biliary atresia, choledochal cyst

Front 939

cholestatic jaundice
1. definition

Back 939

1. retention of bile within the liver, direct bilirubin >2 or 15% of total bilirubin

Front 940

neonatal jaundice
1. what kind of jaundice do 50% of neonates have
2. what is the progression of neonatal jaundice
3. does degree of jaundice indicate the level of bilirubin

Back 940

1. transient unconjugated hyperbilirubinemia-- due to decreased function of UDP glucuronyl
2. cranial to caudal in an otherwise healthy appearing infant
3. NO MUST STILL MEASURE DIRECT/INDIRECT LEVELS OF BILIRUBIN

Front 941

inspissated bile syndrome

Back 941

massive hemolysis ex. large hematoma or ABO incompatability -->

Front 942

what diseases have UDP glucuronyl transferase deficiency

Back 942

gilberts syndrome,
crigler najjar type 1 (AR) - 90% of enzyme funciton deficient
crigler najjar type 2 (AD) ~ 100% of enzyme function

Front 943

causes of cholestatic jaundice(increase conjugated bilirubin)
1. infections -
2. metabolic derrangements
3. extrahepatic mechanical obstruction
4. intrahepatic mechanical obstruction
- idiopathic, neonatal hepatits
- alpha 1 antitrypisn deficinecy
- TPN associated
5. what are the clinical features of cholestasis

Back 943

1. sepsis, hepatitis, viral infections
2. cystic fibrosis, hypothyroidism
3. biliary atresia, bile duct stricture
4. paucity of intrahepatic bile duct, alagille syndrome
5. jaundice, acholic(light color) stools, dark urine, hepatomegaly, bleeding (dec. factors), FTT

Front 944

neonatal hepatitis
1. pathogenesis
2. treatmnet

Back 944

1. idiopathic hepatitis--> transient jaundice, acholic stools, cirrhosis, portal hypertension (VERY WIDE RANGE OF SEVERITY)
2. nutritional support + ADEK supplements, TPN, liver transplantation

Front 945

biliary atresia
-etiology unknown
1. pathogenesis + clinical presentation
2. associated syndrome
3. dx/tx

Back 945

1. progressive fibrosclerotic disease of the extrahepatic biliary tree --> jaundice, dark urine, pale/acholic stools, hepatosplenomegaly, ascites, peripheral edema, coagulopathy --> obliteration of the bile duct/cirrhosis by 4 months of age (EASY TO CONFUSE WITH PHYSIOLOGIC JAUNDICE)
2. polysplenia syndrome - bilobed lungs, abdominal heterotaxia, situs ambiguous
3. cholangiogram with laparotomy
tx = kasai portoenterostomy establishes bile flow most successful if child <50-70 days of age, cholangitis is worst complication (dec. all bile flow),
liver transplant
supportive care- ADEK, TPN, ursodeoxycholic acid

Front 946

alagille syndrome
1. pathogenesis + clinical presentation

Back 946

1. autosomal dominant paucity of intrahepatic ducts--> CHOLESTATIC LIVER DISEASE + EXTREME PRURITUS, unusual facial characteristics, pulmonary outflow obstruction/ToF, renal disease, postery embryotoxon, butterfly vertebrae/broad thumbs, pancreatic insufficiency, hypercholesterolemia

Front 947

clinical features of hepatitis** (infectious, autoimmune, drug induced)

Back 947

asymptomatic
jaundice, hepatosplenomegaly, ascites, increased abdominal vascular markings, caput medusae, spider hemangiomas, clubbing

Front 948

hepatits A (picornavirus)
1. pathogenesis
2. diagnosis

Back 948

1. fecal oral -->2-6 week incubation--> asymptomatic jaundice
2. IgM anti-HAV then lifelon IgG anti-HAV

Front 949

hepatitis B
1. pathogenesis
2. clinical presentation (acute/chronic)
3. diagnosis

Back 949

1. perinatal vertical exposure, parenteral route (IVDU, needle expossure, blood products, body secretions) --> incubtion--> sx + virus in blood,tears, saliva, semen, urine, feces, breast milk
2. acute - nonspecific systemic illnesses, or clinical heptaitits, or fulminant liver failure
chronic (MCC in infants)- cirrhosis, hepatic fibroisis, protal hypertension, inc. risk for HCC

Front 950

hepatitis B serology
1. HBsAg
2. IgG anti HbS
3. IgG anti HBc
4. HBeAg
5. IgG anti HBe

Back 950

1. active disease
2. protective: from vaccination* or recovery from natural infection*
3. from natural infection ONLY persists life long (IgM early, IgG late)
4. rises early in active infections, useful to diagnose acute infection
5. rises late in infection

Front 951

treatment of hepatits B

Back 951

1. supportive for acute
interferon alpha and antivirals for chronic

Front 952

hepatitits C
1. pathogenesis
2. clinical presentation (acute/chronic)
3. diagnosis

Back 952

1. perinatal vertical , transfusion, parenteral -->
2. rarely symptomatic acute infection
chronic infection occurs in 80% of infected--> cirrhosis and hepatic fibrosis

Front 953

hepatitis D infection

Back 953

deltavirus that requires HBsAg for replication, cuases progression of hepatitis B or causes fulminant liver failure

Front 954

hepatitis E infection

Back 954

fecal oral , common in developing countries (50% of all cases),
***** 20% MORTALITY IN PREGNANT WOMEN**** NO CHRONIC DISEASE
IgM/IgG anti HEV diagnostic

Front 955

autoimmune hepatitis
1. pathogenesis (type1/type 2)
2. clinical presentation
3. dx
4. treatment

Back 955

1. type 1: ANA or anti-smooth muscle antibodies
type 2: anti-liver kidney microsome or anti- liver cytosol type 1 antibody
2. usually females before puberty, --> mimics viral hepatitis+fatigue, anorexia, arthritis, rash, nephritis, vasculitis
3. elevated serum transaminases, hypergammaglobulinemia, circulating autoantibodies, liver bipsy
4. corticosteroids (acute), then immunosuppressents (azathioprine, 6-mercaptopurine

Front 956

causes of direct hyperbilirubinemia in an infant

Back 956

hepatitis,
biliary atresia, choledochal cyst (abdominal ultrasound, radionucleotide imaging, liver biopsy)

Front 957

treatment for biliary atresia

Back 957

liver transplant

Front 958

what is phototherapy used for

Back 958

INDIRECT HYPERBILIRUBINEMIA ONLY, NOT DIRECT

Front 959

what fraction of hepatitis A infections in children have syymptoms

Back 959

only 30%

Front 960

what is a patients total body fluid requirement

Back 960

maintenence (sensible losses + insensible losses) + ongoing losses

Front 961

how to calculate maintenance water requirement

Back 961

100mL/kg/day for the first 10kg
50mL/kg/day for the second 10kg
20ml/kg/day for each kg above the first 20 kg of body weight

Front 962

how should maintenance fluid be adjusted for a patient with fever

Back 962

increase maintenance fluids by 12% for every degree above 38'c

Front 963

how to calculate maintenance sodium
how to calculate maintenance potassium

Back 963

2-3 mEq/kg/d maintenance sodium
2 mEq/kg/d maintenance

Front 964

hyponatremia
isonatremia
hypernatremia

Back 964

1. Na < 130
2. Na 130-150
3. Na > 150

Front 965

what are three levels of dehydration

Back 965

mild (3-5%), moderate (7-10%), severe (>12%)

Front 966

two phases of parenteral rehydration

Back 966

1. emergency phase - 20ml/kg BOLUS of normal saline or lactated ringers
2. repletion phase - acute hyponatremic or isonatremic dehydration correct over 24 hours
hypernatremic dehydration correct over 48 hours
****correct chronic dehydration much more slowly

Front 967

what is the risk of correcting hypernatremia too quickly

Back 967

cerebral edema, central pontine myelinolysis

Front 968

1. oral rehydration therapy mechanism
2. ORT not used when

Back 968

1. glucose + electrolytes ~~ electrolyte transport is enhanced by glucose, this leads to increased uptake of solutes~~~ this mechanism is not effected during secretory diarrhea
2. severe life threatening dehydration, paralytic ileus, GI obstruction, rapid stool losses or repeated severe emesis losses

Front 969

microscopic hematuria
proteinuria

Back 969

>6 RBCs per high powered field
>100mg/m2/day (by urine dipstick)

Front 970

RBCs in the urine identification
1. RBC casts
2. blebbed RBCs
3. normal biconcave RBCs
4. large numbers of RBCs + dysuria

Back 970

1. glomerular bleeding~ acute/active glomerulonephritis
2. RBCs originating in the glomerulus
3. lower urinary tract in origin (ie. distal to the glomerulus)
4. acute hemorrhagic cystitis

Front 971

urine findings
1. crystals

Back 971

1. renal stone disease

Front 972

causes of acute hemorrhaggic cystitis

Back 972

bacterial, viral (adenovirus) , chemotherapeutic (cyclophosphamide)

Front 973

what can cause a false positives/negatives urine dipstick for protein

Back 973

SG >1.025, ph>7, penicillin, aspirin, oral hypoglycemia

false negative - dilute urine

Front 974

proteinuria
total protein:creatinine ratio
1. normal TP/CR for infants/children

Back 974

1. 6-24 months is <0.5, >2 years <0.2 is normal

Front 975

pg 328/329 differential diagnosis for hematuria/red urine

Back 975

.........

Front 976

causes of benign transient proteinuria

Back 976

vigorous exercise, fever, dehydration, CHF

Front 977

orthostatic proteinuria + dx

Back 977

seen in athletic individuals have increased protein when upright, but not supine
dx = normal TP/CR in the morning, but elevated in the afternoon

Front 978

1. what does persistent proteinuria indicate
2. what kind of protein in glomerular proteinuria + causes
3. what kind of protein in tubular proteinuria + causes

Back 978

1. renal diseaes
2. large molecular weight proteins (ALBUMIN**), seen in glomerulonephritis/nephrotic syndrome
3. small molecular weight proteins (BETA 2 MICROLOBULIN**) (from decreased reabsorbtion by damaged tubular epithelium) interstitial nephritis, ischemic renal injury (ATN), nephrotoxic drugs

Front 979

what substances are seen in the urine from tubular damage

Back 979

biconcave RBCs, glucosuria, aminoaciduria, BETA 2 MICROGLOBULIN

Front 980

nephritic vs nephrotic

Back 980

HERAN - hypertension, edema, acute sedement, reduced GFR, (RBC/WBC casts), non-nephrotic proteinurina <50mg/kg/day
HELLP - hypertension, edema, lipiduria, hyperlipidemia, proteinuria >50mg/kg/day

Front 981

laboratory evaluation for suspected glomerulonephritis

Back 981

urinalysis, urinary TP/CR, blood chemistries (electrolytes/BUN/creatinine/serum albumin/liverenzymes/cholesterol), serum complement, antibody testing (ANA, ASO, anti-DNase B), IgA level

~ can also consider hep C/B titers, HIV testing

Front 982

what are the causes of post-infectious glomerulonephritis

Back 982

strep A, HIV, hepatitis B

Front 983

post streptococcal glomerulonephritis
1. pathogenesis + clinical presentaiton
-recorvery in 6-8 weeks, chronic renal failure rare
2. lab findings

Back 983

1. 8-14 after nephritogenic strain of group A B-hemolytic strep skin/pharynx infection --> immune complex deposition+ complement in the glomerulus--> hematuria, proteinuria, hypertension, edema (HERAN)
2. low serum complement (low C3,low CH50, C4 normal), +ASO titer (only 50% positive after impetigo), anti-DNase B (positive after respiratory and skin infection)

Front 984

post strep glomerulonephritis
1. renal biopsy findings
2. treatment
3. does abx treatment reduce the risk of post strep glomerulonephritis

Back 984

1. mesangial cell proliferation, increased mesangial cell matrix, lumpy bumpy immunoflursent, subEPIthelial deposits on electron microscopy
2. fluid restriction, antihypertensive drugs, restriction of protein/sodium/potassium/phosphorus
3. NOPE, only dec. risk of rheumatic fever, and

Front 985

demographics of IgA nephropathy

Back 985

asia, australia, native americans

Front 986

IgA nephropathy (bergers disease)
1. pathogenesis + clinical presentation
2. biopsy and lab diagnosis
3. treatment
20-40% go to end stage renal disease

Back 986

1. respiratory infection--> recurrent hematuria
2. mesangial proliferation, increased mesangial matrix, high serum IgA
3. supportive, ACE inhibitors (for htn), steroids, immunosuppressants

Front 987

henoch schonlein purpura
1. pathogenesis + clinical presentation
1-5% --> renal failure

Back 987

1. IgA vascultiis --> non-thrombocytopenic palpable purpura on the buttocks, thighs, abdominal pain, arthritis, gross/microscopic hematuria

Front 988

MPGN
1. biopsy findings
- many causes, most patients develop end stage renal disease
2. treatment

Back 988

1. lobular mesangial hypercellularity, thickening of the glomerular basement membrane
2. corticosteroids/ace inhibitors

Front 989

membranous nephropathy
- progresses to renal insuficiency
1. associaation

Back 989

hepatatits B

Front 990

nephrotic syndrome
1. most common nephrotic syndrome in children
2. pathogenesis
3. clinical presentation

Back 990

1. miniaml change disease (90%)
2. membranous, membranoproliferative, IgA, minimal change disease --> loss of normal charge/size barrier--> hypoproteinemia from urinary loss of proteins, hypercholesterolemia from from dec. plasma oncotic pressure, decreased lipoprotein lipase activity
3. upper respiratory tract infection --> HELLP symptoms + thrombosis (due to hypercoagulablity) + spontaneous bacterial peritonitis, pneumonia, sepis1

Front 991

nephrotic syndrome
1. lab findings of nephrotic syndrome
2. electrolytes
3. ultrasound
4. tx
- 5% mortality for nephrotic syndrome - in pts that are steroid resistant, or have thrombosis~~~ most common cause of death is BACTERIAL PERITONITIS + THROMBOEMBOLIC EVENTS

Back 991

1. 3+/4+ proteinuria, microscopic hematuria, elevated TP/CR, RBC casts (not in MCD), elevated hematocrit (from hypoproteinemia), thrombocytosis, hypercholesterolemia,
2. metabolic acidosis (RTA)
3. enlarged kidnesy
4. supportive, 25% albumin for hypotension, edema, or pleural effusions, corticosteroids, cyclophosphamide, cyclosporine

Front 992

child with nephrotic syndrome has chest pain, fever what should be done

Back 992

treat for pneumococcal pneumonia empircilly and obtain blood cultures, urine cultures, chest radiograph

Front 993

HUS
1. pathogenesis + clinical pres
2. treatment
3. poor prognostic signs

Back 993

1. uncooked beef, unpasurized milk, contaminated fruit juice --> vascular endothelial damage from shiga toxin (EHEC/shigella) --> diarrhea --> MAHA, thrombocytopenia (from vascular damage and thrombus formation), acute renal failure
2. NO ANTIBIOTICS - treatment of ecoli hemorrhagic colitis leads to an increase that the patient will develop HUS
3. high WBC count, prolonged oliguria, TOXIC MEGACOLON (rare)

Front 994

what does steroid resistant minimal change disease develop into

Back 994

FSGS

Front 995

atypical HUS
1. causes
-similar clinical presentation as shiga toxin HUS
- higher risk of ESRD than

Back 995

1. OCP, cyclosporine, tacrolimus, OKT3, inherited (AD/AR)

Front 996

alports syndrome
1. pathogenesis

Back 996

1. X linked dominant- defect in type IV collagen in the basement membrane --> ESRD, hypertension, hematuria, HEARING LOSS, ocular abnormalities of the lens and retina

Front 997

multicystic renal dysplasia
1. clinical presentaiton
- most common renal mass in a newborn

Back 997

unilateral renal mass

Front 998

autosomal recessive polycistic kidney disease (infantile)
1. clinical presentation
-always progresses to renal insufficiency needing transplantation

Back 998

1. AR inheritance --> oligohydrominos/pulmonary hypoplasia, enlarged cystic kidneys, severe hypertension, ****cirrhosis/portal hypertension***

Front 999

autosomal dominant polycystic kidney disease
1. clinical presentation

Back 999

1. AD inheritance --> adulthood presentation with abdominal pain, flank mass, UTI, gross or microscopic hematuria, hypertension, renal insufficiency, CEREBRAL ANEURYSMS

Front 1000

medullary sponge kidney
1. pathogenesis + clinical presentation

Back 1000

1. AUTOSOMAL DOMINANT, asymptomatic or have hematuria, UTI, or nephrolithiasis

Front 1001

hyper tension
1. normal systolic/diastolic pressure
2. normal high blood pressures
3. hypertension
4. severe hypertension
5. malignant hypertension

Back 1001

1. age based blood pressures <90th percentile
2. 90-95th percentile for age
3. >95th percentile for age
4. >99th percentile for age
5. hyper tension with end organ damage

Front 1002

essential hypretension
secondary hypretension

Back 1002

hypertension with no known etiology (rare)
hypertension with a etiology (most common)

Front 1003

pg 340 hypertension in children

Back 1003

.....

Front 1004

1. most common cause of hypretension in children age 1-10 years vs adolescents

Back 1004

1-10 = renal disease/coarctation of the aorta
adolescents = renal disease and essential hypertension

Front 1005

hypertension
1. clinical presentation
2. physical exam

Back 1005

1. non-specific signs/symptoms, irritabliity, vomitting, FTT, seizures, headaches, stroke,
2. four limb blood pressures (coarct), fundoscopic examination whows hemorrhages, papilledema, AV nicking, CHF findings, cafe au lait spots, abdominal masses

Front 1006

hypertension lab evaluation

Back 1006

CBC, electrolytes, BUN, creatinine, urinalysis, renin, chest radiograph

Front 1007

renal tubular acidosis

Back 1007

inability of the kidney to maintain normal acid base balance

Front 1008

what electrolyte imbalance associated with RTA in the blood / urine

Back 1008

hyperchloremic metabolic acidosis with a normal serum anion gap ********

positive urine anion gap in the urine (Na + K - Cl)

Front 1009

RTA type 1
1. pathogenesis + clinical presentation
2. treatment

Back 1009

1. inherited, nephrotic synd, ampho B --> inability of distal tubule to excrete acid--> comiting, FTT, acidosis, nephrocalcinosis/nephrolithiasis
2. small dosese of alkali

Front 1010

RTA type II (proximal)
1. pathogenesis + clinical presentation
2. treatment

Back 1010

1. heavy metals/gentamycin, fanconi syndrome --> impaired reabsorbtion of bicarb by proximal tubule --> vomiting, FTT, acidosis, muscle weakness
2. large doses of alkali

Front 1011

RTA type III

Back 1011

same as type 2 but presents as wasting in infancy, same treatment

Front 1012

RTA type IV
1. pathogenesis + clinical presentation
2. treatment

Back 1012

1. obstructive uropathy, aldosterone deficient states, diabetes --> transient acidosis + HYPERKALEMIA --> asymptomatic or FTT
2. furosemide, lower serum potassium, oral alkali

Front 1013

clinical presentation of renal failure

Back 1013

lethargy, N/V, respiratory distress, hypretension, seizures, OLIGURIC

Front 1014

oliguria

Back 1014

<1ml/kg/hr urine output

Front 1015

lab evaluation of acute renal failure

Back 1015

serum electrolytes, BUN, creatinine, urinalysis, urinary protein, renal/pelvic ultrasound, nuclear renal scan

Front 1016

management of renal failure
1. what maintenance fluid should be given to a patient with acute renal failure
2. other management for acute renal failure

Back 1016

300ml/m2/day+urine/stool replacement
~~ only correcting for insensible loss
other =
electrolyte intake (low sodium, potassium, phosphorus diet)
protein restriction
dialysis
monitor BUN/creatinine, calcium, ALP
blood pressure management
EPO for anemia

Front 1017

prerenal failure
1. pathogenesis
2. lab findings

Back 1017

1. dehydration, hemorrhage, CHF, septic shock, hypoproteinemia --> decreased renal perfusion --> REVERSIBLE decrease in renal perfusion and dec. GFR
2. BUN/CR >20, urine SG >1.030, urine osmolarity>500, urine Na <20, FENa<1% in older children, <2.5 in neonates

Front 1018

renal parenchymal glomeruluar damge
1. pathogenesis
2. lab findings

Back 1018

1. PSGN, lupus nephritis, HUS--> glomerular damage--> hematuria/proteinuria
2. hematuria/proteinuria

Front 1019

renal parenchymal tubular damage (renal failure)
1. pathogenesis
2. lab findings

Back 1019

1. ischemic injury from hypoperfusion/heme pigments/aminoglycosides-->damage to the tubules (ATN)
2. increased urinary B2 MICROGLOBULIN, FENa >1% or >2.5% in neonates

Front 1020

renal parenchymal acute interstitial nephritis (renal failure)
1. pathogenesis
2. lab findings

Back 1020

1. semisynthetic penicillins --> damage to the interstitium
2. eosinophilia, increased urinary B2 MICROGLOBULIN,

Front 1021

post renal failure (renal failure)
1. pathogenesis
2. lab findings

Back 1021

1. stones/tumor/ureterocoele/urethral tumor, neurogenic bladder, posterior urethral valves-->obstruction of urine flow
2. dilation of renal collecting system

Front 1022

vascular (renal failure)
1. pathogenesis
2. lab findings

Back 1022

1. renal artery embolus (umbilical artery catheter), renal vein thrombosis --> dec. kidney perfusion
2. dec. renal blood flow on nuclear scan

Front 1023

what infants are at high risk for renal vein thrombosis

Back 1023

infants of diabetic mothers

Front 1024

what patients are at high risk for renal artery emboli

Back 1024

patients with umbilical catheters

Front 1025

when is dialysis started for ARF
how long can a kidney graft last after transplant

Back 1025

~ when GFR is 5-10% of normal usually peritoneal dialysis
~ 5 years

Front 1026

where can obstructions distal to the collecting duct occur

Back 1026

ureteropelvic junction
ureterovesicular junction
posteriour urethral valves
prune belly syndrome (bladder outlet)

Front 1027

prune belly syndrome

Back 1027

absense of rectus abdominus, bladder outlet obstruction, cryptochordism, rednal dysplasia/dysfunction---> OLIGOHYDRAMINOS--> lung hypoplasia, hydronephrosis,

Front 1028

renal dysplasia + clinical finding in utero

Back 1028

abnormal renal development due to in utero obstruction or developmental impairments -->defective concentration, RTA, renal insufficiency

in utero see oligohydrominos--> pulmonary hypoplasia

Front 1029

renal agenesis

Back 1029

failure of metanephritc blastema or mesonephric duct to develop~ can be uni/bilateral

Front 1030

multicystic dysplastic kidney

Back 1030

most common abdominal mass in newborns associated with atretic ureter

Front 1031

vesicoureteral reflux
1. pathogenesis
2. dx/tx

Back 1031

1. ureterovesicular junction abnormalities (short submucosal tunnel)--> reflux of urine into the ureters and collecting system--> UTIs (30-50% of UTIs in infants) --> pyelonephritis/RENAL SCARRING**** --> reflux nephropathy (segmental scars/contraction/interstitial nephritits)
2. voiding cystourethrogram (VCUG)
tx = low dose abx,

Front 1032

grade 1-5 vesicoureteral reflux

Back 1032

distal ureter
extension to calyces
extension to calyces with dilation
clubbed calyces/greater dilation
dilation of entire system,severe clubbin and tortuosity of the ureters

Front 1033

what labs should be ordered for urolithiasis

Back 1033

electrolytes, BUN, creatinine, calcium, phosphorus, parathyroid hormone, uric acid, venous blood gas

urine culture

plain radiograph/renal ultrasound

Front 1034

treatment for urolithiasis

Back 1034

hydration, abx for struvite UTI related stone

Front 1035

UTI
1. frequency based on gender
2. pathogenesis
3. clinical presentation

Back 1035

1. up to 6 months of age >boys, after 6 months of age more frequent in girls
2. ECOLI, klebsiella, pseudomonas, s.saprophyticus, serratia, proteus (high pH urine), enterococcus -->ascend in the urethra
3. neonates/infants present with fever, lethargy, irritability, jaundice
children present with noctural enuresis, or diurnal enuresis, low grade fever, dysuria, freq/urge

Front 1036

pyelonephritis clinical presentation

Back 1036

infants - nonspecific fever, lethargy irritability
children - back/flank pain, high fever, vomiting, dehydration

Front 1037

UTI diagnosis
UTI treatment

Back 1037

urinalysis shows >5-10 WBC/high powered field, positive nitirite/leukocyte esterase,
urine culture shows >10000 colonies by urethral cath
urine shows >50000 colonies by clean catch

tx = TMPSMX, cephalexin
toxic appearing- IV antibiotics
neonates - ampicillin/gentamycin

Front 1038

****kid with UTI --> do a VCUG

Back 1038

...

