Clerkship - Pediatrics

Front 1

What are the MC bacterial causes of community acquired pneumo?

Back 1

S. pneumoniae
GAS, S. aureus, H. flu (less common)
Mycoplasma pneumo, Chlamydia pneumo

Front 2

What are the MC viral causes of CAP?

Back 2

RSV (<3 yo)
Adenovirus, Parainfluenza, Influenza, Human metapneumovirus

Front 3

The majority of reported cases of CAP have what etiology?

Back 3

Mixed (30-50%)

Front 4

What are the best physical exam measures of CAP in children < 5yo?

Back 4

Signs of respiratory distress:
nasal flaring (esp < 12 mos.)
O2 stat < 94%
tachypnea
retractions

Front 5

For each age group, what are the normal respiratory rates and tachypnea thresholds?
2-12 mos.
1-5 yrs.
>/= 5 yrs

Back 5

2-12 mos: nl RR 25-40 BPM, Theshold 50 BPM
1-5 yrs: nl RR 20-30 BPM, threshold 40 BPM
>/= 5 yo: nl RR 15-25, threshold 20 BPM

Front 6

According to the Evidence-based Care guidlines for Children with CAP, which of the following are recommended assessment tools?
CXR
CBC
Blood cultures
CRP
ESR
sputum Gram stain
rapid viral studies

Back 6

None are recommended for unambiguous standard CAP:
CXR - doesn't alter management or improve outcomes
CBC - WBC needed only if questioning use of antibiotics
CRP and ESR - not specific enough
Sputum stain - only in severe cases

Front 7

The pathogens of CAP are the same as the pathogens for what other common childhood illness?

Back 7

Acute otitis media (AOM)

Front 8

Resistance of S. pneumo to penicillin (inlcuding amox) is mediated through what mechanism?

Back 8

Alterations in penicillin-binding proteins.

Front 9

What is the first line therapy for CAP caused by M. pneumo and C. pneumo?

Back 9

Macrolides - Azithromycin

Front 10

Hospital admission for CAP should be considered in pts with what criteria?

Back 10

1.O2 stat < 91%
2. severe dehydration
3. moderate - severe respiratory distress
4. failed antibiotic treatment
5. clinician discression

Front 11

According to the AAP Clinical Practice Guidelines, what are the major risk factors for Neonatal Severe Hyperbilirubinemia?

Back 11

1. Predischarge TSB of TcB in the high risk zone**
2.Jaundice observed in the first 24 h
3. Blood group incompatibility with + direct Coomb's
4. Other hemolytic dz (e.g., G6PD)
5. Early gestational age
6. Previous sibling needing light therapy**
7. Cephalohematoma or significant bruising
**Most significant factors

Front 12

What are the signs of intermediate/advances stages of acute bilirubin encephalopathy?

Back 12

1. hypertonia
2. arching/opisthotonos
3. retrocollis (involumtary neck movements to the L,R, up and down)
4. fever
5. high pitched cry

Front 13

A TSB that does not decrease or continues to rise in an infant receiving phototherapy strongly suggests which etiology of hyperbilirubinemia?

Back 13

Hemolysis

Front 14

What is the risk of a child with fever of uncertain source (FUS) developing meningitis?

Back 14

1:2,500 (0.04%)

Front 15

What is the MCC of FUS in children?

Back 15

viral infeciton

Front 16

What is the definition of fever?

Back 16

Rectal temp of at least 38 (100.4).

Front 17

T/F: a parental report of fever detected only by touch is likely to be accurate.

Back 17

True: sensitivity: 82-89% specificity: 76-86%

Front 18

What is the MCC of acute gastroenteritis in children?

Back 18

Rotavirus

Front 19

What are the signs/symptoms associated with bacterial gastroenteritis?

Back 19

bloody or mucoid stools

Front 20

Define simple febrile seizure.