Front 1039

should ipecac be given for hydrocarbon ingestions

Back 1039

no becuase of the risk of aspiration and subsequent necrotizing pneumonia

~~ so - no ipecac for corrosives and hydrocarbons (ex. mineral oil)

Front 1040

organophosphate poisoning mechanism + clinical presentation + tx

Back 1040

inhibits carboxylic esterase enzymes including acetylcholinesterase leading to elevated acetylcholine--> constricted pupils, bradycardia, muscle fasiculations, diaphoresis, diarrhea, salvation
tx = pralidoxime and atropine

Front 1041

scabies pathogenesis + clinical presentation + tx

Back 1041

sarcoptes scabiei var hominins
pruritic threadlike burrows in interdigital areas, groin, elbows, ankles, palms/soles, NO FACE/HEAD
infants present with bullae/pustules

tx = 5% permethrin cream

Front 1042

hypotonia vs weakness

Back 1042

hypotonia = dec. RESISTANCE during passive stretching
weakness = decreased force on active contraction

Front 1043

what antenatal and neonatal findings indicate hypotonia

Back 1043

decreased fetal movements
weak breech presentation
seizures in the neonatal period
weak cry, decreaesd spontaneous movement, frog leg posturing, muscle contractures

Front 1044

central vs peripheral hypotonia effect on DTRs

Back 1044

central increased DTRs, peripheral decreased`

Front 1045

what are the two broad causes of hypotonia

Back 1045

systemic pathologies - sepsis/meningitis, electrolytes, hepatorenal abnormalities etc
neurological pathologies - damage to the neurons (central or peripheral)

Front 1046

1. how is acute life threatening causes of hypotonia ruled out
2. how is central hypotonia evaluated
3. how is peripheral hypotonia evaluated

Back 1046

1. sepsis work up, liver enzymes, CBC, inflammatory markers, electrolyte panel (ex. urea cycle diseases) + others
2. CT head scan, serum electrolytes (Ca/Mg/ammonia/lactate/pyruvate), chromosome stuidies (ex. prader willi)
3. serum creatine kinase, DNA tests, EMG + nerve conduction, muscle biopsy

Front 1047

spinal muscular atrophy
1. pathogenesis + clinical presentation
2. type 1 vs 2 vs 3

Back 1047

1.autosomal recessive SMN1 mutation chromocomse 5 --> anterior horn degeneration by astrocytes/microgia -->hypotonia, weakness, tongue fasiculations, bell shaped chest, frog leg posture, areflexia,
NORMAL EXTRAOCULAR MOVEMENTS NORMAL SENSORY EXAM
2. type 1 = infantile(werdnig hoffman) onset, death by 1 year
type 2 = 6-12 months onset, adolescent survival
type 3 = >3 years onset adult survival

Front 1048

dx/tx of SMA

Back 1048

dx = muscle biopsy no cure
gastrotomy feeding
physical therapy/surveillance for infections

Front 1049

infantile botulism
1. pathogenesis
2. dx/tx
*GOOD PROGNOSIS*~ complete resolution of symptoms***

Back 1049

1. honey/dirt -->c.botulinum spores --> 24-48 hour incubation --> inhibition Ach release --> CONSTIPATION--> weak cry/suck, loss of milestones, ophthalmoplegia, hyporeflexia, SYMMETRIC DESCENDING* PARALYSIS
2. dx = toxin /bacteria in stool, EMG shows INCREMENTAL RESPONSE DURING HIGH FREQ. STIMULATION
tx = botulism Ig, NG feeding, assisted ventilation, NO ABX

Front 1050

congenital myotonic dystrophy
1. pathogensis + clinical presentation (early and late)

Back 1050

1. autosomal dominant trinucleotide repeate expansion chromosome 19 with maternal transmission-->
Early: polyhydraminos, poor feeding/respiration, facial diplegia, hypotonia,
Late: myotonic facies (atrophic masseter/temporalis), ptosis, stiff straight smile GRIP MYOTONIA, cataracts, cardiac arrythmias, infertility

Front 1051

congenital myotonic dystrophy
1. dx/tx
-infant mortality 40% due to respiratory problems, feeding improves with time

Back 1051

1. ELEVATED CPK***, hypotonia, examine the mother, DNA testing

Front 1052

hydrocephalus
1. pathogenesis

Back 1052

1. NON-COMMUNICATING (ex. aqueductal)
COMMUNICATING (inc. production of CSF by tumors, or dec. absorption of CSF ex. meningitis)
HYDROCEPHALOUS EX VACUO- brain atrophy leads to venricular enlargment

Front 1053

chiari type 2 + other associated conditions

Back 1053

downward displacement of the cerebellum/medulla through the foramen magnum blocking CSF flow (-->hydrocephalous) + lumbosacral myelomeningocoele***

also assc- cervical hydrosyringomyelia, gyral anomalies, agenesis of the corpus callosum, orthoedic problems, genitourinary sx

Front 1054

dandy-walker malformation

Back 1054

hypoplastic cerebellar vermis (truncal ataxia), 4th ventrile enlargement blocks CSF flow (-->hydrocephalous)

Front 1055

congenital aqueductal stenosis
1. pathogensis

Back 1055

X linked trait, associated with thumb abnormalities, and other CNS anomalies like spina bifida

Front 1056

acquired causes of hydrocephalous (list)

Back 1056

bacterial meningitis, brain tumors, intraventricular hemorrhage (preterm)

Front 1057

clinical presentation of hydrocephalous in infnats

Back 1057

head circumference >97% for age, large anterior/posterior fontanelles with split sutures
sunset sign - downward deviation of both eyes (from pressure of third ventricle on superior colliculus)

Front 1058

clinical presentation of hydrocephalous in older children

Back 1058

increased intracranial pressure
1headache, nausea, unilateral 6th nerve palsy, papilledema,brisk DTRs + downward plantar response

Front 1059

treatment of hydrocephalous + complications

Back 1059

ventriculoperitoneal shunt diverts flunt of CSF --- complication = shunt infection/obstruction

Front 1060

1. spina bifida
2. neural tube defect
3. myelomeningocoele + clinical findigs
4. meningocele + clinical findings
5. spina bifida occulta + clinical findings

Back 1060

1. any failure of bone fusion of the posterior midline of the spine
2. any failure of neural tube closure (anecephaly/meningocele)
3. herniation of spinal cord and meninges through bony cleft in lumbosacral region + fluctuant midline mass, neurologic defecits ( higher than L3 = paraplegia, lower than S3 = bowel and bladder only)
4. herniation of meninges only through bony cleft (no neural defecit) + fluctuant midline mass (CSF filled) over the spine
5. just bony cleft, no herniation through cleft + hair patch/dimple over the area of the defect

Front 1061

what vitamin is critical for pregnant mothers to take to prevent spina bifida/neural tube defects

Back 1061

folate

Front 1062

diagnosis of neural tube defects + spina bifida + any spinal defect

Back 1062

ALPHA FETO PROTEIN******************* MEASURED AT 16-18 WEEKS
+ fetal ultrasound

Front 1063

which spinal defect requires immediate surgical repair

Back 1063

myelomeningocoele

Front 1064

1. coma
2. MCC coma <5 years old
3. MCC coma older children

Back 1064

1. unawareness of self/environment, unarrousable, eyes closed
2. nonaccidental trauma + near drowning
3. drug overdose, accidental head injury

Front 1065

what focal lesions of the brain can cause coma

Back 1065

subarrachnoid hemorrhage, multiple infarcts, thalamic infarcts, subdural hematoma, non-accidental trauma, cerebellar hemorrhage, post infectious encephalitis

Front 1066

what drugs can cause coma

Back 1066

atropine, scopalamine, benzodiazepines, barbiturates, ethanol, lithium, opiates, TCA

Front 1067

what toxins can cause coma

Back 1067

lead, mercury

Front 1068

1. what metabolic/endocrine abnormalties can cause coma
2. what organs/seizure types """

Back 1068

1. hyper/hypoglycemia
hyper/hyponatremia
hyper/hypothyroidism
hyper/hypocortisolism
diabetic ketoacidosis
2. uremia, hepatic failure, reyes syndrome,cardiac arrest, non-convulsive status epilepticus

Front 1069

decerebrate vs decorticate postureing

Back 1069

decerebrate = extended arms and legs extremities- subcortical damage
decorticate = flexion of arms and extension of legs indicates bilateral cortical injury

Front 1070

breathing pattern indications
1. hypoventilation
2. hyperventilation
3. cheyne stokes
4. apneustic
5. ataxic or agonal

Back 1070

1. opiate or sedatie overdose
2. metabolic acidosis (deep rapid breathing)
3. alternating apneas and hyperpneas (bilateral cortical injury)
4. pons damage (pausing at full inspiration)
5. irregular respirations with no patter - medullary injury or impending brain death

Front 1071

pupillary size and reactivity indications
1. unilateral dilated non-reactive
2. bilateral dilated non-reactive pupils
3. bilateral constricted reactive

Back 1071

1. UNCAL HERNIATION
2. topical application of dilator, post ictal state, irreversible brainstem injury, epinephrine,
3. opiate ingestion, or pontine injury, coma, pilocarpine, horner syndrome, nerve gas, pesticides

Front 1072

oculocephalic manuver (coma eval)

Back 1072

patients eye turn back to midline after turning the head to the side , if brainstem damage has occured, head movement does not cause eye movement = (negative dolls head)

Front 1073

caloric irrigation (come eval)

Back 1073

10-30ml of ice water into the ear, eyes deviate toward irrigated side, defective response indicates pontine injury

Front 1074

abnoraml gag/corneal reflexes

Back 1074

BRAINSTEM INJURY

Front 1075

evalulation of comatose patient

Back 1075

1. ABCs
2. serum glucose
3. urine toxicology screen + serum electrolytes + metabolic panel
4. lumbar puncture to rule out meningoencephalitis
5. EEG to rule out seizures

Front 1076

definitions
1. seizure
2. epilepsy
3. status epilepticus

Back 1076

1. involuntary alternation of consiousness, motor activity, sensation or autonomic function caused by excessive discharge from a population of cerebral neurons (due to excessive excitatory, or deficient inhibitory activity)
2. >2 spontaneous seizures without obvious precipitating cause
3. seizure >30 minutes during which the patient doesnt regain consciousness

Front 1077

what percentage of children have a single afebrile seizure, what fraction go on to develop epilepsy

Back 1077

4-6 percent
<1/3 go on to develop epilepsy
epilepsy freq = 0.5-0.8% of the population

Front 1078

1. generalized seizure
2. tonic clonic seizure
3. absence seizures

Back 1078

1. discharge from both hemispheres - tonic, tonic clonic, clonic, myoclonic, absence, atonic
2. increased thoracic/abdominal tone --> clonic movement of arms/legs, incontinence, decreaesed consciousness, with POSTICTAL STATE*
3. staring spells that occur without loss of posture, only eye movements, lasts less than 15 seconds

Front 1079

list of seizure causes

Back 1079

hypocalcemia, hypoglycemia, hypomagnesemia, hyper/hyponatremia, pryixoxine deficeincy,
contusion, subdural hematoma
astrocytoma, meningioma
amphetamines, cocaine
meningitis, encephalitis, brain abscess, neruocysticercosis
cerebral infarction, intracerebral hemorrhage

Front 1080

partial seizures

Back 1080

discharge of only one hemisphere, seizures are predominantly motor, sensory, psychomotor
simple = no loss of consciousness
complex = consciousness is impaired

Front 1081

1. diagnosis of seizure
2. evaluation for patient with history of >1 afebrile seizures
3. evaluation of patient with febrile seizure

Back 1081

1. EEG, video EEG
2. serum electrolytes/neuroimaging
3. CNS infection by spinal tap, CBC, CXR, urine/blood culture

Front 1082

treatment of status epilepticus

Back 1082

intravenous anticonvulsants, short acting benzodiazepines followed by phenobarbitol or phenytoin

Front 1083

treatment of:
1. generalized epilepsy
2. absence epilepsy
3. partial epilepsy
4. medically intractable epilepsy

Back 1083

1. valproate or phenobarbital or phenytoin
2. ethosuximide or valproate
3. carbamazepine or phenytoin
4. surgery to remove epileptic tissue

Front 1084

non-pharmacological treamtnet for epilepsy

Back 1084

vagal nerve stimulator, ketogenic diet (suppresses seizure activity)

Front 1085

clinical presentation of febrile seizures (simple vs complex)

Back 1085

6 months - 6 years of age, FEVER >102.2
simple = <15 minute generalized seizure
complex = >15 minutes, or has focal features, or recurs within 24 hours

Front 1086

1. what fraction of patients that have one febrile seizure will have another
2. treatment of recurrent febrile seizures (should not treat an isolated febrile seizure)

Back 1086

1. 30%, 2% risk of epilepsy
2. rectal diazepam, prophylaxis with valproic acid or phenobarbital

Front 1087

infantile spasms (west syndrome)
1. pathogeneis + clinical presentation/age
2. EEG findings
3. tx
-poor prognosis, children develop intellectual disability

Back 1087

1. tuberous sclerosis***/PKU/hypoxia ischemia/intraventricular hemorrhage, meningitis, encephaltis 3-8 months of age --> brief myoclonic jerks consisting of sudden arm extensions or head/trunk flexion (jackknife or salaam seizures), clusters of 5-10 seizures over 3-5 minutes
2. hypsarrythmia pattern, with disorganized pattern of high amplitude spikes
3. ACTH***, valproic acid, vigabatrin

Front 1088

absence epilepsy of childhood
1. pathogenesis + clinical presentation/age
2. EEG
3. tx
-good prognosis

Back 1088

1. 5-9 years, AUTO DOM w/ age dependent penetrance ---> absence seizures lasting 5-10 seconds occuring frequently w/ automatisms NO LOSS OF POSTURE/INCONTINENCE/POST ICTAL STATE
2. 3Hz spike and wave discharge from both hemispheres (generalized)
3. ethosuximide or valproate

Front 1089

benign rolandic epilepsy (benign centrotemporal epilepsy)
1. pathogenesis + clinical presentation/age
2. EEG + TX
*good prognosis

Back 1089

1. AUTOSOMAL DOMINANT 3-13 years of age --> early morning hours, with oral/buccal manifestations (moaning, grunting, pooling or saliva) --> tonic clonic seizures
2. spike and sharp wave disturbance in the mid temporal/central regions
tx= valproic acid, carbamezipine

Front 1090

headaches
1. primary headache
2. secondary headache

Back 1090

1. neuron or muscular in origin
2. increased ICP, meningeal irritation (meningitis/ subarrachnoid hemorrhage)

Front 1091

extracranial headache causes

Back 1091

sinusitis, perioral abscess, toothache, chronic otitis, refractive errors

Front 1092

systemic causes of headache

Back 1092

anemia, hypoglycmeia, depression, hypretension, CO poisoning

Front 1093

migraine (MCC headache in children)
1. pathogenesis + clinical presentaation

Back 1093

1. AUTOSOMAL DOMINANT changes in cerebral blood flow DUE TO SERATONIN (5HT) RELEASE, SUB. P--> +/- AURA -->unilateral THROBBING headache associated with N/V, visual changes, streaks of light (fortifications), photo/phonophobia, normal neurologic examination

Front 1094

1. migraine aura
2. migraine equivalent
3. ophthalmologic migraine
4. basilar artery migraine

Back 1094

1. preceeding change of vision, or unilateral paresthesias or weakness
2. no actual headache, but alteration of behavior, cyclic vomitting, or paroxysmal vertigo
3. unilateral ptosis or CN3 palsy + migraine
4. vertigo, tinnitus, ataxia, dysarthria precedes migraine

Front 1095

migraine treatment

Back 1095

sumatriptan - 5HT agonist
propranolol - prophylaxis

Front 1096

tension headaches
1. pathognesis
2. treatment

Back 1096

1. muscle contraction --> bifrontal or diffuse dull aching, rarely throbbing increases intensity during day, contraction of temporalis, masseter, trapezius, NO VOMITTING, VISUAL CHANGES, OR PARESTHESIAS
2. acetaminophen, ibuprofen, stress/anxiety reduction

Front 1097

1. cluster headaches
2. tx

Back 1097

unilateral frontal or facial pain + conjunctival erythema, lacrimation, nasal congestion last less then 30 minutes but recur several times a day then go away for several months
tx = sumatriptan, prophylactic CCB/valproate

Front 1098

definitions
1. ataxia
2. cerebellar dysfunction gait
3. weakness in gait
- also encephalopathy, seizures +post ictal, vision problems, migraines, acute labyrinthitis, brainstem tumors

Back 1098

1. inability to coordinate muscle activity during voluntary movement
2. unsteady wide based gait, irregular steps/veering
3. spinal cord lesions, or disorders of the motor unit

Front 1099

acute cerebellar ataxia of childhood
-MCC ataxia in children
1. pathogenesis + clinical presentation
2. dx / tx

Back 1099

1. varicella, influenza, EBV, mycoplasma--> 2-3 weeks -->AUTOIMMUNE/POST INFECTIOUS--> truncal ataxia, slurred speech/nystagmus, afebrile
2. dx of exclusion, NORMAL HEAD CT SCAN
tx = supportive, completely resolves in 2-3 months

Front 1100

guillian barre syndorme
1. pathogenesis + clinical presentation

Back 1100

1. c.jejuni**, EBV, HSV, influenza, varicella, coxsackie --> GI illness-->resolution--> CELL MEDIATED IMMUNE RESPONSE TO SCHWANN CELL MEMBRANES -> demyelination of ventral spinal roots + peripheral myelinated neurons --> ASCENDING SYMMETRIC PARALYSIS + RESPIRATORY DISTRESS, lower back pain, leg discomfort, no loss of sensory, cranial nerve involvment

Front 1101

miller fisher syndrome

Back 1101

varient of guillian barre -- ophthalmoplegia, ataxia, areflexia

Front 1102

guillain barre dx/tx

Back 1102

dx - lumbar puncture shows albuminocytologic dissociation*** (inc. CSF protein*, but normal cell count)
EMG - dec. conduction velocity
spinal MRI to rule out compression

Tx = IVIG, plasmaphoresis, steroids

Front 1103

sydenham chorea (St. Vitus dance)
-25% of people with rheumatic fever
1. pathogenesis + clinical presentation
2. dx / tx
- good prognosis, lasts from several months to 2 years

Back 1103

1. rheumatic fever --> autoimmune antibodies cross react with group A strep and BASAL GANGLIA CELLS --> chorea (face/hands), jerky speech, chameleon tongue, milkmaids grip, no change in gait/cognition
2. ASO/anti DNase B titers, increased T2 signal in the caudate/putamen, SPECT shows inc. perfusion of thalamus/striatum
tx = haloperidol, valproic acid, phenobarbital

Front 1104

what conditions cause chorea

Back 1104

syndhams chorea
encephalitis
kernicterus
SLE
huntingtons disease
wilsons disease

Front 1105

tourettes syndrome
1. pathogenesis
2. tx

Back 1105

1. unknown/genetic --> motor/phonic tics(sterotypical behaviors), coprolalia, learning disabilities, ADHD, obsessive-compulsion
2. pimozide**, clonidine (se=sedation), haloperidol, hypnotherapy

Front 1106

duchennes/beckers muscular dystrophy
1. pathogenesis
2. clinical presentation

Back 1106

1. X linked recessive dystrophin mutation -->weakness/rupture of muscle plasma membrane --> degeneration/regeneration + infiltration with lymphocytes-->replacement of muscle fibers with fibroblasts/lipids
2. begins age 2-5 with progressive weakness starting in the legs --> loss of ability to walk by age 10 (20 for BMD), pseudohypertrophy of the calves, gowers sign (proximal hip muscle weakness), cardiomegaly/cardiac failure, mild cognitive impairement in DMD

Front 1107

DMD/BMD
1. dx/tx
- DMD death from resp. failure in late teens
-BMD - wheelchair by ~ 20 years of age

Back 1107

1. enlarged calf muscles with weakness, high CK,
EMG shows polyphasic muscle potentials with normal condution
muscle biopsy with dystrophic pattern
absent or decreased dystrophin
tx = corticosteroids early in disease (mild improvement)

Front 1108

myasthenia gravis
1. pathogenesis
2. dx/tx
*60% children remission after thymectomy
*symptomatic tx. for adults b/c it is self limited

Back 1108

1. autoimmune antibodies against Ach-R --> hypotonia, weakness, poor feeding, BILATERAL PTOSIS, increasing weakness later in the day, DIPLOPIA, preserved DTRs,
2. tensilon test - IV edrophonium (acetylcholinesterase inhibitor) transiently increases strength
decremental response to low frequency repetitive nerve stimualtion
AChR antibody titers
TX = pyridostigmine (cholinesterase inhibitory), corticosteroids , plasmaphoresis, IVIG, thymectomy

Front 1109

neonatal myasthenia

Back 1109

transient myasthenia from transplacental transfer of maternal antibodies to the fetus

Front 1110

when after birth does Hgb reach its lowest point (nadir)

Back 1110

2-3 months of age, and 1-2 months in preterm infant

Front 1111

what does a low retic count in the context of anemia indicate

Back 1111

bone marrow failure or diminished hematopoiesis (red cell aplasias, pancytopenia, malignancy)

Front 1112

iron deficinecy anemia
1. pathogenesis + clinical presentaiton
2. lab findings

Back 1112

1. birth blood loss/cows milk/adolescent menstruation/poor diet--> low iron --> hypochromic microcytic anemia --> pallor, poor weight gain, weakness/fatigue, tachycardia tachypnea, systolic murmur, CHF, dilated cardiomyopathy, SOB, HSM spoon shaped nails, dec. ability to learn
2. low ferritin, low transferrin, high transferrin saturation, high TIBC, high free erythrocyte protoporphyrin, high reticulocytes (surprisingly)

Front 1113

Iron deficiency anemia
1. treatment

Back 1113

1. elemental iron taken with orange juice (vitamin C enhances intestinal absorbtion)

Front 1114

alpha thalassemia
-asian demographics
1. pathogenesis + clinical presentation (based on # of deletions)

Back 1114

1. deletion(s) in alpha globin chain (total 4 genes) -->
- one deletion - silent carrier, no anemia
-two deletions- alpha thalassemia minor - mild anemia
- three deletions- HbH disease- severe anemia at birth, elevate Hgb Barts (tetramers of beta chains, severe lifelong anemia
-four deletions - only Hgb Barts, fetal hydrops - profound anemia, CHF/death

Front 1115

beta thalassemia
-Mediterranean demographics
1. pathogenesis + clinical presentation (based on # of deletions)
2. complications

Back 1115

1. deletion of beta globin genes -->
-one deletion - mild asymptomatic anemia, hypochromic microcytic anemia, target cells, anisocytosis
-two deletions - HSM, extramedullary hematopoiesis, thalassemia (chipmunk) facies, target cells, poikilocytes, indirect hyperbilirubinemia, high serum iron, high LDH, low HbA, high HbF
2. hemochromatosis (heart, liver, lungs, pancreas, skin) from transfusions

Front 1116

treament of beta thalassemia major/minor

Back 1116

major - splenectomy, lifelong transfusions, desferoxamine (tx. for hemochromatosis)

minor - NO IRON SUPPLEMENTATION (often misdiagnosed as IDA)

Front 1117

sideroblastic anemia
1. pathogenesis

Back 1117

1. alcohol, lead, isoniazid, chloramphenicol -->accumulation of iron in the mitochondria --> RINGED SIDEROBLASTS

Front 1118

anemia of chronic disease

Back 1118

malig/infection/kidney disease/lead poisoing-->microcytic hypochromic anemia
hypochormic microcytic anemia, low iron, low TIBC

Front 1119

folic acid deficieny anemia
1. pathogenesis

Back 1119

1. low fruits/vegetables, goat milk**, celiac disease, infectious enteritis, crohns, anticonvulsants, OCP --> dec. folate levels --> megaloblastic (macrocytic)anemia --> sx. of anemia, FTT, chronic diarrhea, irritability

Front 1120

b12 anemia
1. normal physiology
2. pathogenesis + clinical pres

Back 1120

1. B12 + intrinsic factor (from parietal cells)--> absorption in the terminal ileum
2. strict vegan/pernicious anemia(no GIF)/crohns (term.ileum)--> inability to absorb B12-->smooth red tongue, combined system degeneration (ataxia, hyporeflexia, + babinski)

Front 1121

what conditions have a high reticulocyte count

Back 1121

hemolytic anemia, sickle cell anemia (increased marrow production)

Front 1122

hereditary spherocytosis
-eastern europeans
1. pathogenesis + clinical presentaiton
2. dx
3. tx

Back 1122

1. AUTOSOMAL DOMINANT mutation in SPECTRIN --> spherical shape RBC --> splenomegaly , pallor, pigmented gallstones, aplastic crisis (assc. Parvo B19), jaundice/anemia in infants
2. spherocytes on smear, INC. OSMOTIC FRAGILITY
3. splenectomy

Front 1123

pyruvate kinase deficiency
1. pathogenesis+ clinical presentation
2. dx
3. tx

Back 1123

1. AUTOSOMAL RECESSIVE pyruvate kinase deficiency --> decreased ATP, and dec. RBC survival--> pallor, jaundice, splenomegaly, kernicterus
2. polychromatic RBCs, PK activity of RBCs
3. transfusions/splenectomy

Front 1124

glucose 6 phosphate DH deficinecy
1. pathogenesis+ clinical presentation
2. dx
3. tx

Back 1124

1. X linked G6PD deficiency (low reduced glutathione) :: fava beans, infection, sulfa/salacylates/antimalarials --> oxidative damage --> hemolysis--> abdominal pain, V/D, fever, hemoglobinuria/jaundice, HSM
2. hemoglobinuria, inc. Retics, bite cells, heinz bodies, hemighosts, low G6PD
3. transfusions

Front 1125

autoimmune hemolytic anemai (AIHA)
1. pathogenesis+ clinical presentation
2. dx
3. tx

Back 1125

1. idiopathic/viral/lymphoma/SLE/immunodeficiency --> autoantibodies against RBCs--> anemia, pallor, jaundice, hemoglobinuria, splenomegaly -->complete recovery
~ prolonged form has high mortality
2. DIRECT COOMBS TEST POSITIVE (antibodies positive, complement positive directed toward RBCs)
3. corticosteroids (most effective for acute form)

Front 1126

alloimmune hemolytic anemia
1. Rh hemolytic disease
2. tx

Back 1126

1. Rh(-) mother produces antibodies to Rh(+) fetus during first pregnancy ~~~ second pregnancy mothers IgG anti-Rh crosses placenta causing severe jaundice/kernicterus, anemia, hepatosplenomegaly, hydrops fetalis
2. phototherpay + RHOGAM for the mother during first pregnancy or after blood transfusion

Front 1127

alloimmune hemolytic anemia
1. ABO hemolytic anemia
2. tx

Back 1127

1. mother is blood group O, and fetus is A,B or AB mother produces IgG anti-AB which passes through placenta
~ occurs during FIRST PREGNANCY**** (direct coombs positive)
2. phototherapy

Front 1128

sickle cell anemia
1. pathogenesis+ clinical presentation
2. dx
3. tx

Back 1128

1. autosomal recessive substitution position 6 beta globin chain glutamate to valine--> stacking of HbA in low oxygen/acidotic condtions --> distorted shape/hemolysis and OCCLUSION OF SMALL VESSELS--> distal ischemia, infarction, organ dysfunction
2. fetal newborn Hgb electrophroesis

Front 1129

microangiopathic hemolytic anemia
1. pathogenesis+ clinical presentation + dx

Back 1129

1. HUS, TTP, DIC, artificial heart valves, giant hemangioma --> mechanical damage to RBCs -->burr cells, target cells, iregularly shaped cells, helmet cells, thrombocytopenia

Front 1130

when does sickle cell symptoms begin

Back 1130

at 6 months of age when HbF declines

Front 1131

sickle cell clinical features
1. vasocclusive crisis + tx
2. acute abdominal crisis + tx
3. stroke
4. priapism
5. acute chest syndrome
6. sequestration crisis

Back 1131

1. ischemia/infarction of bones, dactylitis, pain, 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry
2. sickling in the mesenteric artery --> abdominal pain/distension + 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry
3. dysarthrisa, hemoplegia + 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry
4. painful sustained erection + 2x IV maintenance fluids, partial exchange transfusion, incentive spirometry
5. mycoplasma, chlamydia, s.pneumo --> new pulmonary infiltrate with respiratory symptoms, hypoxemi + 2x IV maintenance, oxygen, cefuroxime/azithro, incentive spirometry, partial exchange transfusion
6. rapid accumulation of blood in the spleen --> abdominal distension/pain, SOB, tachycardia, pallor, fatigue, shock

Front 1132

1. sickle aplastic crisis
2. hyperhemolytic crisis

Back 1132

1. cessation of RBC production after parvo B19 --> low HbA, low reticulocytes
2. rapid hemolysis in aptients with G6PD, dec. HbA, high reticulocytes

Front 1133

1. what causes increased risk of death from infection in patients with sickle cell disease
2. what kind of osteomyelitis in patients with sickle cell disease

Back 1133

1. autosplenectomy leads to risk for infection with encapsulated bacteria, HITB, s.pneumo, n.meningitidis, salmonella
2. salmonella osteomyeliits from the GI tract, and s.aureus osteomyelitis

Front 1134

sickle cell disease
1. RBC lifespan
2. retic count
3. WBC count
4. platelet cell count
*5. peripheral smear

Back 1134

1. 10-50 days
2. 5-15%
3. 12-20k
4. increased >500k
5. sickles, targets, howell jolly bodies

Front 1135

treament of sickle cell anemia
1. hydroxyurea+
2. penicillin
3 trans cranial doppler/MRI

- daily folate + routine imuizations

Back 1135

1. chemotherapeutic inc. HbF
2. prophylaxis for s.pneumo
3. to monitor for stroke

Front 1136

complications of sickle cell dieseae

Back 1136

delayed growth, cor pulmonale, gallstones, poor wound healing, avascular necrosis of the femoral/humoral heads

Front 1137

congenital hypoplastic anemia (diamond blackfan)
1. pathogenesis + clinical pres
2. dx
3. tx

Back 1137

1. AR/AD inheritance--> pure red cell aplasia--> rapid onset anemia in first year of life, craniofacial, renal, cardiac abnormalities, triphalangeal thubs
2. decreased Hgb, dec. retics, inc. HbF, dec RBC precursors in the marrow
3. RBC transfusion, corticosteroids, BMT

Front 1138

transient erythroblastopenia of childhood
1. pathogenesis + clinical pres
2. dx
3. tx

Back 1138

1. *post viral autoimmune --> slow onset symptomatic anemia
2. low Hgb, low retics, dec. RBC precursors in the marrow
3. no treatment, spontaneous recovery

Front 1139

parvo B19 pure red cell aplasia
1. pathogenesis + clinical pres
2. dx
3. tx

Back 1139

1. parvovirus (also EBV/CMV/HIV/chlorampheicol) --> URI symptoms/slapped cheeks fifth disease--> aplastic crisis in SS disease, ASYMPTOMATIC ANEMIA IN HEALTHY CHILDREN
2. dec Hgb, dec. retics, normal plateltes
3. sponatneous recovery in 2 weeks, RBC transfusion for SS

Front 1140

fanconi anemia
1. pathogenesis + clinical pres
2. dx
3. tx

Back 1140

1. Autosomal recessive --> marrow failure at age 7 ***presents with ecchymosis/petechiae** short stature, hypoplasia of the thumb* and radius, skin hyperpigmentation, renal abnormalities
2. pancytopenia, RBC macrocytosis, low reticulocyte count, elevated HbF, bone marrow hypocellularity
3. transfusions/platelets, BMT, corticosteroids

Front 1141

acquired aplastic anemia
1. pathogenesis + clinical pres
2. dx
3. tx

Back 1141

1. sulfonammides,anticonvulsants, HIV/EBV/CMV, chemicals, radiation, idiopathic--> brusing, petechiae, pallor, neutropenia/infection
2. pancytopenia, low retics, hypocellular marrow
3. stop causative agent, BMT, immunosuppressants

Front 1142

polycythemia
1. definition
2. primary polycythemia (vera)
3.appropriate polycythemia
4. inappropriate polycythemia

Back 1142

1. increase in relative RBCs, Hct >60%
2. malignancy of RBC precursors
3. CHF, pulmonary disease, cyanotic heart disease, high altitude living
4. tumors of the kidney, cerebellum, ovary,liver, adrenal, hydronephrosis, corticosteroids,

Front 1143

relative polycythemia

Back 1143

1. apparant increase in RBC mass caused by decrease in plasma volume *** DEHYDRATION

Front 1144

complicatiosn of polycythemia

Back 1144

thrombosis (vasoocclusive, stroke, MI), bleeding

Front 1145

clotting abnormality lab evaluation

Back 1145

CBC, platelet count, blood smear, PTT, PT, platelet function assay

Front 1146

hemophilia A
1. pathogenesis + clinical pres
2. dx
3. tx

Back 1146

1. X linked recessive factor VIII defieciency --< hearthrosis, DEEP soft tissue bleeding, life threatening hemorrhage
2. prolonged PTT, normal PT, normal bleeding time/platelet count/platelet function, low factor VIII activity
3. recombinant factor VIII, DDAVP (inc. release of stored VIII)

Front 1147

pg 408 chart of PT/PTT/bleeding time + ddx. hemostasis

Back 1147

....

Front 1148

von willibrands disease
1. pathogenesis (types 1/2/3)
2. clinical presentation
3. treatment

Back 1148

1. defects in vWF portion of factor VIII complex -->
Type1 = quantitative deficiency of vWF/VIII
Type 2 = qualitative abnormalitiy in vWF
Type 3 = absesne of vWF
2. mucocutaneous bleeding, epistxis, menorrhagia, dental bleeding, NO HEMARTHROSES
3. DDAVP (Type 1/2), cryoprecipitate (used during surgery, bleeding, or type III disease)

Front 1149

factor IX deficiency
1. pathogenesis
3. treatment

Back 1149

1. X linked disorder, similar to VIII deficiency, PTT and PT --
2. treat with recombinant factor IX

Front 1150

vitamin K deficiency
1. pathogenesis
2. clinical presentation
3. dx/tx

Back 1150

1. rifampin/isoniazid/warfarin/cephalosporins infant deficiency, pancreatic insuff, biliary obstruction --> cannot synth 2/7/19/10 protein C/S
2. bruising, oozing from punctures, bleeding into organs
3. prolonged PTT and PT, normal bleeding time (no problem with platelets)

Front 1151

1. hemorrhagic disease of the newborn
2. dx/tx

Back 1151

serious bleeding from vitamin K deficiency - presents only with cutaneous bleeding and bleeding from circumcision site or umbilical cord
2. dx = prlonged PT and PTT
tx = intramuscular vitamin K at birth***, FFP, platelets

Front 1152

liver disease coagulopathy
1. pathogenesis
2. clinical presentation
3. dx/tx

Back 1152

1. liver diseaes--> dec clotting factor synth especially vitamin K dependent factors
2. same as vitamin K def
3. dx = prolonged PT and PTT, increased fibrin degradation products, thrombocytopenia
tx = vitamin K, FFP, platelets

Front 1153

DIC
1. pathogenesis
2. clinical presentation
3. dx/tx

Back 1153

1. large hemangiomas, sepsis, hypothermia, malignancy, heat stroke, snake bites, burns-->clotting/consuption of procoagulant factors -->HEMORRHAGE
2. cutaenous and internal bleeding
3.dx = thrombocytopenia (prolonged BT), prolonged PT and PTT, reduced clotting factors, low fibrinogen*, low II,V,VIII, ELEVATED D-DIMERS (fibrin degradation products)+ smear shows schistocytes and helmet cells
tx = fibrinogen, FFP, platelets, heparin

Front 1154

kasabach-merritt syndrome

Back 1154

large hemangiomas --> MAHA, thrombocytopenia, DIC

Front 1155

hereditary telangiectasisa

Back 1155

1. autosomal dominant locally dilated totuous veins/capilaries of the skin/mucus membranes

Front 1156

scurvy

Back 1156

vitamin C deficiency --> weakness of collagen especially in blood vessels, poor wound healing, diffuse tenderness in the legs, petechiae/ecchymosis (fragility of BV)

Front 1157

wiskott aldrich syndrome
1. pathogenesis / clinical presentation
2. associated cancers

Back 1157

1. X linked defect in actin polymerization--> thrombocytopenia*, small platelets, eczema*, T/B cell immune defects
2. lymphoma, leukemia

Front 1158

thrombocytopenia-absent radius syndrome (TAR)
1. pathogenesis / clinical presentation

Back 1158

1. thrombocytopenia (improves by age 2-3), limb abnormalities (no radius), there is thumb present (compare to fanconi), cardiac and renal disease

Front 1159

ITP
1 pathoenesis
2. clinical pres
3. dx . tx
~ resoleves spontaneously,

Back 1159

1. idiopathic*, drug induced, 1-4 weeks post-viral** --> antibodies against platelets --> destruction/removal by spleen
2. abrupt cutaneous bleeding (epistaxis/gum bleeding), internal bleeding into the brain/kidneys/GI tract (Rare)
3. thrombocytopenia +LARGE STICKY PLATELETS platelets of blood smear
tx = IVIG, corticosteroids, Anti-D immunoglobulin (binds Rh on RBCs allowing platelets to escape the spleen), splenectomy~~~~ no platelet transfusions

Front 1160

neonatal ITP
1. pathogensis

Back 1160

1. mother has ITP and antibodies against platelets cross placenta and destroy fetal platelets, mother also has thrombocytopenia

Front 1161

glanzmanns thrombasthenia

Back 1161

autosomal recessive defect in gpIIb/IIIa leads to decreased platelet aggregation

Front 1162

bernard souliers + blood smear findings

Back 1162

autosomal recessive disorder of gpIb platelets cannot adhere to vWF on endothelium --> severe hemorrhage
~ smear shows large platelets

Front 1163

protein C/S deficiency
1 pathogenesis + clinical pres (hetero/homo)
2. dx/tx

Back 1163

1. AR/AD deficiency -->
homozygotes- purpural fulminans= nonthrombotic cytopenic purpura, fever, shock, rapid skin bleeding/intravascular thrombosis
heterozygtes - DVT or CNS thrombosis
2. protein C/S testing,
tx = heparin, FFP, warfarin, purified protein C

Front 1164

which drugs inhibit platelet function

Back 1164

aspirin, valproic acid

Front 1165

what metabolic conditions effect platelet funciton

Back 1165

uremia, and liver disease

Front 1166

1. what hemostatic conditions cause hypercoagulability
2. what disease states cause hypercoagulability

Back 1166

1. protein S/C deficiency, antithrombin C deficiency, factor V leiden
2. sickle cell, malignancy, inflammatory disease, liver disease, nephrotic syndrome, ulcerative colitis, dehydration, vascultiis, diabetes, homocystinuria

Front 1167

neutropenia
1. types of infections with mild neutropenia (1000-1500)
2. types of infections with severe neutropenia (<500)

Back 1167

1. infection of mucuous membranes: stomatitis, cellulitis, gingivitis
2. pneumonia, sepsis, meningitis (s.aureus, klebsiella, serratia, e.coli, pseudomonas)

Front 1168

what infectoius agents cause neutropenia

Back 1168

HIV, EBV, CMV, hep A/B, influenza A, parvo B19
typhus, rocky mountain spotted fever, malaria

Front 1169

chronic benign neutropneia of childhood
- <4 years of age
1. pathogenesis/clinical pres
2. dx/tx

Back 1169

1. acquired/inherited non-cyclic neutropenia---> mild infections (otitis,sinusitis,pharyngitis, cellulitis)
2. low neutrophils, normal WBCs, bone marrow shows immature neutrophil precursors
tx = nothing, resolves spontaneously

Front 1170

kostmann syndrome

Back 1170

congenital agranulocytosisis, frequent life threatening pyogenic infections, ANC <300

Front 1171

cyclic neutropenia

Back 1171

21 day cyclic alterations in neutrophil counts --> episodes of neutropenia-->

Front 1172

chediak higashi syndrome
1. pathogensis + clinical presentation
2. tx

Back 1172

1. auto recessive --> oculocutaneous albinishm, blond/brown hair with silver streaks azurophilic granules in neutrophils/dec. degranulation, peripheral neuropathy, neutropenia, thrombocytopenia ~~ recurrent infections with s.aureus
2. TMPSMX prophylaxis, daily ascorbic acid

Front 1173

cartilage hair hypoplasia syndrome

Back 1173

Auto recessive - short stature, immunodeficiency, fine hair, neutropenia

Front 1174

schwachman diamond syndrome

Back 1174

exocrine pancreatic insufficeincy (chronic diarrhea), short stature, (metaphyseal chondroplasia) neutropenia

Front 1175

what drugs cause neutropenia

Back 1175

antibiotics, anticonvulsants, aspirin, radiation, chemotherapy

Front 1176

what metabolic diseases cause neutropenia

Back 1176

hyperglycine*mia, methylmalonic acidemia, gauchers disease

Front 1177

autoimmune neutropenia

Back 1177

EBV/drugs/SLE/juvenille rheumatoid arthritis --> anti-neutrophil antibodies --> neutropenia

Front 1178

isoimmune neutropenia

Back 1178

1. passive transfer of anti-neutrophil antibodies from mother to fetus after maternal sensitization by antigens on fetal neutrophils ~~~ resolves in infant by 8 weeks of life

Front 1179

sickle cell trait (HbA/S) clinical presentation

Back 1179

1. no anemia, no crisis (EXCEPT severe hypoxemia), normal spleen, no prophylactic penicillin
****ONLY CLINICAL MANIFESTATION = INABILITY TO CONCENTRATE URINE OR HEMATURIA

Front 1180

treatment for sickle cell bone crisis (vasoocclusive crisis)

Back 1180

pain control + high volume 1.5-2x maintenance IV fluids

Front 1181

clinical presentation of childhood cancer

Back 1181

**persistent fever, weight loss, night sweats, fatigue~~~ assc. with leukemia, lymphoma and other cancers
**palpable mass
**abdominal mass
**mass on trunk or extremities

Front 1182

what tumors present as an abdominal mass

Back 1182

WILMS TUMOR
NEUROBLASTOMA

Front 1183

what does bone pain indicate in a child with cancer

Back 1183

metastatic disease, primary tumor of bone/connective tissue, or leukemic infiltration

Front 1184

causes of supraclavicular lymphoadenopathy

Back 1184

leukemia, lymphoma, metastatic disease

Front 1185

early morning headache and vomiting in a child

Back 1185

space occupying tumor in the CNS

Front 1186

bruising, petechiae, pallor in a child with cancer

Back 1186

infiltration of the bone marrow

Front 1187

leukocoria

Back 1187

RB, cataract, vitreous hemorrhage

Front 1188

hypertension in a child with cancer

Back 1188

wilms tumor, pheochromocytoma, neuroblastoma

Front 1189

ALL
- MCC childhood cancer, peak 2-6 years of age
- long term survival 85% of patients**
1. pathogenesis
2. cell morphology/types
3. Pre-B cell subtyping

Back 1189

1. unknown/down syndrome, bloom syndrome/chemical agents/immunodeficiency --> lymphoblast proliferation
2. Pre B cell (80%) >T cell>B cell
L1/L2/L3 ~ L1 = small cells with little cytoplasm, L3 = large cells > 1 nucleoli
3. common acute lymphocytic leukemia antigen (70% + or -)

Front 1190

ALL
4. clinical presentation
5. lab diagnosis

Back 1190

4. fever, bone/joint pain/refusal to bear weight, pallor, bruising, hepatosplenomegaly, lymphadenopathy
+/- epistaxis, anorexia, fatigue, abdominal pain
5. CBC: leukemic blasts in blood, high 1/3 >50k, 1/3 normal, 1/3 <10k **MAY BE NORMAL, thrombocytopenia, anemia
Marrow tap --> replacement of marrow by lymphoblasts

Front 1191

favorable prognostic factors for ALL

Back 1191

1-9 years of age
female
white
<50k wbcs
hyperploidy
no organ involvment/ no mediastinal mass
CALLA(+)
no chromosomal translocation (9;22 is bad)

Front 1192

ALL
6. tx
7. treatment of relapse

Back 1192

6. induction - corticosteroids, vincristine, L-asparginine, **intrathecal methotrexate
consolidation - **intrathecal methotrexate, cranial irradiation (children>5yo)
maintenance - daily/periodic chemo up to 3 years and discontinued after 2-3 years of disease free
7. BMT

Front 1193

ALL
8. infectious complications of ALL
9. what does fever >1 week while on antibiotics indicate
10. tumorlysis syndrome

Back 1193

8. neutropenia (<500) + assc sepsis - s.aureus, s.epidermidis, + oppurtunistic: pseudomonas, HSV, PCP, candida, aspergillus
9. fungal infection
10. hyperuricemia/hyperkalemia/hyperphosphatemia-->hypocalecmia~~ from rapid death/lysis of tumor cells

Front 1194

ALL chemotherapy side effects
1.asparaginase and corticosteroids
2. doxorubicin
3. cyclophosphamide
4. cranial irradiation

Back 1194

1. pancreatitis
2. cardiomyopathy
3. cystitis
4. mental retardation, stroke, hormonal problems

Front 1195

AML
15-20% of childhood leukemias
1. pathogenesis
2. M1-M7
3. clinical presentation

Back 1195

1. UNKNOWN/down syndrome/fanconi/kostmann/neurofibromatosis/ionizing radiation
2. acute myeloblastic, acute myeloblastic some maturation, acute promyelocytic, acute myelomonocytic, acute monocytic, erythroleukemia, acute megakaryocytic
3. similar to ALL but with more CNS involvment~ fevers, HSM, gingival hypertrophy, bone pain, DIC******

Front 1196

AML
1. diagnosis
2. treatmnet
-50% remission from chemotherapy, 70% cure rate with sibling bone marrow transplant

Back 1196

1. blood smear showing leukemic myeloblasts, AUER RODS, bone marrow biopsy
2. intense myeloablative therapy to cause remission, BMT after remission from HLA matched donarCML

Front 1197

CML
1. adult type CML pathogenesis
2. adult type clinical presentation
3. juvenille CML pathogenesis
4. juvenille CML clinical presentation

Back 1197

1. philidelphia chromosom t(9;22) BCR-Abl fusion kinase
2. fatigue, weight loss MASSIVE splenomegaly, WBC>100k***
3. abnormalities of chromosome 7 or 8, no BCR-abl
4. chronic eczema like facial rash, suppurative lymphadenopathy, petechiae/purpura, leukocytosis <100k, anemia, thrombocytopenia

Front 1198

1. treatment of CML
adult CML - chronic phase - contorlled by chemo/BMT-->blast crisis acute leukemia -->unresponsive to therpay
juvenille CML often fatal, relapse in 50% of cases

Back 1198

BMT - for both adult/juvenille

Front 1199

hodgkins disease
1. pathogenesis + clinical presentation
2. is extranodal/SVC syndrome/airway compression common in hodgkins disease
3. dx/tx
4. complications of therapy
- 60-70% survival

Back 1199

1. EBV infection --> infection of APCs --> REED STERNBERG CELLS- slow progressing painless, SYSTEMIC B SYMPTOMS, cervical/supraclavicular lymphoadenopathy, PROGRESSIVE COUGH (pulmonary symptoms is highly suggestive of hodgkins disease)
2. no
3. INITIAL CXR SHOWING MEDIASTINAL MASS lymph notde biopsy shows reed sternberg cells
tx =chemotherapy + radiation
4. growth retardation, secondary malignancies (breast, AML, non-hodgkins), hypothyroidism, male sterility

Front 1200

ann arbor staging system of lymphomas

Back 1200

stage 1 = single lymph node or extra lymphatic site
stage 2 = 2 or more node regions, same side of diaphragm, extension to extralymphatic site or extra nodal site + one lymph node same side of diaphragm
stage 3 = nodes both side of diaphragm (spleen counts)
stage 4 = diffuse disseminated of one or more extralymphatic organs/tissues
A = asymptomatic, B is symptomatic*****

Front 1201

non-hodgkins lymphoma
- males>females
1. pathogenesis + clinical presentation
3. lymphoblastic lymphoma - clinical pres
4. small non-cleaved cell lymphoma (two types) - clinical pres
5. large cell lymphoma - clinical pres

Back 1201

1. immunodeficiency states/HIV/wiskott aldrich/ataxia telangiectasia/EBV --> painless lymphadenopathy,
3. T cell - similar to lymphoblasts in ALL, presents as anterior mediastinal mass, SVC syndrome, airway obstrucion
4. often presents as intussuception***********
Burkitts presents as jaw mass
5. enlargment of lymphoid tissue of tonsils, adenoids, peyers patches

Front 1202

non-hodgkins lymphoma
1. dx/tx
- poor prognosis for disseminated disease, good prognosis for localized lymphoma

Back 1202

1. biopsy, liver enzymes, bone scan, CSF analysis
tx = surgery + chemotherpay

Front 1203

brain tumors
- most common solid dutmor
- 20% of childhood cancers
1. which conditions are associated with brain tumors
2. are supra or infratentorial glial tumors more aggressive
-astrocytomas
3. PNET
- also ependymomas, and craniopharyngiomas

Back 1203

1. VHL, neurofibromatosis, tuberous scleorsis
2. supratentorial
3. medulloblastomas

Front 1204

1. high grade vs low grade tumor

Back 1204

1. high grade = agressive, proliferative undifferentiated cels
low grade = less aggressive, more differentiated cells

Front 1205

most common infra/supratentorial tumor in children

Back 1205

1. infra = medulloblastoma
supra = astrocytoma

Front 1206

1. clinical presentation of brain tumors

Back 1206

inc intracranial pressure --> worse when supine during sleep/awakening: headache, vomiting, drowsiness, irritability, abnormal behavior, ataxia (cerebellar), seizures (supratentorial), head tilt

enlarged/bulging fontanelles, nystagmus, papilledema, 6th nerve palsy, lethargy/irritability

Front 1207

clinical presentation of
1. optic glioma
2. craniopharyngioma

Back 1207

1. decreased vision, decreased visual field, strabismus
2. growth retardation, delayed puberty (disruption of the pituitary), visual changes, central diabetes inspidus, precocious puberty, papilledema/hydrocephalous (50%), vomitting

Front 1208

1. diagnosis of brain tumor
2. brain tumor treatment
- astrocytomas 35%-75% survival (depends on grade)
-PNET - 75% survival
- brainstem gliomas - poorest prognosis

Back 1208

1. neuroimaging MRI>CT
CSF
2. surgical debulking, and radiation, chemotherapy

Front 1209

brain tumor CSF findings
1. AFP/B-HCG
2. HVA, VMA, polyamines

Back 1209

1. germ cell tumors
2. medulloblastoma

Front 1210

1. neuroblastoma origin
2. location
3. genetics

Back 1210

neural crest cell tumor that arises anywhere along the sympathetic ganglia chain and in the adrenal medulla
2. abdomen or pelvis, posterior mediatinum
3. unbalanced translocation between 1p and 17q, and 14q / 22q abnormalities

Front 1211

neuroblastoma
1. clinical presentation
2. diagnosis

Back 1211

1. firm abdominal mass(MCC), respiratory distress/anorexia, tracheal compression, horners syndrome, flushing, hypertension, headache, sweating, fever, weight loss
acute cerebellar atropy - ataxia/opsoclonus/myoclonus
metastasis - periorbital eccymosis/proptosis, blueberry muffin skin nodules, bone pain or limping, hepatomegaly
2. excessive urinary catecholamines - VMA, HVA, positive bone marrow/tissue biopsy
CT/MRI to find mets
skeletal survey or technetium 99m bone scan to find mets to bone

Front 1212

neuroblastoma staging

Back 1212

1. confined to structure of origin
2. extends beyond structure of origin but doesnt cross the midline
3. tumor extends past the midline
4. metastasis to bone/lymph nodes/ bone marrow/soft tissue
4s. localized tumor at stage 1 or two with distant mets to any organ but bone

Front 1213

1. neuroblastoma treatment
2. what are poor prognostic features of neuroblastoma

Back 1213

surgery alone for stage I/II
chemotherapy for metastatic disease (IV/IVs)
radiation for advanced diseaes
****IVs may spontaneously regress
2. N-myc III/IV, amplification of N-myc (chromo 2) diploidy, high levels of ferritin, lactic dehydrogenase, neuron specific enolase

Front 1214

Wilms tumor
~ presents age 2 to 5 years
1. associated conditions
2. clnical presentation

Back 1214

1. beckwith wiedeman/WAGR syndrome, chromosome 11 abnormalities--> most common renal tumor of childhood
2. CHILD WITH abdominal mass***, abdominal pain, hematuria****, hypertension(pressure on renal artery), renin secretion, fever/anorexia, weight loss, hemihypertrophy, aniridia

Front 1215

WAGR syndrome

Back 1215

wilms tumor, aniridia, genitourinary abnormalities, mental retardation

Front 1216

wilms tumor staging

Back 1216

1. limited to kidney + complete excision w/o rupture
2. tumor extends locally but can still be excised
3. residual tumor remains in abdomen, or spillage durign resection
4. bilateral renal involvment

Front 1217

rhabdomyosarcoma
-MCC soft tissue sarcoma in childhood
- higher risks in patients with NF1/2
1. clinical presentation
2. dx/tx

Back 1217

1. painless soft tissue mass, head and neck, orbital (propotosis/chemosis/CN palsies), nasopharyngeal (epistaxis/airway obstruciton), laryngeal (hoarsness) genitourinary tract (hematuria/obstruction), painless mass in the extremities
2. dx = painless enalrging mass, CT or MRI
tx = complete surgical resection

Front 1218

1. osteogenic sarcoma pathogenesis
-most common malignant bone tumor, peaks during puberty growth spurt
2. clinical associations
3. radiographic findings
4. sites of metastasis

Back 1218

1. Puberty during rapid bone growth--> tumor that forms osteoid (new bone) of the metaphysis** of long bones (proximal tibia/humerous/femur, distal femur) --> pain, swelling, soft tissue mass, no systemic symptoms***
2. retinoblastoma, pagets disease, radiation for cancer, fibrous dysplasia
3. periosteal reaction with sunburst appearance, lytic/destructive changes
4. lungs, bone

Front 1219

Ewing sarcoma
1. pathogenesis + clinical presentation
2. radiographic findings
3. sites of metastasis

Back 1219

1. adolescence chromosomal translocation (11;21) --> undifferentiated small blue cell tumor in flat bones and diaphysis of tubular bones- AXIAL SKELETON, humerous, femur --> pain, swelling, FEVER, MALAISE, WEIGHT LOSS, leukocytosis, inc. ESR`
2. periosteal reactoin with onion skinning
3. lungs, bone, bone marrow

Front 1220

1. what diagnostic tests must be done for suspected osteogenic sarcoma or ewings sarcoma
2. treatment for these two cancers
osteogenic sarcoma more common than ewings sarcoma

Back 1220

1. bone scan and chest CT to evaluate for mets
2. chemotherapy and surgical removal, also can use

Front 1221

what genetic condition is associated hepatoblastoma

Back 1221

beckwith wiedemann

Front 1222

1. what conditions is hepatocellular carcinoma associated with
2. clinical presentation
3. diagnosis
4. treatment
-poor prognosis,mostly non-resectable and mets to lungs, brain, lymph nodes

Back 1222

1. chronic hepatits B, biliary atresia, glycogen storage diseases, alpha 1 antitrypsin deficiency, hereditary tyrosinemia
2. right upper abdominal mass, loss of appetite, and weight loss
3. elevated ALPHA FETO PROTEIN, and CT/MRI of the liver
4. surgical resection, and chemotherapy (to make tumor operable)

Front 1223

sacrococcygel teratomas
1. clinical presentation + tx

Back 1223

1. usually in females, arises form the coccyx and forms soft tissue mass, benign and can be excised

Front 1224

1. anterior mediastinal teratomas clinical presentation + tx
2. ovarian teratomas clinical presentation + tx

Back 1224

1. benign, present with symptoms of airway obstruction
2. benign, finding calcium on the abdominal radiograph near the ovaries

Front 1225

1. testicular tumor frequency
2. treatment

Back 1225

1. yolk sac (60%)>teratomas> seminomas/embryonal carcinomas
2. radical orchiectomy/node dissection

Front 1226

what is the risk of cryptorchid testis

Back 1226

increased risk of testicular cancer

Front 1227

yolk sac tumor marker

Back 1227

alpha feto protein

Front 1228

ovarian tumors
1. what fraction are malignant
2. what fraction of testicular tumors are malignant
3. treatmnet

Back 1228

1. 1/3
2. 2/3
3. surgery, chemotherapy, radiation therapy

Front 1229

langerhans cell histiocytosis
1. diseases
2. clinical presentation
-also non-specific: fever, faitgue, FTT,
3. treatment

Back 1229

1. eosinophilic granuloma (focal), hand schuller christian, letterer siwe (disseminated)
2. skull most commonly involved, single or multiple bony lesions, chronic draining ears , skin involvement of the diaper area and scalp, pituitary/hypothalamic (growth retardation/DI/hypogonadism/panhypopituitarism)
3. coritcosteroids, local curettage, low dose radiation

Front 1230

anaphylaxis
1. pathognesis + clinical presentation
2. treatment

Back 1230

1. drugs/insect venom/foods/latex/biologic agents --> antigen binds to IgE on the surface of mast cells/basophils -->release of vasoactive substances--> RAPID pruritus, urtiaria, angioedema, dyspnea/wheezing, N/V, hypotension/shock
2. EPINEPHRINE (resp/cardio), antihistamines, corticosteroids, B-agonists

Front 1231

allergic rhinitis
1. pathogenesis + clinical presentaiton
2. seasonal rhinitis
3. perennial rhinitis
4. dx.
5. nasal smear findings

Back 1231

1. IgE mediated inflammatory response in the nasal mucosa to inhaled antigens
2. trees, grass, weed pollens (grass in spring, ragweed in fall)
3. indoor allergens - dust mites, animal dander
4. total IgE, allergen skin testing (no antihistamines before testing)
5. 10% eosinophils indicates rhinitis, high levels of PMNs suggests infectious not allergic

Front 1232

clinical features of allergic rhinitis

Back 1232

sneezing, nasal congestion, rhinorrhea, pale nasal mucosa
allergic shiners- dark circles under eyes
dennies lines - creases under eyes from chronic edema
allergic salute - use palm of hand to elevate tip of nose to help itching

Front 1233

what is the clinical associations with allergic rhinitis

Back 1233

asthma, chronic sinusitis, otitis media w/ effusion, nasal polyps, atopic dermatitis

Front 1234

treatment of allergic rhinitis

Back 1234

intranasal steroids (SE = local irritation, no systemic effects on HPA axis)
Antihistamines - diphenhydramine ,cetirizine, fexofenadine, loratadine
cromylyn sodium
psuedoephedrine - insomnia, nervousness, rebound rhinitis,

Front 1235

immunotherapy for allergy

Back 1235

1. repeated exposure to antigens leads to better tolerance
~ use when other therapies are ineffective, environmental controls have failed

Front 1236

atopic dermatitis (eczema)
- commonly runs infamilies
1. clinical presentation
2. diagnostic criteria
3. management

Back 1236

1. inflammatory dermatitis characterized by dry skin and lichenification, worse with extremes of temperature, pruritus, erythema, weeping, crusting-- tends to effect FLEXOR SURFACES
2. pruritus, personal/family hx of atopy, morphology/distribution, chonic dermatitis,
3. avoid triggers, low/medium corticosteroids, antihistamines, baths in warm water + skin moisturizers

Front 1237

food allergy
1. common triggers + clinical presentation

Back 1237

1. egg, milk, peanut, soy, wheat, fish--> itching/swelling of the lips, tongue, throat, GI sypmtoms like N/V, diarrhea, rhinorrhea, wheezing, sneezing, atopic dermatitis, urticaria/angioedema, anaphylaxis

Front 1238

what can a mother do to decrease food allergies/atopic dermatitis

Back 1238

BREASTFEEDING

Front 1239

1. diagnosis of food allergy
2. treatment

Back 1239

1. skin tests, radio allergosorbant tests identify serum IgE to specific food allergens, provocative food challenge
2. avoidance, and injectable epinephrine

Front 1240

urticaria (hives)
1.pathogenesis + clinical presentation
2. chronic urticaria pathogenesisi

Back 1240

1. penicillin, NSAIDs, eggs, nuts, latex, GABHS, EBV, venoms, heat/cold, malignancy, SLE --> circumscirbed raised evanescent areas of edema that are pruritic
2. malignancy IgG anti IgE

Front 1241

what people are more likely to have latex allergy

Back 1241

healthcare workers, and patients with myelomeningocoele (due to multiple catheterizatoins)

Front 1242

drug allergy
1. what common drugs cause allergy
2. clinical presentation
3. tx

Back 1242

1. penicillin, sulfonamides, cephalosporins, aspirin/NSAIDS, narcotics
2. urticaria, angioedema, anaphylaxis
3. antihistamines, epinephrine, corticosteroids, beta agonists

Front 1243

what is angioedema

Back 1243

vascular reaction of the dermis with localized edema from dilted capillaries characterized by giant wheals

Front 1244

components of the innate immune system

Back 1244

phagocytes, NK cells, TLRs, manose binding protein, alternate complement path

Front 1245

components of the adaptive immune system

Back 1245

T cells, B cells, immunoglobulins

Front 1246

selective IgA deficiency
-most common immune deficiency
1.clinical presentation
2. dx/tx

Back 1246

1. sinusitis, pneumonia, otitis, bronchitis, chronic diarrhea/giardia infection,
SLE, juvenille arthritis, celiac disease, atopic diseases
***anaphylaxis from blood products
2. serum immunoglobulin, manage infections/complications, NO IVIG

Front 1247

CVID
1. pathogenesis + clinical presentation
2. dx/tx

Back 1247

1. MATURE Bcell/Tcell dysfunction (normal counts) --> HYPOGAMMAGLOBULINEMIA--> respiratory infections (h.flu, m.catarrhalis, s.pneumo), GI (g.lambia, c.jejuni),
rheumatoid arthitis, autoimmune thrombocyotopenia, autoimmune hemolytic aneima
**malignancy
2. dec. immunoglobulin, dec. antibody function, dec. T cell mitogen proliferation
tx = IVIG replacement, abx, diarrhea management

Front 1248

SCID
1. pathogenesis (XLR/AR) + clinical presentation
2. dx/tx

Back 1248

1. defective T cell and B cell function
X linked - deficiency gamma chain for IL-2 receptor
AR - ADA deficiency + others
---> inc. infection with oppurtunistic pathogens (c.albicans, p.carinii)
2. <1500 lymphocytes/mL, dec. # of T cells, severe hypogammaglobulinemia, depressed response to mitogens/antigens
TX = abx, blood products irradiated, IVIG replacement, P.carinii, bone marrow transplant**** (curative)

Front 1249

ataxia telangiectasia
1. pathogenesis + clinical presentation
2. dx/tx

Back 1249

1. AR Chromosome 11 mutation--> defective DNA repair, defective DNA recomb/cell cycle--> cafe aulait, vitiligo, early gray hair, CID, progressive cerebellar ataxia, oculocoutaneous telangiectasias, malignancy (lymphoma/carcinoma),
2. low IgE***, low IgA***, skin test anergy/dec. proliferation to migotgens
tx = treat infections, monitor for malignancy, avoid ionizing radiation

Front 1250

digeorge sydnrome
1. pathogenesis + clinical presentation

Back 1250

1. 22q11 deletion--> loss of 3rd/4th pharyngeal pouches--> CATCH22- cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia

Front 1251

wiskott aldrich syndrome
1. pathogenesis + clinical presentation
2. dx/tx

Back 1251

1. X linked T cell receptor signaling/cytoskeleton organization--> CID, ECZEMA, congenital thrombocytopenia (micro platelets) --> frequent encapsulated organism infections, bleeding/hemorrhage, skin infections (from eczema)
2. CBC (small platelets/thrombocytopenia), low IgM***, elevated IgA/IgE*** defective polysaccharide antigen response, anergy with normal number of T cells, NO ANTIGEN SPECIFIC CYTOTOXIC T CELLS

Front 1252

brutons agammaglobulinemia
1. pathogenesis + clinical presentation
2. dx/tx

Back 1252

1. X linked BTK tyrosine kinase deficiency --> no development of mature B cells --> encapsulated infections, chronic enteroviral infections, no tonsils
2. dx - decrease in all**** immunoglobulin types, no B cells, T cells present with preserved function, BTK mutation
tx = IVIG replacement monthly

Front 1253

chronic granulomatous disease
1. pathogenesis + clinical presentation
2. pathogens
2. dx/tx

Back 1253

1. X linked defect in NADPH oxidase --> imparied neutrophil oxidative metabolism --> impaired killing of CATALAE POS. BACTERIA AND FUNGI--> abcess formation with increased succeptability to lung/liver/bone/skin infection
2. s.aureus, psuedomaonas, salmonella, klebsiella, serratia, E.coli, c.albicans, aspergillus
3. nitroblue tetrazoleum test, flow cytometery
tx = abscess drainage, TMPSMX/itraconazole prophylaxis, IFN-y, BMT***(curative)

Front 1254

schwachman diamond syndrome
1. pathognesis + clinical presentation

Back 1254

1. AUTO REC: DEC. NEUTORPHIL CHEMOTAXIS, CYCLIC NEUTROPENIA, PANCREATIC EXOCRINE INSUFFICIENCY, RECURRENT SOFT TISSUE INFECTIONS, CHRONIC DIARRHEA, FTT

Front 1255

Complement deficiencies (usually autosomal recessive)
1. C1q/C2/C4 (early classic pathway) clinical assc + tx
2. C5/C6/C8 (late classic pathway) clinical assc + tx
3. C1 esterase inhibitor clinical assc + tx
4. dx of complement deficieny

Back 1255

1. autoimmune disease ex. SLE + treat autoimmun dz/infections
2. meningococal/gonococcal infections + treat infectiosn
3. hereditary angioedema + fibrinolysis inhibitors (danazol),
4. abnormal CH50 indicates that there is an issue SOMEWHERE in the complement path--> specific assay to identify where

Front 1256

Henoch Schonlein purpura treatment

Back 1256

pain control, hydration, steroids

Front 1257

Kawasaki disease
-MCC acquired heard disease in the US
-asians, M>F
1. what age does this occur
2. diagnositic criteria

Back 1257

1. 80% of cases younger than age
2. Fever >102 for > 5 days
4/5: non-exudative bilateral conjunctivitis with limbic sparing
oropharyngeal changes- pharyngitis, strawberry tongue, red, cracked swollen lips
cervical adenopathy
polymorphous rash
distal extremity changes - brawny edema/induration of hands/feet with erythema of the palms/soles
late - (7-10 days) peeling around nail beds of distal extremities
***exclude other causes

Front 1258

kawasaki disease
1. cardiovascular manifestiation
2. systemic manifestatiosn
-also:
arthitis, hydrops of gallbladder, anterior uveitis

Back 1258

1. coronary artery aneurysms in 20% of untreated pts, myocarditis, arrythmias, brachial artery aneurysms
2. aseptic meningitis, urethritis (sterile pyuria)

Front 1259

kawasaki disease
1. lab findings during acute/subacute/convalescent phase

Back 1259

1. acute phase 1-2 weeks- elevated ESR/CRP
subacute phase weeks-months - dec. ESR/CRP, inc. platelets**
convalescent phase weeks to years- normal lab values

Front 1260

kawasaki disease
1. treatment acute/subacute/convalescent phase
-consider steroid use in pts. refractory to IVIG
2. general JRA laboratory findings

Back 1260

acute = high dose IVIG (2g/kg), high dose aspirin
subacute = low dose aspirin
convalescent = low dose aspirin if aneurysms remain

Front 1261

juvenile rheumatoid arthritis
- > in females, males >systemic onset/late onset
1. clinical presentation pauciarticular
2. general JRA laboratory findings

Back 1261

1. < or equal to 4 joints involved usually knees and hips, may be asymmetric
Early - female predominant - chronic uveititis** (iris/ciliary body), +ANA, risk of blindness
Late - male predominant - HLA B27 positive, hips/sacroiliac joint involvment (less uveitis), risk of spondyloarthritis**
2. microcytic hypochromic aneima (AOCD), elevated ESR/CRP/platelets, RF usually negative, ANA 75% postive in early onset pauciarticular, and 50% polyarticular

Front 1262

juvenile rheumatoid arthritis
1. clinical presentation polyarticular JRA
2. general JRA laboratory findings

Back 1262

1. >4 joints, mild/absent extraarticular features, symmetric polyarthritis both small and large joints
RF(-) - >females - early/late onset
RF(+)- >females - late onseth - high risk of severe arthritis
2. microcytic hypochromic aneima (AOCD), elevated ESR/CRP/platelets, RF usually negative, ANA 75% postive in early onset pauciarticular, and 50% polyarticular

Front 1263

systemic onset JRA (Stills disease)
1. clinical presentation
2. general JRA laboratory findings
3. what fraction fully recover, what fraction go on to develop chronic destructive arthritis

Back 1263

1. multiple large/small joints inc. sternoclavicular
Male = female, HIGH SPIKING feveres, >39, transient evanescent non-pruritic salmon colored rash, HSM, lymphadenopathy, fatigue, anorexia, weight loss, FTT, serositis (pericariditis, pleuritis), myositis, tenosynovitis, meningitis, encephalopathy
2. microcytic hypochromic aneima (AOCD), elevated ESR/CRP/platelets, RF usually negative, ANA 75% postive in early onset pauciarticular, and 50% polyarticular
3. 50/50

Front 1264

treatment of juvenille rheumatoid arthritis

Back 1264

NSAIDs
glucocorticoids, MTX, sulfasalazine, hydroxychloroquine
surgery for recalcitrant joints
psychosocial support

Front 1265

juvenille rheumatoid arthritis diagnositic criteria

Back 1265

age of onset <16
arthritis >1 joint- swelling or effusion or limited motion, tenderness, increased warmth,
>6 weeks duration
exclusion of other causes

Front 1266

SLE
females >males 8:1
1. when is peak age of onset
2. pathogenseis

Back 1266

1. adolecence
2. UNKNOWN, drug reactions, sun exposure, infections, hormonal changes--> widespread inflmmation of connective tissues and immune mediated vascultiis

Front 1267

SLE
1. clinical presentation

Back 1267

1. fever, weight loss, malaise
malar rash, photo sensitivity, alopecia, raynauds
arthrialgias WITHOUT joint deformity/erosions
pericarditis, libman sacks endocarditis, congenital heart block*
pleuritis, interstitial fibrosis, pulmonary hemorrhage
glomerulonephritisi, nephrotic syndrome, hypretension
leukocytopneia, AOCD, thrombocytopenia, coombs positive hemolytic anemia

Front 1268

SLE
1. lab findings
2. treatment
3. tx for patient with thrombosis/anti-phospholipid antiboides
4. cause of mortality

Back 1268

1. ANA+, RF+, anti -dsDNA (specific), anti-Sm (specific), anti-phospholipid (inc. risk for thrombosis), dec. C3/C4 in active disease
2. NSAIDS for inflammation, glucocorticoids (main tx), cyclophosphamide (nephritis), MTX, azathioprine, cyclosporine
3. heparin/warfarin
4. infection, renal failure, nephritis, CNS complications

Front 1269

SLE mneumonic pg 475

Back 1269

...

Front 1270

dermatomyositis
ages 5-14 (avg. age 6), F>M
1. clinical presentation
2. dx
3. tx

Back 1270

1. progressive muscle weakness + constitutional, periorbital violaceous heliotrope rash (sun exposed), gottrons papules, proximal muscle weakness (gowers), neck flexor weakness, calcinosis (in muscle,"", nail telangiectasias,
2. clinical pres + EMG abnormal, muscle biopsy abnormal, inc. CK/AST/ALT/LDH/aldolase
3. corticosteroids, MTX, cyclophosphamide, cyclosporine, vitamin D/calcium supplementation

Front 1271

dermatomyositis complications

Back 1271

aspiration pneumonia (dec. gag reflex), intestinal perforation (GI vascultis), osteopenia(secondary to intestinal perforation)

Front 1272

rheumatic fever
- children 5-15 year of age
1. pathogenesis
2. clinical dx/presentation

Back 1272

1. autoimmune complication of GABHS URI.pharyngitis (NOT IMPETIGO)
2. Jones criteria, 2 major or 1 major + 2 minor
Major: migratory polyarthritis,
carditis(transmural, left sided endocarditis, tachycardia)
syndhams chorea (basal ganglia autoimmune
erythema marginatum (non-pruritic pink/red macules that spread with centrpitally), subcutaneous nodules
Minor: fever, arthralgia, leukocytosis, CRP/ESR, prolonged PR on ECG

Front 1273

1. rheumatic fever lab findings
2. treatment
- eventually cardiac damage/valvular insufficiency may require valve replacement

Back 1273

1. ESR/CRP elevated, leukocytoisis, ASO titers/anti-DNase,anti-hyaluronidase elevated,
echocardiography shows decreased ventricular function,
2. benzathine penicillin, or oral penicillin
NSAIDS (after DX)
corticosteroids for severe cardiac involvement
diuretics,digoxin, salt restriction for CHF
haloperidol for syndenhams chorea

Front 1274

what must a patient who has rhuematic fever prophylax against

Back 1274

antimicrobial prophylaxis especially before minor dental procedures

Front 1275

lyme disease
1. pathogenesis
2. clinical presentation early/early disseminated/late

Back 1275

1. New england US borellia burgdorferi tick bite via ixodes tick
2. early = erythema chronicum migrans (target like annular rash), constitutional symptoms (headache, myaligias, fatigue, arthralgias,lymphadenopathy)
early disseminated = multiple secondary erythema migrans, constitutional symptoms, FACIAL NERVE PALSY (UNI/BILATERAL), encephalitis, heart block/carditis (arrythmias), aseptic meningitis
Late: arthritis

Front 1276

lyme disease
1. diagnosis
2. treatmnet

Back 1276

1. clinical pres + ELISA (sensitive), then western blot (sens + specific),
2. early doxycycline or amoxicillin
ceftriaxone or penicillin for carditis/meningitis

Front 1277

reactive reiters disease
1. clinical presentation

Back 1277

1. chlamydia trachomatis--> arthritis, urethritis, conjunctivitis (cant see cant pee cant climb a tree - TF)

Front 1278

psoriatic arthritis

Back 1278

small/large joint arthritis associated with psoriasis (caly skin plaques, nail pitting, onychoysisi)

Front 1279

ankylosing spondylitis + clinical association

Back 1279

HLAB27 male predomiant axial skeleton and lower extremity joints + enthesis
~ associated with pauciarticular JRA in MALES*****

Front 1280

arthritits of inflammatory bowel disease

Back 1280

UC/crohns HLAB27 positive, have axial skeleton arthritis indistinguishable from ankylosing spondylitis

Front 1281

takayasu arteritis

Back 1281

large vessel vascultis with aneurysmal dilation of the aorta, carotid, and subclavian artiers~~ seen in asian adolescents/young adults

Front 1282

polyarteritis nodosa

Back 1282

small and medium size vessel aneurysms, associate with hepatitis B

Front 1283

wegeners granulomatosis

Back 1283

necrotizing granulomas of the upper/lower respiratory tract and kidneys---- sinusitis, hemoptysis, glomerulonephritis

Front 1284

sjogrens syndrome

Back 1284

sicca sydnrome (dry eyes and mouth), high titers of autoantibodies, connective tissue disease

Front 1285

scleroderma
1. pathogenesis + classic clinical presentation

Back 1285

1. fibrosis and dysfunctions of multiple organ systems - skin, heart, kidneys, lungs, GI

Thickening of the skin with loss of dermal ridges

Front 1286

crest syndrome
1

Back 1286

calcinosis, raynauds, esophageal, sclerosis of the skin, telangiectasias

less systemic involvement compared to scleroderma

Front 1287

what causes brachial plexus injury during birth

Back 1287

excess traction on neonates head/neck/arm which stretches the brachial plexus

Front 1288

erbs palsy

Back 1288

C5 C6 nerve root injury --> flaccid arm/asymmetric moro reflex
*arm internally rotated, elbow extended, forearm pronated, wrist/fingers in flexion

Front 1289

klumpkes palsy

Back 1289

C7 C8 - claw hand from unopposed finger flexion, decreased ability to extend the elbow
+/- horners syndrome*******

Front 1290

nursemaids elbow
+ tx

Back 1290

subluxation of radial head from upward force on the arm (radial head is slender shape in toddlers)
ex. pulling a toddler upward by the hand to make them stand
*toddler presents holding the elbow flexed
tx = flexing the elbow and supinating the hand

Front 1291

anterior shoulder dislocation

Back 1291

ext. rotation, abduction and extension of the shoulder~~~ occurs in gymnastics/wrestling
recurrance is 90%

Front 1292

congenital torticollis
1. pathogenesis + clinical pres
2. tx

Back 1292

1. uterine constraint or birth trauma --> head tilts toward affected side, asymmetry of the head/ears if intreated
**may feel soft tissue mass in SCM = bleeding into muscle
2.stretching exercises, helmet therapy (misshapen head), radiograph to find any cervical spine abnormalities

Front 1293

causes of acquired torticollis + first diagnostic test

Back 1293

upper respiratory infections, cervical lymph adenitis, peritonsillar/retropharyngeal abscess, cervical diskitis, osteomyelitis, neoplasms, strabismus, refractive errors, CERVICAL VERTEBRAE FRACTURE****

first test = xray of the neck

Front 1294

atlantoaxial instability
1. pathogenesis
2. dx

Back 1294

1. down syndrome**, keletal dysplasia, klippel feil syndrome --> unstable 1st/2nd cervical vertebrae joint --> spinal cord injury --> paralysis
2. x ray, C1/C2 cervical spine fusion

Front 1295

klippel feil syndrome

Back 1295

developmental failure of vertebral segmentation--> fusion of vertebrae--> congenital torticollis, GU anomalies, CHD, sprengels deformity

Front 1296

sprengels deformitiy

Back 1296

seen in klippel feil syndrome
scapula is rotated laterally leading to shoulder asymmetry and dec. shoulder motion

Front 1297

scoliosis
1. pathogenesis + clinical pres
2. dx
3. what degree of scoliosis recquires bracing or surgery
- major complications are respiratory or cardiovascular compromise

Back 1297

1. idiopathic or multiple causes --> lateral curvature of the spine
2. Adam's forward bending test and measurement of Cobb angle
Tx = bracing prevents progression,
surgery prevents progression, or treats sever curves
3. 20-40 deg = brace
>40 deg or proggression of 5 deg in 4-6 months

Front 1298

kyphosis - two types

Back 1298

1. anterior posterior curvature of the thoracic spine two types = flexible (self correctable) and scheurermanns kyphosis (non-flexible)

Front 1299

back strain

Back 1299

MCC back pain, muscular soreness from overuse or bad body mechanics

Front 1300

spondylolysis + clinical presentation

Back 1300

stress fracture of pars interarticularis from repetitive hyper-extension of the spine most commonly in L5 ~~ pain with hyperextension

Front 1301

spondylolithesis

Back 1301

L5-S1 vertebral body slips anteriorly and can impinge on nerve roots, "PALPABLE STEP OFF" in the lumbosacral area

Front 1302

treatment for spondylolysis and spondylolithesis

Back 1302

bracing and surgery if bracing does not improve the condition

Front 1303

1. diskitis + clinical presentation
2. dx/tx

Back 1303

1. URI/minor trauma --> infection of the intervertebral disk (s. aureus) --> tenderness above the affected disk, and refusal to flex the spine/ambulate
2. elevated ESR, MRI/bone scan, bed rest, anti-staphylococcal

Front 1304

1 developmental dysplasia of the hip - pathogenesis + clinical pres
girls 6x> boys
2. dx
3. tx
- complicatoins = avascular necrosis of femoral head, limb length discrepancy

Back 1304

1. abnormally flat acetabulum --> easy disolcation
2. postive barlow ("clunk"), positive ortolani, abnormal galeazzi sign (one knee higher than the other), asymmetric abduction of the hips
3. pavlik harness - hold head of femur against acetabulum, surgery (if pavlik fails)

Front 1305

septic arthritis
1. pathogenesis/clinical presentation
2. which joint most commonly effected in younger and older children

Back 1305

1. s.aureus/s.pyogenes, n.gonorrhoeae hematogenous spread, contiguous spread, direct inoculoation--> erythema, swelling, asymmetry of soft tissue, pain with movment, hip held in flexion/abduction/external rotation to eleviate the pain
2. hip in young, knee in older

Front 1306

septic arthritis
1. dx/tx
-complications = avascular necrosis/cartilage damage

Back 1306

1. elevated WBC/ESR/CRP, postive blood culture(50%), SYNOVIAL FUID SHOWS WBC >50K-100K, radiograph shows widened joint space
Tx = EMERGENCY surgical decompression/drainage, by joint aspiration, empiric IV abx

Front 1307

transient synovitis
1. pathogenesis + clinical presentation
2. dx/tx

Back 1307

1. MCC painful limp in toddlers- post infectous resposne of hip joint --> low grade fever, limp, mild irritability- similar limb positioning as septic arthritis,
2. WBC/ESR may be normal, aspirate/culture hip effusion
tx = NSAID, bed rest, obs

Front 1308

legg calve perthes
age 4-9, boys >girls
1. pathogeness + clinical presentation
2. dx
3. tx

Back 1308

1. idiopathic avascular necorsis of the femoral head, decreased internal rotation/abduction of the hip (ELICITS PAIN) + PAIN IN THE ANTERIOR THIGH
2. radiographs show increased density of the femoral head, or "crescent sign" subchondral fracture
3. physical therpay, restrict exercise, surgery (if 50% necrosis), self resolves in children <9 years of age (>9 --> osteoarthritis)

Front 1309

slipped capital femoral epiphysis
1. pathogenesis/clinical pres
2. dx/tx

Back 1309

obese* adolescent adult boy/hypothyroidism* --> slipping of femoral head off femoral neck--> painful limp, decreased internal rotation/abduction of the hip-->avascular necrosis of femoral head/chondryolysis/limb length discrepancy/osteoarthritis
2. radiograph shows klein line (line drawn from lateral surface of femoral neck doesnt cross epiphysis)
Tx = pinning the epiphysis to prevent slippage

Front 1310

osteomyelitis
1. pathogenesis/clinical pres
2. dx/tx

Back 1310

1. s. aureus/s.pyogenes/salmonella (sickle cell)/ pseudomonas (nail in foot thorugh shoe), hematogenous (MCC)--> fever, bone pain, erythema, swelling, induration
2. elevated WBC/CRP/ESR, MRI or bone scan, NOT RADIOGRAPH(will be normal early in infection)
tx = 6wks abx, monitor ESR (dec. = good response), surgery for abscess if fever/swelling persist

Front 1311

osteomyelitis complications

Back 1311

1. spread to distant joint or pneumonia
chronic osteomyelitis due to a *sequestrum (necrotic focus) or *involucrum (new bone/fibrosis around infected bone)

Front 1312

metatarsus adductus (in toeing)
1. pathogenesis + clinical presentation
2. treatment

Back 1312

1. intrauterine constraint (ie. first child primigravid uterus) --> medial curvature of the midfoot with passive dorsiflexion and flexibility preserved
2. passive motion/stretching (dont over correct), stiff foot that cannot be straightened must be cased for 3-6 weeks

Front 1313

talipes equinovarus (clubfoot)
1. pathogenesis + clinical presentation
2. treatment

Back 1313

1. DDH/myelomeningocele/myotonic dystrophy/skeletal dysplasia/genetics, abnormal positioning... multifactorial --> FIXED foot inversion/plantarflexion with no flexibility (compare to metatarsus adductus), 50% bilateral
2. casting in the first week of life, surgical if this doesnt work (MUST BE DONE EARLY****)

Front 1314

internal tibial torsion
1. pathogenesis + clinical presentation
2. tx

Back 1314

1. in utero positioning --> medial rotation of the tibia (MCC in-toe-ing <2 years of age), patella faces forwards, usually bilateral
2. observation

Front 1315

femoral anteversion
1. pathogenesis + clinical presentation
2. treatment

Back 1315

1. inward anteversion with both feet and patella being inward facing, increased internal rotation of the hips, child prefers sitting in W POSITION***
2. observation, res. by 8 years of age

Front 1316

out toe-ing
1. pathogenesis + clinical presentation
2. treatment

Back 1316

1. intrauterine constraint(first child) --> calcaneovalgus foot = flexible foot held in a lateral position
2. stretching and casting

Front 1317

bowed legs (genu varum)
1. pathogenesis + clinical presentation
2. treatment

Back 1317

1. asymmetric bowing <2 years of age --> cowboy stance (lateral bowing), normal gait
2. observation, if does not resolve by 2 years of age consider rickets, growth plate injury, blounts disease, skeletal dysplasias, measure intercondylar distance

Front 1318

blounts disease (tibia vara)
1. pathogenesis + clinical presentation
2. dx/tx

Back 1318

1. obese African American boys who are early walkers, overload--> medial tibia growth plate injury --> inhibited growth only on the meidal side--> PROGRESSIVE UNILATERAL BOWING, >2 years of age --> recurrence of angulation/osteoarthritis if not corrected
2. dx = metaphyseal diaphyseal angle >11 degrees
tx = bracing, surgery if there is no improvemnt, or if the patient is >4 years of age

Front 1319

knock knees (genu valgum)
1. pathogenesis + clinical presentation
2. tx

Back 1319

1. over correction of genu varum--> seperation of ankles with knees together, swinging legs laterally with walking/running --> osteoarthritis if not treated
2. surgery if >10 years old

Front 1320

osgood schlatter disease
1. pathogenesis + clinical presentation
2. dx/tx

Back 1320

1. children who participate in sports involving jumping --> apophysitis (inflamm of a tuberosity) +
2. tenderness with extension of the knee against resistance
tx = rest/stretching of the quadriceps/hamstrings

Front 1321

patellofemoral syndrome
1. pathogenesis + clinical presentation
2. dx/tx

Back 1321

1. malalignment of the patella --> knee pain worst with activity or running
2. patella in the lateral position on "sunrise" radiograph,
Tx = rest, stretching, strengthening the medial quadriceps

Front 1322

growing pains
1. pathogenesis + clinical presentation
2. dx/tx

Back 1322

1. idiopathic bilateral leg pain that occurs afternoon/evening but does not interfere with play, children wake up crying in pain ages 4-12
2. analgesics/reassurance

Front 1323

open vs closed fracture

Back 1323

open = break in the skin
closed = skin intact

Front 1324

fracture
1. nondisplaced(non angulated) fracture
2. displaced
3. angulated
4. overriding

Back 1324

1. fracture ends that are near each other
2. fractured ends have shifted
3. fractured ends for m an angle
4. fractured ends override without contact

Front 1325

compression fracture (aka torus aka buckle) + treatment

Back 1325

bony cortex buckles under compressive force usually at the metaphysis
tx. with splinting

Front 1326

incomplete fracture (aka greenstick) + treatement

Back 1326

one side of the cortex fractured with the other side intact
tx = fracture other side of cortex and reduce

Front 1327

1.transverse fracture
2. oblique fracture
3. spiral fracture
4. comminuted fracture

Back 1327

1. horizontal across the bone
2. diagonal fracture across bone
3. oblique fracture that encircles the bone associated with twisting injury/child abuse***
4. multiple fracture fragments

Front 1328

salter harris fracture classification
I
II
III
IV
V

Back 1328

I= fracture in the physis
II = fracture in the physis extends to metaphysis
III= fracture in the physis extends to the epiphysiss
IV = fracture in the physisi through both metaphysis/epiphysis
V = crush of the physis

Front 1329

diaphyseal fracture

Back 1329

central shaft fracture

Front 1330

what is the major cause of clavicular fractures in childhood and neonates

Back 1330

falling ont the shoulder, and birth injury in neonates

Front 1331

clinical presentation of a neonatal clavicular fracture
+ tx

Back 1331

asymmetric mororeflex, or pseudoparalysis, palpable crepitus over the fracture
tx = sling 4-6 weeks for children, infants no treatment

Front 1332

supracondylar fracture
1. pathogenesis + clinical presentation
2. dx/tx

Back 1332

1. child falls onto outstretched arm/elbow--> point tenderness/swelling, --> possible neurovascular injury/compartments syndrome
2. POSTERIOR FAT PAD SIGN on xray
TX: DONT PASSIVLY MOVE THE ARM, casting for nondisplaced/nonangulated, surgery for displaced/angulated

Front 1333

compartment syndrome
1. pathogenesis + clinical presentation
2. dx/tx

Back 1333

1. FOOSH injury (supracondylar fracture)--> pressure in the anterior fascial compartment >30-45 mmHg--> ischemia/volkmanns contracture pain with passive extension of the fingers + "the 5Ps" (pain, pallor, poikilothermia, paresthesias, pulselessness, paralysis)

Front 1334

compartment syndrome pneumonic

Back 1334

5Ps
pallor, pulselessness, paraysis, pain, paresthesisas

Front 1335

cubitus varus

Back 1335

movent of limb to midline after healing of a supracondylar fracture due to poor positioning of the distal fragment

Front 1336

colles fracture

Back 1336

distal radius fracture

Front 1337

monteggia fracture

Back 1337

proximal ulna with dislocation of the radial head

Front 1338

galeazzi fracture

Back 1338

fracture of the radius with distal radioulnar joint dislocation

Front 1339

treatment of forearm fractures

Back 1339

splint for 4-7 days until swelling resolves, then cast for 6-8 weeks

Front 1340

toddlers fracture
1. pathogenesis + clinical presentation
2. dx/tx

Back 1340

1. 9 months - 3 years child trips and falls while running--> spiral fracture of the tibia
2. radiographs, put in a cast for 3-4 weeks

Front 1341

what fractures are suspicious for child abuse

Back 1341

FOUND WITH BONE SURVEY
metaphyseal fractures
posterior or first rib fractures
multiple fractures at varied ages of healing
complex skull fractures
scapular, sternal, and spinous process fractures
*** fracture that dont fit childs DEVELOPMENTAL ABILITIES ---- ex. spiral fracture of the lower extremities in a child who is not ambulatory

Front 1342

what is the treatment for scoliosis before and after puberty growth spurt

Back 1342

before puberty growht spurt can treat with bracing or surgery
after puberty growth spurt, bracing is not an option, however the scoliosis should not progress at this point

Front 1343

how to evaluate the red reflex in an infant

Back 1343

bruckner test - direct ophthalmoscope from 2 feet away into the patients eyes

Front 1344

hirschberg test

Back 1344

evaluate symmetry of light off the patients corneas

Front 1345

what is visual acuity at birth

Back 1345

20/200, and improves during the first 3-4 months of life

Front 1346

1. when is visual development most important
2. what can cause abnormal visual development

Back 1346

1. first 3-4 months of life
2. improper eye alignment (strabismus)
any condition that blocks retinal stimulation

Front 1347

amblyopia
1. pathogenesis + clinical presentation
2. dx/tx
*earlier onset = worse vision loss

Back 1347

1. strabismus, opacification of the lens, severe refractive error, anisometropia, viterious hemorrhage --> abnormal visual development
2. dx = abnormal bruckner and hirshberg test
tx = correct refractive errors, patch the normal eye ASAP

Front 1348

neonatal conjunctivitis
1. pathogenesis + clinical presentation
2. dx/tx

Back 1348

1. vagianl canal transmits neisseria gonorrhoeae, chlamydia trachomatis and HSV, 1% silver nitrate (for gonorrhoea)--> red watery eyes

Front 1349

neisserira gonorrhoeae conjunctivitis (neonatal)
1. pathogenesis + clinical presentation
2. dx/tx

Back 1349

1. vaginal canal --> 2-4 days purulent dishcharge/eyelid welling/corneal ulcer
2. dx: gram negative diplococci
tx: IV cefotaxime, topical erythromycin

Front 1350

chlamydia trachomatous conjunctivitis (neonatal)
1. pathogenesis + clinical presentation
2. dx/tx

Back 1350

1. vaginal canal -->4-10days of life -->serous or purulent discharge +/- lid swelling
2. cytoplasmic influsion bodies, positive culture
oral erythromycin

Front 1351

HSV conjunctivitis (neonatal)
1. pathogenesis + clinical presentation
2. dx/tx

Back 1351

1.vaginal canal --> 6 days - 2 weeks of life- usually unilateral with serous discharge,
2, dx = multinucleated giant cells on gram stave, positive culture
tx = acyclovir, topical triflurothymidine

Front 1352

causes of red teary eyes in a newborn

Back 1352

conjunctivitis, glaucoma, dacryocystitis, enophthalmitis

Front 1353

causes of red eye in older infants/children

Back 1353

infectious, allergic, contact lens, corneal abrasion, bacterial corneal ulcer, HSV keratitis,

Front 1354

what does positive fluorescein staining indicate in the eye

Back 1354

trauma, bacterial corneal ulcer, HSV keratitis

Front 1355

bacterial conjunctivitis
1. pathogenesis + clinical presentation
2. dx/tx

Back 1355

1. NTHI, s.pneumo, m. catarrhalis, s.aureus--> PURULENT discharge, +/- otitis media
2. dx = conjunctival cultures
tx =topical antibiotics- sulfacetamide, polymixin B, TMP, gentamycin, tobramycin, erythromycin

Front 1356

viral conjunctivitis
1. pathogenesis + clinical presentation
2. tx

Back 1356

1. adenovirus 3 and 7 --> bilateral conjunctivitis, pharyngitis, fever--> watery conjunctival discharge, hyperemic conjunctiva, preauricular adenopathy
2. supportive treatment + NSAIDs,

Front 1357

epidemic keratoconjunctivitis
1. pathogenesis + clinical presentation
2. tx

Back 1357

1. adeovirus 8,19,37 --> petechial conjunctival hemorrhage, pseudomembrane on the conjuntiva, keratits/photophobia (hypersensitivity reaction) pre-auricular lymphadenopathy, NO FEVER OR PHARYNGITIS
2. supportive treatment

Front 1358

primary ocular herpes
1. pathogenesis + clinical presentation
2. dx/tx

Back 1358

1. HSV1 exposure--> skin eruption + corneal ulcer
2. positive viral culture + systemic or topical acyclovir

Front 1359

allergic conjunctivitis
1. pathogenesis + clinical presentation
2. tx

Back 1359

1. type 1 hypersensitivity reaction accompanies seasonal allergic rhinitis--> itching + watery discharge
2. cromolyn eye drops,topical antihistamines

Front 1360

hemorrhagic conjunctivitis
1. pathogenesis + clinical presentation

Back 1360

1. subconjunctival hemorrhage cuased by h.flu, adeno, picronaviruses

Front 1361

blepharitis
1. pathogenesis + clinical presentation
2. tx

Back 1361

1. s. aureus --> eyelid inflammation and red eye, crusting, scaling of the eyelash base, eyelids stick together in the monring
2. eyelid hygene with baby shampoo, topical erythromycin

Front 1362

nasolacrimal duct obstructino
1. pathogenesis + clinical presentation
2. tx

Back 1362

1. failure of canalization of the lacrimal system and obstruction at Hasners valve --> watery eyes, matted eyelashes, mucus
2. spontaneously resolves, nasolacrimal massage, toical abx, NLD probing

Front 1363

amniotocele (dacrocele)
1. pathogenesis + clinical presentation
2. tx

Back 1363

1. swelling of nasolacrimal sac --> bluish swelling in medial canthal area/warmth/erythema/tenderness
2. local massage, IV abx, NLD probing

Front 1364

retinal hemorrhages
1. pathogenesis + clinical presentation

Back 1364

1. child abuse ***, birth trauma, inc. ICP, malignant hypertension, ITP, cardiopulmary resusitation

Front 1365

corneal abrasion
1. pathogenesis + clinical presentation
2. dx/tx

Back 1365

1. damage/loss of the corneal epithelium--> severe pain/foreign body sensation
2.dx = flurescein staining of the cornea
tx = protective shield and antibiotics

Front 1366

hyphema
1. pathogenesis + clinical presentation
2. tx

Back 1366

1. blunt trauma, diabetic neovascularization, retinal vascular diseases, juvenille xanthogranuloma--> tearing of iris vasculature--> blood in the anterior chamber--> glaucoma, staining of the cornea, optic nerve damage, rebleeding
2. bed rest 5 days

Front 1367

orbital floor fracture (blow out fracture)
1. pathogenesis + clinical presentation
2. tx

Back 1367

1. blunt trauma --> fractures in the orbital floor--> entrapment of the orbital fat apad and inferior rectus muscle --> strabismus and enophthalmos + numbness of cheek/upper teeth (infraorbital nerve damage)
2. oral antibiotics, surgical repair if diplopia >2-4 weeks

Front 1368

congenital glaucoma
1. pathogenesis + clinical presentation
2. tx

Back 1368

1. AD maldevelopment of the trabecular meshwork, congenital rubella, aniridia --> presssure >15mmHg--> damage to the optic nerve and increase in the size of eye (b/c eye is elastic in children), --> tearing, enlarged cornea/corneal clouding, dull red reflex
2. surgery, beta agonists, carbonic anhydrase inhibitors

Front 1369

which synromes have congenital glaucoma

Back 1369

sturge weber, neurofibromatosis, marfans

Front 1370

retinopahty of prematurity
1. pathogenesis + clinical presentation
2. tx

Back 1370

1. preterm, hyaline membrane disease, intracranial hemmorrhage--> proliferation of retinal blood vessels exposed to oxygen --> myopia, astigmatism ,amblyopia, strabismus
2. minimize O2 delivery, treat hyaline membrane disease

Front 1371

congenital cataract
1. pathogenesis + clinical presentation
2. tx

Back 1371

1. idiopathic, downs syndrome, marfan, alport, smith-lemli-opitz, hypoglycemia, galactosemia, CMV/rubella, truama--> amblyopia
2. early surgery

Front 1372

retinoblastoma
1. pathogenesis + clinical presentation
2. tx
3. most common late complication

Back 1372

1. chromosome 13 mutation--> leukocoria/strabismus, retinal detachment, hyphema
2. dx = calcification within tumor seen on CT
tx = enucleation, or radiation/laser/cryo
3. cancer later in life especially OSTEOGENIC SARCOMA

Front 1373

strabismus
1. pathogenesis + clinical presentation
2. tx

Back 1373

1. misalignment of the eyes, esotropia, exotropia, vertical strabismus --> suppression of the suppression of the deveiated eye -->amblyopia (<5), diplopia (>7)
2. patching, corrective lenses, surgery

Front 1374

pseudostrabismus

Back 1374

apparant strabismus caused by prominent epicanthal folds

Front 1375

1. macule
2. patch
3. papule
4. plaque

Back 1375

1. flat non-palpable, cutaneous color change
2. large macule
3. elevated superficial dermal lesion
4. large/coalesced papules

Front 1376

1. vesicle
2. bulla
3. pustule
4. cyst
5. wheals

Back 1376

1. fluid filled papule
2. large vesicle
3. purulent filled papules
4. nodules with expressable material
5. cutaneous elevation with dermal edema

Front 1377

1. scaling
2. crusting
3. excoriations

Back 1377

1. desquamation of stratum corneum
2. dried exudate/debris
3. linear erosions of the epidermis caused by fingernail scratches

Front 1378

1. scars
2. ulcer
3. atrophy
4. fissure

Back 1378

1. thickened fibrotic dermis
2. absense of epidermis + some of the dermis
3. thinning of epidermis/dermis
4. linear cracks in the dermis

Front 1379

woods light

Back 1379

used to identify pigmentary changes + some dermatophytes

Front 1380

KOH

Back 1380

used to identify fungal hyphae

Front 1381

how does cutaneous absorbtion of substances differ in a preterm infant

Back 1381

increased absorbtion through the skin

Front 1382

1. ointment
2. cream
3. lotion

Back 1382

1. little or no water, max water retaining properties
2. 20-50% water, average dryness
3. > water than cream, used for minimally dry skin or large surfaces

Front 1383

keratolytics -

Back 1383

salicylates, urea, alpha hydroxy acids, retinoic acid~~ hyperkeratosis

Front 1384

destructive therapies

Back 1384

high dose salicylic acid, podophyllin, 5-FU, cryotherapy, electrotherapy, laser therapy~~ for warts of molluscum contagiosum

Front 1385

what are the side effects of systemic steroids

Back 1385

adrenal suppression, depressed growth, cataracts, glaucoma, cushings syndrome

Front 1386

local side effects of steroids

Back 1386

acne, hirsutism, folliculitis, striae, hyper/hypopigmentation, atrophy, eccymosis/telangiectasias, tachyphylaxis

Front 1387

tacrolimus ointment used to treat

Back 1387

atopic dermatits

Front 1388

1-5% sulfur used to treat

Back 1388

acne

Front 1389

tar used to treat

Back 1389

eczema, psoriasis

Front 1390

contact dermatitis
1. pathogenesis + clinical presentation
2. tx

Back 1390

1. poison ivy/nickel jewlery, lotions/cream, perfumes --> T cell mediated -->erythematous papules/vesicles @ area of contact
2. topical corticosterods/avoidance of allergen

Front 1391

primary irritant contact dermatitis
1. pathogenesis + clinical presentation
2. tx

Back 1391

1. substances/feces/urine/diaper --> inflammatory dermatitis-->erythema, papules w/o inguinal crease involvement--> secondary infection with candida ~~ classic diaper dermatitis
2. barrier creams (ZnO), keep skin clean, skin moisturzers, nystatin/clotrimazole

Front 1392

seborrheic dermatitis
1. pathogenesis + clinical presentation
2. dx/tx

Back 1392

1. pityosporum ovale/unknown --> eruption of greasy red scales/crusts in areas with high sebaceous glands (scalp/face/brows/nose), cradle cap on infants, nasolabial folds, pinna, scalp, diaper areas
2. low dose topical steroids, sulfur, zinc or salicylic acid shampoos, topical antifungals

Front 1393

pityriasis rosea
1. pathogenesis + clinical presentation
2. tx

Back 1393

1. hypersensitivity to a virus --> herald patch --> 1-2 weeks --> oval erythematous macules and papules of the trunk/legs follows skin lines (CHRISTMAS TREE), pruritic
2. antihistamines, UV light

Front 1394

psoriasis
1. pathogenesis + clinical presentation
2. nail findings
3. treatment

Back 1394

1. AUTO DOM immune dysregulation --> epidermal proliferation --> SILVER SCALING papules/plaques on scalp, ears, elbows, knees that KOEBERNIZE, ARTHRITIS
2. pitting, distal thickening, lifting of nail bed
3. topical corticosteroids, UV light, vitamin D analogs, 3% salicylic acid, anthralin (downregulates EGF)

Front 1395

miliaria rubra
1. pathogenesis + clinical presentation
2. tx

Back 1395

1 disrupted sweat ducts --> sweating/occlusion--> small erythematous pruritic papules or vescles on intertriginous areas
2. avoid occlusive clotion

Front 1396

serum sickness

Back 1396

urticara + systemic signs (fever/adenopathy) after medications

Front 1397

erythema multiforme
1. pathogenesis + clinical presentation

Back 1397

1. hypresensitivity reaction to various stimuli (infectious/non-infectious)--> targetoid lesions
*three types minor/major/stevens johnson syndrome

Front 1398

erythema multiforme minor
1. pathogenesis + clinical presentation
2. tx

Back 1398

1. HSV --> low grade fever, arthralgials/myalgias --> symmetric target lesions in an acral distribution + ONE mucous membrane finding (usually mouth)
2. acyclovir

Front 1399

erythema multiforme major
1. pathogenesis + clinical presentation
2. tx

Back 1399

1. mycoplasma pneumoniae/drugs --> low grade fever/arthralgias/myalgias--> symmetric targetoid lesions of the trunk AND extremeities + TWO mucus membrane surfaces
2. erythromycin for m.pneumoniae, stop drug

Front 1400

Stephen johnsons syndrome
1. pathogenesis + clinical presentation
2. tx

Back 1400

1 drugs (phenytoin, barbiturates, sulfonamides, penicillin), m.pneumoniae, HSV1 --> high fever/cough/malaise--> erythema multiforme (targetoid lesions), lesions/blisters/necrosis + two mucosal surfaces involved
2. steroids, IVIG, burn care, stop drug

Front 1401

Toxic epidermal necrolysis

Back 1401

1. drug reaction--> widespread epidermal necrosis--> sloughing of the skin, NIKOLSKY SIGN~ HIGH DEATH due to sepsis, dehydration, electrolyte

Front 1402

tinea capitis
1. pathogenesis + clinical presentation
2. dx/tx

Back 1402

1. trichophyton tonsurans(from humans)/microsporum canis (from dogs) --> patchy hair loss with hairs that break + scales/pustules + kerion (large red boggy nodule hypersendivity reaction) + occipital/pos. cervical lymphoadenopathy
2. dx = KOH identifies fungae, woods light for m. canis
tx = systemic griseofulvin, topical antifungals NOT EFFECTIVE

Front 1403

tinea corporis,/pedis/cruris
1. pathogenesis + clinical pres
2. dx/tx

Back 1403

m.canis, t.tonsurans, other trichophyton
corporis = oval/circular scaly erythematous patches with partial clearing
pedis = scaling/erythema of the toes/plantar foot
cruris = scales/erythema in the groin/inguin
2. KOH skin scrapings, topical antifungals (clotrimazole, terbinafine, ketoconazole

Front 1404

tinea unguium
1. pathogenesis + clinical presentation
2. tx

Back 1404

1. fungal infection of the nails
2. topical antifungals dont work very well, use systemic griseofulvin, terbinafine, ketoconazole

Front 1405

tinea versicolor
1. pathogenesis + clinical presentation
2. tx

Back 1405

1. pityosporum orbiculare yeast --> superficial fungal infection--> scaly oval macules, which may be hypo/hyperpigmented, and become more prominent with sun exposure
2. 2.5% selenium sulfide, ketoconazole shampoo, systemic antifungals

Front 1406

morbilliform rash vs scarlatiniform rash

Back 1406

morbilliform = measles like
scarlatiniform = papular, vesicular, petechial

Front 1407

erythema infectiosum (fifth disease)
1. pathogenesis + clinical presentation
2. complications
3. tx

Back 1407

1. parvo B19 by respiratory secretions--> upper URI --> slapped cheek rash (bright red macular rash)-> lacy reticular rash on the trunk +/- arthralgias
2. aplastic crisis, prolonged anemia, fetal hydrops in pregnant women
3. none- supportive, IVIG for chronic anemia in immunosuppressed

Front 1408

roseola infantum (exanthem subitum)
1. pathogenesis + clinical presentation
2. tx

Back 1408

1. HHV6/7, adeno, parvo, echo --> 3-5 days high fever--> pink papular eruption on the trunk that spreads to the extremities (fever is gone when rash comes)
2. supportive

Front 1409

gianotti-crosti syndrome (papular acrodermatitis
1. pathogenesis + clinical presentation
2. tx

Back 1409

1. HBV, EBV, CMV, coxsackie,--> red flat topped papules in acral areas

Front 1410

varicella
1. pathogenesis + clinical presentation
2. tx

Back 1410

1. respiratory transmission --> 3 week incubation--> macules w/ central vesicles within 1-2 days (dew drops on a rose petal) CROPS of different age lesiosn + fever
2. antipyretics with antibacterial soaps, antihistamines, acyclovir for varicella pneumonia/encphalitis

Front 1411

HSV1 /HSV2
1. pathogenesis + clinical presentation
2. dx/tx

Back 1411

1. passage through birth canal--> gingivostomatitis + grouped lesions on an erythematous base, meningitis, meningoencephalitis, herpetic whitlow--> dormant in the DRG -->recurrent infections
2. dx =- tzank preparation, direct fluorescent antibody testing, PCR for CSF
tx = acyclovir for cutaneous HSV,

Front 1412

hand foot mouth disease
1. pathogenesis + clinical presentation
2. tx

Back 1412

1. coxsackievirus A16--> vesicles, papules, pustules on the palms/soles/fingertips + mouth (herpangina) + non specific fever, headache, backache and vomiting
2. supportive

Front 1413

warts
1. pathogenesis + clinical presentation
2. tx

Back 1413

1. HPV --> discrete flesh colored papules, condyloma acumulata = multiple external warts on genital area
2. liquid nitrogne, salicyclic acid, podophyllin, surgery

Front 1414

molluscum contagiosum
1. pathogenesis + clinical presentation
2. tx

Back 1414

1. mollusci pox virus --> flesh colored papules with central umbilication ~~~ very high numbers of eruptions in pts with HIV
2. resolves without tx , can also use curettage, podophyllin, 0.9% catharidin, liquid nitrogen

Front 1415

louse infection
1. pathogenesis + clinical presentation
2. tx

Back 1415

1. pediculus humanus, phthirus pubis --> sharing of clothes, combs--> pubic lice attaches to skin and ingests blood --> itching
2. head lice- 1% permethrin shampoo _ comb, 5% permethrin/malathion for resistant lice,
body lice - 12 hour application of 1% y-benzene hydrochloride lotion

Front 1416

scabies
1. pathogenesis + clinical presentation
2. tx

Back 1416

1. sarcoptes scabiei --> papules or veseicles in the abdomen, dorsum of hands, groin, axilla, flexor surfaces of wrist/interdigital spaces + SEVERE ITCHING + S shaped BURROWS
2. DX = examination shows mite eggs or mite feces
TX = overnight permethrin lotion, or 1% lindane

Front 1417

pityriasis alba

Back 1417

similar to atopic dermatitis, hypopigmented dry scaly patches on the cheeks

Front 1418

vitiligo + tx

Back 1418

loss of skin pigmentation
tx = psoralen + UV light

Front 1419

tuberous sclerosis
1. pathogenesis + clinical presentation

Back 1419

1. AD mutation -->
cerebral sclerotic tubercles --> myoclonic seizures
ASH LEAF SPOTS (hypopigmented oval shaped macules seen underwoods lamp
ADENOMA SEBACUM - angiofibromas on the nose/face
SHAGREEN PATCH - thickened orange peel like skin
INFANTILE SPASMS,intracranial calcifications,
renal cysts, cardiac rhabdomyomas( #1 pediatric cardiac tumor)
retinal astrocytoma/hamartoma

Front 1420

neurofibromatosis type 1
1. pathogenesis + clinical presentation

Back 1420

1.AD mutation (defect in neural crest differentiation)--> cafe aulait spots (>6), axillary/inguinal freckleiing, skin neurofibromas, optic glioma, intracranial calcifications, lisch nodules (iris hamartomas), CNS neurofibromas, scoliosis, hypertension

Front 1421

nevocellular nevi
1. congenital nevi + risk
2. acquired nevi

Back 1421

1. before 6 months of age, black/brown/tan inc. risk of malignancy, giant nevi >20cm have 7% chance of forming melanomas
2. forms between age 2 and 18 years old, usually junctinoal nevi, lower risk of malignancy

Front 1422

alopecia areata
1. pathogenesis + clinical presentation
2. tx

Back 1422

autoimmune lymphoccyte mediated hair follicle damage--> complete hair loss complete hair loss + pitting of nails
2., hair regrowth wihtin 1 year accelerated with steroids or minooxidil

Front 1423

traumatic hair loss

Back 1423

associated with anxiety

Front 1424

traction alopecia

Back 1424

hair loss assoicated with hair bands, find patchy areas of alopecia

Front 1425

telogen effluvium

Back 1425

acute stressful even --> forces hair to change from anaogen to telogen--> generalized hair loss (>100 hairs/day)

Front 1426

conditions that can cause hair loss

Back 1426

hypothyroidism, diabetes, hypopituitarism, hypervitaminosis A, zinc deficiency, marasmus, warfarin, heparin, chemotherpay,

Front 1427

acne vulgaris
1. pathogenesis + clinical presentation
2. tx

Back 1427

1. excessive coheision of cells in sebaceous follicles + sebum --> comedones --> proliferaiton of propionibacterium acnes occurs 1-2 years before puberty
2. benzoyl peroxide

Front 1428

open comedones
closed compedones

Back 1428

blackheads
whiteheads

Front 1429

acutane

Back 1429

acne treatment, highly teratogenic, women must be using birth control while on this drug

Front 1430

lichen planus

Back 1430

intensely pruritic raised papular rash is found on flexoral surfaces of the wrists, forearms, inner thighs,

Front 1431

auspitz sign

Back 1431

a drop of blood is formed when a scale from a psoriasis patch is removed

Front 1432

1. how to treat mammalian bites
2. how to treat cat, human, monkey bites
3. what bacteria in human bites + tx

Back 1432

1. soap and water (only 4% get infected)
2. abx prophylaxes (35-50%)
3. viridans strep, s.aureus, bacteroides, amoxicillin clavulanate, erythromycin

Front 1433

what is the screening test for lead poisoning

Back 1433

blood lead level

Front 1434

child just had a seizure and mom says she dilutes the fomrula

Back 1434

hyponatremia from too much water

Front 1435

sebaceous nevi

Back 1435

small sharply edged slightly raised yellow-orange hairless lesions that occur on the head and neck

Front 1436

somnambulinism

Back 1436

sleepwalking occurs in 15% of children usually occurs during stage 4 non-REM sleep

Front 1437

somniloquy

Back 1437

sleeptalking, can occur in any phase of sleep

Front 1438

most common age for
1. choking
2. drowning
3. pedestrian injuries
4. baby walker injuries
5. accidental poisoning

Back 1438

1. 1 year
2. 2 years
3. 6 years
4. 6 month
5. 2 years

Front 1439

why is a corn rich diet more likely to lead to pellegra

Back 1439

corn is poor in tryptophan which leads to low niacin --> PELLEGRA

Front 1440

rachitic rosary

Back 1440

enlargment costochondral junctions seen in vitamin D deficient rickets

Front 1441

warrdenberg syndrome

Back 1441

autosomal dominant-
iris abnormalities - sectoral heterochromia/heterochromia
poliosis (forelock of white hair)
wide set eyes,broad nasal root,
hearing loss

Front 1442

Treacher Collins syndrome (mandibulofacial dystosis)

Back 1442

autosomal dominant- downslanting eyes, micrgnathia, missing face bones, conductive hearing loss, downslanting palpebral fissures

Front 1443

treatment of aspirin poisoning

Back 1443

acetazolamide (CA inhibitor) and sodium bicarb

Front 1444

phenothiazine toxicity symptoms + tx

Back 1444

extrapyramidal symptoms, oculogyric crisis, tremors, torticollis, opisthotonus, dysphagia
tx = DIPHENHYDRAMINE

Front 1445

phenothiazine

Back 1445

antipsychotic/antihistaminic class
drugs include chlorpromazine (dopamine antagonist), promethazine (antiemetic, sedetive)

Front 1446

vitamin toxicities
1. vitamin a
2. nictotinic acid
3. vitamin C
4. vitmain D
5. vitmain B6

Back 1446

1. hyperosteosis + slowed growth, hepatomegaly, inc. CSF pressure, drying of skin
2. flushing/pruritus, tachycardia, liver damage, hyperglycemia/hyperureciemia
3. kidney stones, diarrhea, cramping
4. Nausea, diarrhea,calcification of heart/kidney/BV
5. sensory neuropathy

Front 1447

which vitamin should be given to children with measels

Back 1447

vitamin A, decreaes measels releated mortality

Front 1448

why must vitamin K be supplemented for newborns

Back 1448

immature intestinal flora leads to low vitamin K levels

Front 1449

how does maternal phenobarbital use effect neonatal jaundice

Back 1449

decreases jaundice because phenobarbital induced glucuronyl transferase

Front 1450

when should VZIG be given

Back 1450

maternal onset of varicella within 5 days prior to delivery, or if onset is <2 days after delivery

~ outside of this time frame, infants have no higher risk of complicatiosn than older children

Front 1451

1. what is basal metabolic rate in cal/kg*day for: newborn, 1wk-6mo, 6mo-1year,
2. what is approximate caloric number needed for growth and activity for ages 1-3
3. what is typical caloric density for formula

Back 1451

1. 50, 65, 60
2. 100 cal/day
3. 0.67 calories/ml

Front 1452

what does the Apt test detect

Back 1452

maternal/fetal hemoglobin in a bloody specimen such as vomit or melena~~ identifies the source of the blood--- treating specimen with alkali turns mother hemoglobin to hematin and fetal hemoglobin is unchanged

Front 1453

what drugs are contraindicated during breast feeding

Back 1453

lithium, cyclosporine, anti-neoplastics, ergotamines, bromocriptine, illicit drugs

Front 1454

which electrolyte is severely deficient in breast milk and poses risk for electrolyte imbalance in the neonate

Back 1454

calcium

Front 1455

how does a neonate adapt to a cold environment, what are the consequences if the neonates is not brought to a warmer ambient temperature

Back 1455

increasing metabolic rate, increasing heat production from brown fat
if they are not brought to warmer temperature, lactate will accumultae and resultating metabolic acidosis and compensatory hyperventilation

Front 1456

apneic episodes in preterm infants (NOT periodic breathing)

Back 1456

>20s pauses due to undeveloped respiratory center

Front 1457

complete metabolic panel (CHEM12)

Back 1457

electrolytes - Na, K, CO2, chloride
kidney - BUN, creatinine
*proteins- Albumin, total protein
*liver tests - ALP, AST,ALT, Bilirubin
glucose and calcium

Front 1458

basic metabolic panel (CHEM7)

Back 1458

electroloytes - Na, K, CO2, chloride
kidney - BUN and creatinine
glucose and calcium

Front 1459

for a neonate with jaundance, lethargy, distended abdomen, dec. ins/outs what should be initial treatment

Back 1459

thyroixine - 10-15mg/kg

Front 1460

neonate with erbs or klumpkes palsy has tachypnia and respiratory distress- test + diagnosis

Back 1460

chest fluroscopy and ultrasound shows paralyzed diaphragm due to damage to damaged C3/C4/C5 phrenic nerve roots

Front 1461

what is normal birth weight

Back 1461

7 pounds 8 ounces, 3400 grams

Front 1462

why does SGA neonatal hypoglycemia and polycythemia occur in mothers who have gestational hypertension or toxemia

Back 1462

placental infarction/toxemia decreaes uteroplacental blood flow leading to dec. glycogen storage--> compensatory hypoglycemia and polycythemia

Front 1463

what are the manifestations of polycythemia in a SGA infant
+ tx

Back 1463

hyperviscosity syndrome - seizure, tremulousness, jitteriness--> seizure activity due to slow blood in the brain + renal vein thrombosis, NEC, tachypnea
tx = partial exchange transfusion

Front 1464

propranolol effect on fetus

Back 1464

dec. heart rate, dec. ability to compensate during asphyxia, hypoglycemia/apnea

Front 1465

at what level of bilirubin does kernicterus occur

Back 1465

>18mg/dl

Front 1466

evaluation of jaundice in a newborn

Back 1466

maternal/infant Rh types + ABO groups + coombs
total, direct, indirect bilirubin
Hct, reticulocyte count
CBC (to screen for sepsis)

Front 1467

what is the frequency of cleft palate in siblings who have cleft palate vs general population

Back 1467

4% for siblings
general population is 1/1000

Front 1468

transient tachypnea of the newborn

Back 1468

healthy infant, normal birth, --> respiratory distress and inc. pulmonary vascular markings with fluid fissures and hyperexpansion
tx= oxygen to maintain oxygen saturation

Front 1469

what must be investigated in a black vs white child with polydactyly

Back 1469

black child - nothing, 10x more common in black children

white child - look for heart defects, inc. incidence of heart defects

Front 1470

what are the causes of apnea in a HEALTHY TERM infant

Back 1470

***major pathological issue (compare to preterm)

sepsis, GERD, CHD, seizures, hypoglycemia****

Front 1471

kernig sign,
bruduzinski's sign

Back 1471

cannot extend the knee with the hip flexed

flexing the neck causes hip flexion

Front 1472

what is the important clinical finding for twin transfusion syndrome

Back 1472

difference in hematocrit >15mg/dl between two infants

donar = oligohydrominos, anemia, hypovolemi
recipient = polyhydraminos, plethora, with evidence of shock

Front 1473

longitudinal striations on the metaphysis TORCH infection

Back 1473

congenital rubella

Front 1474

osteochondriitis/periostitis TORCH infection

Back 1474

congenital syphillus

Front 1475

bronchiolitis treatment

Back 1475

inhaled epinephrine

Front 1476

what is the clinical presentation of a tension pneuothorax + tx

Back 1476

dec. lung sounds + displaced heart sounds

tx = immediate needle aspiration and chest tube placement

Front 1477

bronchopulmonary dysplasia

Back 1477

results from intubation --> persistant oxygen demands, respiratory distress, and abnormal cxr, airway hyperresponsiveness, PULMONARY EDEMA REQUIRING DIURETIC TREATMENT

Front 1478

biotin deficiency/biotinidase deficiency clinical presentation

Back 1478

dermatitis, alopecia, ataxia, hypotonia, seizures, deafness, immunodeficiency, metabolic acidosis

Front 1479

what is the treatment for rheumatic fever chorea

Back 1479

chlorpromazine or barbiturates
NOT antibiotics or salicylates

Front 1480

what is the most common clinical criteria in rhuematic fever

Back 1480

arthralgias

Front 1481

hereditary angioedema patho + clinical presentatino

Back 1481

C1 esterase inhibitor deficiency
--> angioedema of the hands and feet + GI symptoms + laryngeal edema/obstruction

Front 1482

Ellis-van Creveld syndrome

Back 1482

(defect is 4p16) is associated
with atrial septal defects.

Front 1483

what is the risk of congenital heart disease for a fetus who has a sibling with congenital heart disease

Back 1483

2-6%
general population = 1%

Front 1484

what neonatal history accompanies tricuspid regurgitation

Back 1484

history of asphyixia or other heart defects

Front 1485

MCC viral myocarditis

Back 1485

adenovirus and coxsackie B virus

Front 1486

what are the MCC of SVT (pulse >250)

Back 1486

WPW
CHD
sympathomimmetic drugs

Front 1487

clinical features of WPW

Back 1487

prolonged PR
SVT
delta wave

Front 1488

loud snoring and suspected sleep apnea, what is the work up/treatment

Back 1488

polysomnography
CPAP/BIPAP

Front 1489

what is the potentially life threatening consequence of lobar pneumonia (staph) + treatment

Back 1489

tension pneumothorax + treatment = chest tube or needle aspiration at the 2nd or 3rd intercostal space

Front 1490

what is the major complication of laryngotracheobronchitis + clinical pres + tx

Back 1490

bacterial tracheitis
presentation = several days of viral URI-->acute fever + respiratory distress
tx = airway management

Front 1491

what is the modern (vaccine era) cause of epiglottitis

Back 1491

s.pneumo, GAS, moraxella

Front 1492

when do sphenoid and frontal sinuses become large enough to harbor infections

Back 1492

frontal = 6th-10th year of life
sphenoid = 5th year of life

Front 1493

clinical presentation of esophageal obstruction

Back 1493

cough, drooling, choking, avoidance of liquids

Front 1494

what is the possible etiologies for chest pain in a sickle cell pt

Back 1494

pneumonia, thromboemboli, sepsis~~ risk of rapid progression

Front 1495

why is cyanosis a bad marker of poor oxygenation if Hbg < 5g

Back 1495

CANNOT SEE THE CYANOSIS

Front 1496

retropharyngeal abscess clinical presentation

Back 1496

pharyngitis, fever, severe sore throat, fluctuant bulge in posterior pharynx suppurative infection of the posterior pharyngeal wall by staph, GAS, or oral anaerobes

Front 1497

clinical presentatino + treatment of foreign body

Back 1497

recurrent pneumonias in a healthy child, decreased inspiratory and expiratory sounds
Dx = rigid bronchoscopy (only way to dx)
tx = rigid bronchoscopy

Front 1498

what are the most common complications of parenteral feeding

Back 1498

sepsis MCC
thrombosis of central lines
pneumothorax
brachial plexus injury
REVERSIBLE mucosal atrophy

Front 1499

hirschprung disease clinical presentation + diagnosis

Back 1499

prolonged constipation from birth, FAILURE TO PASS MECONIUM and signs of enterocolitis (late)
barium enema and manometry

Front 1500

how to diagnose meckles diverticulum

Back 1500

radionucleotide scan

Front 1501

depressed fontanelles, tachycardia, sunken eyes, loss of skin elasticity = ~ what % of dehydration

Back 1501

5-9% dehydrated

Front 1502

mallory weiss tears

Back 1502

repeated vomiting/retching --> streaked blood in the vomit ~ occasionally results in massive hemorrhage

Front 1503

behcet syndrome

Back 1503

vasculitis of small/medium sized arteries
non-destructive arthritis of multiple joints, fever, erythema nodosum, aphthous stomatitis, uveiits, CNS (incl pseudotumor cerebri)

Front 1504

wolman syndrome ~patho + clinical pres

Back 1504

lysosomal acid lipase deficiency --> calcification of the adrenal gland (KUB)
HSM, diarrhea, vomitting, FTT,

Front 1505

drugs that cause nephrotic syndrome

Back 1505

trimethadione, penicillamine**, catopril, probenicid, ethosuximide, methimazole, lithium, procainamide, chlorpropamide, phenytoin, paramethadione, tolbutamide, NSAIDs, gold, mercury

Front 1506

why is the bladder succeptible to rupture in children who have blunt trauma

Back 1506

it is an abdominal organ in a child

Front 1507

what is the management of MOST kidney injuries with the exception of traumatic vascular/ traumatic hydrocoeles

Back 1507

most = observation
vascular/hydrocoeles =

Front 1508

what drugs cause fanconis syndrome

Back 1508

gentamicin, OUTDATED tetracycine, cephalothin (1st gen cephalo), cidofovir (anti-CMV), valproic acid, streptozocin, 6-MP, azathioprine (6-MP prodrug), cisplatin, ifosfamide, heavy metals, paraquat, maleic acid, glue sniffing (toluene)

Front 1509

lithium effect on the kidney

Back 1509

nephrotic syndrome AND RTA

Front 1510

NSAIDS effect on the kidney

Back 1510

nephotic syndrome and interstitial nephritis

Front 1511

sulfonamides effect on the kidney

Back 1511

vasculitis and nephritis

Front 1512

how is low grade vesicoureteral reflux managed

Back 1512

daily antibiotics and urinalysis/culture every 3-4 months

Front 1513

renal osteodystrophy

Back 1513

chronic renal failure --> secondary hyperparathyroidism + rickets + osteitis fibrosa cystica, 1,25(OH)D3 deficiency

Front 1514

what type of anemia in renal failure

Back 1514

normochromic normocytic anemia due to dec. EPO

Front 1515

what are the iatrogenic complications of dialysis

Back 1515

blood loss
dialysis related aluminum toxicity
folic acid deficiency

Front 1516

what causes glucose intolerance/peripheral resistance in renal failure

Back 1516

elevated glucagon and growth hormone

Front 1517

clinical presentation of hyper tension in children

Back 1517

headache, dizziness, visual disturbance, irritability, nocturnal wakening

Front 1518

what is the major risk associated with cryptochordism in a child vs adult

Back 1518

torsion of the testicle because testicle is not fixed to the scrotal sac.

in adulthood major risk is testicular cancer, this IS NOT DECREASED BY ORCHIOPEXY it just makes the testicles easier to examine~ thus surgical intervention does not decrease the risk of testuclar torsion

Front 1519

what colony counts are significant in
1. suprapubic tap
2. midstream clean catch

Back 1519

1. >10^3 - 10^4
2. >10^5 in asymptomatic or >10^4 in symptomatic

Front 1520

how to decrease the edema in a patient with nephrotic disease

Back 1520

albumin and a diuretic combination

Front 1521

bartter syndrome

Back 1521

defect in Na/K/Cl cotransporter in thick ascending limb --> hypokalemia, alkalosis (inc. distal delivery of sodium), hyperaldosteronism, normotension, hyperreninemia

Front 1522

how should needle be advanced in spinal tap of an infant + what is the risk of using no styloid

Back 1522

small advances followed by removing the styloid
no styloid can introduce a core of epidermis into the subarachnoid space which can then form a epidermoid tumor

Front 1523

what is the early signs of myasthenia gravis
+ what are the EMG signs

Back 1523

ptosis, weakness of extraocular muscles, dysphagia and facial muscle weakness, rapid muscle fatiguing
EMG = decremental response during rapid muscle stimulation

Front 1524

acute infantile hemiplegia

Back 1524

~ DOES NOT NECESSARILY OCCUR ONLY IN INFANTS
thrombotic occlusion of the MCA or branches --> classic hemi-syndrome with eyes looking away from the paralyzed side

Front 1525

todds paralysis

Back 1525

post focal or jacksonian seizure, lasts only 24-48 hours

Front 1526

enzyme defec+ clinical pres in:
1. tay sachs
2. gauchers
3. niemann pick
4. krabbes
5. fabrys
6. metachromatic leukodystrophy
7. farber disease

Back 1526

1. hexominidase A - 6-12mo inc. startle + cherry red spot, hypotonia, loss of milestones
2. beta glucosidase - erlenmeyer flask femur, HSM, late onset
3. sphingomyelinase- cherry red spot, HSM death by 2 or 3
4. galactocerebroside beta galactosidase
5. alpha galactosidase --> late childhood onset, acroparesthesia, painful crisis (of abdomen), cataracts
6. galactosyl 3 sulfate ceremide sulfatase - age 1-2 years onset --> peripheral neuropathy, progressive ataxia, gray macular lesions
7. ceramidase --> bone pain and joint swelling

Front 1527

CSF shows lymphocytic pleocytosis, elevated protein, lowered glucose

Back 1527

viral meningitis,

Front 1528

medulloblastoma spinal tap CSF findings

Back 1528

monocytic** pleocytosis

Front 1529

GM1 gangliosidoses type 1 patho + clinical presentation

Back 1529

complete absence of acid - beta galactosidase *******--> infantile onset hypotonia (compare to tay sachs) --> HSM, CNS degeneration, ant. breaks in cervical vertebrae, enlargement of the sella, thickening of calvarium (dystosis multiplex)

Front 1530

rett syndrome pathogenesis + clinical presentation

Back 1530

X chromosome MeCP2 mutation-->rapid decline at 6-18 months of age --> dec. ability to communicate/socialize

Front 1531

congenital CMV vs toxoplasmosis intracranial calcifications

Back 1531

CMV = periventricular
toxo = coritcal

Front 1532

incontinentia pigmenti

Back 1532

familial skin blistering and seizures

Front 1533

guilllan barre spinal tap findings

Back 1533

protein HIGH (2x)
glucose normal
cell counts normal

Front 1534

charcot marie tooth clinical presentation

Back 1534

PMP 22 mutation --> peripheral demyelination--> atrophy of the peroneal and intrinsic foot/hand/proximal leg muscles

Front 1535

tick paralysis- pathogenesis + clinical presentation

Back 1535

eastern dog tick/rocky mountain wood tick--> toxin injection via saliva--> ataxia/areflexia--> prgressive ascending paralysis

Front 1536

scorpion sting clinical rpesentation

Back 1536

shock, salivation, convulsions
tx - phenobarbital (not morphine), and antivenin

Front 1537

transient tic disorder

Back 1537

eye blinking, facial movements, throat clearing, resolves in about a year

Front 1538

what virus must a transfusion be screened for before giving to an HIV patient

Back 1538

CMV

Front 1539

parvo b19 infection of pregnant mother effect on the fetus

Back 1539

severe anemia, hydrops fetalis, and death of the fetus

Front 1540

bacterimia in an infant causes

Back 1540

s.pneumo, h.flu, meningococcus

Front 1541

bacteremia in an infant clinical presentation

Back 1541

fever, left shift + leukocytosis, headache, malaise, poor feeding

Front 1542

orbital vs periorbital (pre-septal) cellultitis

Back 1542

orbital cellulitis- shows proptosis, pain, ophthalmoplegia, and dec. visual acuity
periorbital cellulitis- has full range of motion and no effect on vision

Front 1543

hepatitis A clinical presentation

Back 1543

sudden onset fever, N/V, tenderness of the liver, jaundice, AST elevation <3 weeks,

Front 1544

trichinella spiralis clinical pres

Back 1544

abdominal pain, N/V --> muscle invasion, edema of the eyelids, myalgia, weakness, fever, eosinophilia

Front 1545

job-buckley syndrome

Back 1545

defect in phagocytic chemotaxis- hypergammaglobulin E, PRURITIC DERMATITIS, eczema, EOSINOPHILIA, severe RECURRENT staph infections

Front 1546

what are the complications of staph pneumonia (most commonly seen in children <1 year of age)

Back 1546

pleural effusion, empyema, pyopneumothorax

Front 1547

how does IVIG work to treat ITP

Back 1547

it saturates the RES binding sites for platelet bound self immunoglobulin

Front 1548

what physical exam finding should be taught to parents whose children have sickle cell disease

Back 1548

palpable spleen, to detect ACUTE SPLENIC SEQUESTRATION which can lead to hypovolemia/circulatory collapse

Front 1549

physiologic anemia of infancy

Back 1549

6-8 weeks of age: erythropoiesis temporarily stops after birth and physiologic anemia reaches its nadir at 6-8 weeks

Front 1550

fetomaternal transfusion

Back 1550

fetal blood loss to mother during birth, occurs during normal/abnormal pregnancies

Front 1551

what are the causes of blood eosinophilia

Back 1551

asthma
recrrent urticaria
infantile eczema
serum sickness
helminth infections
collagen vascular disease

Front 1552

what are the causes of spherocytes

Back 1552

hyperthermia
hereditary spherocytosis
G6PD deficiency
ABO incompatibility (NOT RH INCOMPATIBILITY)

Front 1553

treatment for iron deficiency in an infant between the ages of 9 and 15 months

Back 1553

oral ferrous sulfate ~~ expect to see reticulocytosis and rise in hemoglobin/hematocrit

Front 1554

what toxicity is associated with methotrexate

Back 1554

MUCOSITIS (unique), bone marrow suppression, skine erythema, heaptic dysfunction

Front 1555

what toxicity associated with vincristine

Back 1555

peripheral neuropathy, constipation, jaw pain, SIADH

Front 1556

doxorubicin toxicity

Back 1556

CARDIOTOXICITY, alopecia, N/V, stomatitis, tissue necrosis

Front 1557

what materanl disease is associated with
1. neonatal high output heart failure
2. third degree heart block

Back 1557

1. maternal hyperthyroidism/graves
2. maternal SLE

Front 1558

bone age vs height age vs chronologic age in
1. constituional short stature
2. familial short stature

Back 1558

1. bone age/height age the same and both are less than chronological age (BOTH BONE AGE AND HEIGHT AGE ARE LOW)
2. bone age > height age, bone age = chronological age (ONLY HEIGHT AGE IS LOW)

Front 1559

how to assess testicles ability to produce testosterone

Back 1559

administer B-HCG to stmulate the testicles to produce testosterone ~ this is a method to assess testicular function

Front 1560

what are the possibilities for the causes of a cold thyroid nodule

Back 1560

any cancer (anaplastic, folliculary, medullary)
BUT A DYSGENIC THRYOID WILL NOT APPEAR COLD

Front 1561

effects on bone age
1. excess androgens (ex. CAH)
2. thyroid deficiency
3. glucocorticoid excess
4. inorganic FTT

Back 1561

1. increased bone age and early fusion of the growth plate
2. decreased bone age
3. increased boen age and rapid fusion of the growth plates
4. decreased bone age due to low pituitary hormones

Front 1562

what electrolyte imbalance is seen in an immobilized fracture of a weight bearing limb (aka. IMMOBILIZATION HYPERCALCEMIA)
+ tx

Back 1562

hypercalcemia --> high BP, altered mental statuis, encephalopathy, convulsions
2. diet restriction of dairy, diuretics, calcicitonin

Front 1563

alpha 1 antitrypsin deficiency clinical presentation

Back 1563

cholestasis, bleeding into CNS/GI tract, or umbilical stump, elevated transaminatses, portal hypertension,

Front 1564

hypoglycemia in a premature infant born to a normal mother

Back 1564

diminished glycogen and fat stores --> post partum hypoglycemia

Front 1565

medullary thyroid carcinoma calcium and phosphours

Back 1565

NORMAL NORMAL (despite elevated calcitonin)

Front 1566

diabetes insipidus effect on Na and K leveles

Back 1566

both go up due to hemoconcentration

Front 1567

what age in childhood has the highest risk for diabetes inspidus

Back 1567

neonatal period

Front 1568

cystinosis

Back 1568

autosomal recessive lysosomal storatge disease of cystine leads to crystalline deposits in the cornea, FANCONI SYNDROME (MCC), renal failure, FTT

Front 1569

list some mitochondrial disorders

Back 1569

myoclonic epilepsy and ragged red fibers
MELAS
lebers hereditary optic neuropathy

Front 1570

side effects of anabolic steroids

Back 1570

liver damage, dec. testicular size, oligosperrmia, aggression, mood swings, dec HDL, premature closure of the epiphysis

Front 1571

which antidepressant used in anorexia nervosa

Back 1571

imipramine

Front 1572

what is the temporal sequence between genital ulcer and inguinal lymphadenopathy in chlamydia vs h.ducreyi

Back 1572

chlamydia- ulcer disappears then inguinal adenoaphty forms
h.ducreyi - ulcer and inguinal adenopathy occur at the same time

Front 1573

what type of injuries with
1. swimmers
2. football
3. basketball/volleyball
4. running
5. ballet
6. wrestlers
7. skiing

Back 1573

1. rotator cuff
2. knee and foot, head/neck
3. osgood schlatter, ankle
4. patellofemoral, muscle strains
5. delayed menarche/eating disorders
6. shoulder subluxation, pre-patellar bursisits, skin conditions
7. ulnar collateral ligament

Front 1574

thalassemia vs IDA RDW

Back 1574

RDW increased in IDA (inc. variablility of red blood cell width)
RDW normal in thalassemia trait (TIBC and ferritin normal)

Front 1575

post pericardiotomy syndrome

Back 1575

reactive pericarditis/pericardial effusion after surgery for congentital heart disease in an infant

Front 1576

how to remove a foreign body from a childs vagina

Back 1576

first try warm water irrigation to flush it out
if this doesnt work remove it under general anesthesia

Front 1577

causes and clinical presentation of airway compromize by a vascular ring

Back 1577

double aortic arches,right sided aorta, pulmonary sling etc
-- symptoms worse when supine, relieved by neck extension, no response to bronchodilators/corticosteroids

Front 1578

what treatable underlying condition can be predispose to breath holding spells/pallid spells

Back 1578

iron deficiency anema
treat by giving oral ferrous sulfate and iron rich cereal

Front 1579

what kind of fractures lead to compartment syndrome

Back 1579

supracondylar fractures of the radius and humerus

Front 1580

patellofemoral stress syndrome

Back 1580

commonly seen in runners, pain when descending stairs

Front 1581

treatment of breastfeeding jaundice

Back 1581

increase feeding frequency and duration to increase breast milk production which will reduce dehydration and reduce transit time and dec. enterohepatic circulation

Front 1582

riboflavin deficiency clinical presentation

Back 1582

glossitis, seborrheic dermtitits (of groin), chelosis, pharyngitis, edema/erythema of the mouth

Front 1583

pyridoxine deficiency clinical presentation

Back 1583

peripheral neuropathy, irritability, depression, dermatitis, stomatitis, elevated homocystine

Front 1584

sail sign

Back 1584

<2 year old CXR - large thymic shadow overlaps with the heart and looks like a "boat sail"

Front 1585

what are the symptoms of polycythemia in an infant + MCC

Back 1585

due to hyperviscosity of blood: respiratory distress, seizures, cyanosis, jitteriness, irritability, poor feeding
MCC = placental blood transfer due to delayed clamping of the cord

Front 1586

charcots arthritis

Back 1586

joint destruction from neurological loss --- ex. diabetic neuropathy, syringomyelia, spine injury, B12 deficiency, tabes dorsalis

Front 1587

why do antipsychotics cause amenorrhea

Back 1587

dopamine antagonsits results in inc. prolactin which inhibits the HPG axis causing amenorrhea

Front 1588

how should vaccines be administered to a preterm infant

Back 1588

according to chronological age (after birth) not gestational age----- there is no significant reduction of immune response in preterm infants so vaccines are OK...... BUT NO HEPATITIS B until infant is <2kg***

Front 1589

lead poisoning diagnosis in a child

Back 1589

first fingerstick blood lead level >10ug/dL
confirm with serum lead level >10ug/dL

Front 1590

congenital syphilis clinical presentation

Back 1590

lesions on palms/soles, jaundice, anemia, RHINORRHEA, periostitis, metaphyseal dystrophy, Hutchinson teeth, saddle nose,

Front 1591

90% of viral meningitis

Back 1591

echovirus (enterovirus) and coxsackie

Front 1592

kid falls and hits his head with a pencil in his mouth, 24 hours later stroke like symptoms

Back 1592

INTERNAL CAROTID ARTERY DISSECTION

Front 1593

MCC diarrhea in children between age 6 months to 2 years

Back 1593

rotavirus

Front 1594

MCC adult diarrhea in the united states

Back 1594

campylobacter

Front 1595

what is the auscultation finding of pulmonary hypertension (often from congenital heart disease)

Back 1595

LOUD P2 *****

Front 1596

leukocyte adhesion deficiency clinical presentation

Back 1596

neutrophilia, delayed seperation of the umbilical cord, recurrent bacterial infections, gingivitis/peridontitis--> LOSS OF TEETH

Front 1597

when should a child get a lumbar puncture for a febrile seizure

Back 1597

<12 months
uncertain neurological exam

**DONT GIVE LP FOR >18 MONTHS WITH NORMAL NEUROLOGICAL EXAM AND NO SIGNS OF MENINGITISA

Front 1598

most common case of ESRD in children

Back 1598

renal scarring secondary to reflux
dx. with VCUG

Front 1599

two galactose related metabolic disorders

Back 1599

galactose 1 - phosphate uridyl transferase deficiency (severe hepatomegaly, mental retardation, vomitting, diarrhea)
galactokinase - mild (just cataracts)

Front 1600

what is first step in evaluation of a child with a speech delay ie. dec. babbeling, or not meeting language milestones

Back 1600

audiology evaluation

Front 1601

renal masses
1. at birth
2. <3 years of age
3. >3 years of age

Back 1601

1. hydronephrosis
2. neuroblastoma (neural crest) <1 year of age
3. wilms tumor (metanephros) age 2-5

Front 1602

hyper IgM

Back 1602

high IgM, all other immunoglobulins are low (compare to brutons*) + neutropenia
~ recurrent sinopulmonary infections, PCP pneumonia , poor resposne to immunizations

Front 1603

TTP clinical presentation

Back 1603

PENTAD- thrombocytopenia, MAHA, neurologic disturbance, fever, renal dysfunction

Front 1604

is premature adrenarche, thelarche, or pubarche concerning

Back 1604

only pubarche is concerning because this is associated with CNS disorder

Front 1605

breastfeeding contraindications

Back 1605

galactosemia/PKU/urea cycle defects
maternal use of antimetabolits, chemotherapy, radioisotopes
illicit drug use
HSV (w/ breast lesions), active TB**, HIV

Front 1606

signs of rapid onset of precocious puberty and androgen excess

Back 1606

pseudo puberty - independent of of the HPG axis ~~~ ex. late onset CAH, exogenous androgens, or androgen secreting tumor

Front 1607

A infant has RSV, what condition is this patient now at a higher risk for

Back 1607

asthma

Front 1608

treatment of impetigo

Back 1608

mupirocin--- b/c it covers gram positives as well as MRSA

Front 1609

what is the most common cause of communicating hydrocephalous

Back 1609

interventricular hemorrhage (subarachnoid hemorrhage) leading to destruction of the arachnoid villi and cisterns blocking flow of CSF

Front 1610

clinical presentation of hydrocephalous in a neonate

Back 1610

lethargy, prominent scalp viens, DOWNWARD GAZE, tense fontaneles, poor suckling, hypotonia

Front 1611

varicella post exposure prophylaxis

Back 1611

VZIG or acyclovir ~ perferable withing 72 hours of expsure

Front 1612

what is the hematologic finding in SGA infants

Back 1612

inc. risk of hypoxia/asphyxia/MAS--> polycythemia
~ also see hypothermia, hypoglycemia, hypocalcemia

Front 1613

what are the steps of early neonatal care for an uncomplicated pregnancy

Back 1613

removal of airway secretions
drying and wrapping in blanket
silver nitrate eye drops/vitamin K supplementation

Front 1614

how to treat chlamydia conjunctivitis in an infant

Back 1614

ORAL SYSTEMIC erythromycin due to the risk of chlamydia pneumonia

Front 1615

what is the content of breast milk

Back 1615

70% whey, 30% casein, lactose
lactoferrin, lysosyme, secretory IgA

Front 1616

what vitamins are inadequate in breast milk

Back 1616

vitamin D

Front 1617

most common cause of subarachnoid hemorrhage in children

Back 1617

AVM~~~ associated with childhood seizures

Front 1618

peritonsillar abscess clinical presentation

Back 1618

unilateral tonsillar swelling and uvular deviation

Front 1619

jaundice
1. in the first 24 hours
2. """ 2nd or 3rd day
3. 3rd day to 7th day

Back 1619

1. erythroblastosis fetalis, concealed hemorrhage, sepsis or congenitla infections
2. usually phsyiologic
3. bacterial sepsis or UTI (often lacks classic poor feeding, bulging fontanel) --> LP/blood cultures

Front 1620

guthrie test

Back 1620

test that detects metabolic products in the urine ex. PKU

Front 1621

laryngomalacia pathogenesis and clinical presentation

Back 1621

congenital flaccid larynx appears as epiglottis rolling on laryngoscopy--- presents as inspiratory respiratory sounds that are improved when the child is prone
~~ approves with time as the child ages
tx = feed the child while upright and keep the child upright for 30mins after feeds

Front 1622

denys drash

Back 1622

syndrome associated with wilms tumor, mesangial sclerosis/renal failure, pseudohermaphrodism

Front 1623

neotnatal tetanus pathogenesis and clinical presentation

Back 1623

unimmunized mother/unclean obstetric techniques outside hospital leads to umbilical stump infection with tetanus --> erythematous umbilical stump, spasms, opisthotonus, poor feeding

Front 1624

what is the first step when an infant has fever >102.2, signs of septic shock, and a ***bulging fontanelle

Back 1624

empiric antibiotics first
then CT scan (NOT LP FIRST) to see if there is a mass that will lead to herniation. If there is not then can proceed with the LP

Front 1625

clinical pres + treatment for infant clavicle fracture

Back 1625

crepitus over clavicle, and decreased movement of the ipsilateral armf

tx = nothing, reassurance

Front 1626

what is the gold standard of GERD diagnosis

Back 1626

24 hour reflux monitoring

Front 1627

neonatal abstinence syndrome

Back 1627

infant born to heroin/methadone addicted mother
sneezing, tachypnea, irritability, poor feeding/sleeping, vomitting, diarrhea,

Front 1628

interventricular hemorrhage pathogenesis + clinical presentation

Back 1628

PREMATURITY/LOW BIRTH WEIGHT --> bulging fontanelles, pallor, cyanosis, focal neurologic signs, hypotension

Front 1629

what is the major risk in 46XY turner females

Back 1629

gonadoblastoma--- must do bilateral gonadectomy

Front 1630

anemia of prematurity

Back 1630

normochromic normocytic anemia, due to dec. production, shorter half life of RBCs

Front 1631

tricyclic antidepressant overdose treatment

Back 1631

sodium bicarbonate, helps to normalize the wide QRS seen overdose

Front 1632

homocystinuria clinical presentation + tx

Back 1632

marphans like appearance, downward subluxation of the lens, and tx = B6

Front 1633

how to treat a patient with croup whose O2 sat is falling

Back 1633

try inhaled epinephrine , if this does not work then intubate