Back 20

seizure accompanied by fever without CNS infection, occurring in infants between 6mo and 5 years
-primarily generalized seizures < 15 min and not recurring within 24 hours

Front 21

According to the AAP, which of the following diagnostic tests are recommended for presentation of simple febrile seizures:
LP
EEG
Blood studies
Neuroimaging

Back 21

LP- recommended for infants < 12 mos. and considered for 12-18 mos.
EEG- not recommended
Blood studies - not rec.
Neuroimaging - not recommended

Front 22

In pts with VSD, explain the pathophysiology behind the absence of a murmur in the neonate.

Back 22

Neonates are born with elevated pulmonary vascular resistance creating equal resistances to outflow from both R and L ventricles = no murmur

Front 23

What is the clinical picture of prolonged VSD?

Back 23

1. tachycardia and diaphoresis - increase in catecholamines
2. tachypnea - interstitial pulmonary edema
3. hepatomegally
4. congestive heart failure

Front 24

What is the treatment for VSD that does not close?

Back 24

Digoxin and diuretic
Dig - decreased catecholamines
Diuretic - relieves vascular congestion

Front 25

Developmental delay, intrauterine growth retardation (e.g, microcephaly), cataracts, seizures, and prolonged neonatal jaundice are findings consistent with what illness?

Back 25

Congenital infection by CMV or toxoplasmosis

Front 26

What is the difference between renal tubular acidosis (RTA) types 1 and 2?

Back 26

RTA is a common cause of failure to thrive
Type 1: impaired tubular reabsorption of bicarb in the distal tubules
Type 2: same as type 1 but in the proximal tubules

Front 27

What is the difference between substance abuse and substance dependence?

Back 27

Abuse: use leading to impairment of distress resulting in failure to meet social obligations, potential physical harm, legal problems or continued use despite social and person consequences
Dependence: use leading to loss of control with continued use, compulsion to obtain and use the substance

Front 28

Which drug causes a person to have dilated pupils, hyperthermia, paranoid ideation?

Back 28

Cocaine

Front 29

Which drug causes nystagmus, ataxia, hallucinations affecting body image that can result in panic attacks, and hypersalivation?

Back 29

PCP - Phencyclidine

Front 30

What are the most commonly used drugs in adolescence?

Back 30

Cigarettes and alcohol

Front 31

What is the confirmative test in an infant you suspect has Down Syndrome?

Back 31

Chromosomal analysis

Front 32

What is the most common cardiac defect in Down Syndrome infants?

Back 32

endocardial cushion defect

Front 33

An infant with Down Syndrome develops persistent vomiting after feeds. What would most likely be seen on upper GI study?

Back 33

"double bubble" pattern of duodenal atresia

Front 34

What are the three mechanisms for triploidy in Down syndrome, in order of prevalence?

Back 34

Nondysjunction (failure to segregate during meiosis) - 95%
Mosaics - 3%
translocations - 2%

Front 35

In children with Down Syndrome, what well-child care measures should be evaluated at annual exams?

Back 35

thyroid, hearing and vision screenings

Front 36

A child born with low-set, malformed ears, microcephaly, rocker-bottom feet, inguinal hernias, cleft lip and palate and micrognathia is likely to have which chromosome abnormality?

Back 36

Edward's Syndrome (Trisomy 18)

Front 37

A child that is SGA, dysmorphic with microcephaly, sloping forehead, cutis aplasia of the scalp, polydactyly, micropthalmia and omphalocele is likely to have which chromosome abnormality?

Back 37

Patau Syndrome (trisomy 13)

Front 38

A developmentally delayed child with microcephaly, round face and high-pitched cry most likely has what chromosomal abnormality?

Back 38

Cri-du-Chat: macrodeletion of the short arm of Ch 5.
85% are paternal in origin

Front 39

What causes the high-pitched cry in Cri-du-Chat?

Back 39

laryngeal maldevelopment, it diminishes as the child gets older.

Front 40

Which genetic disorder presents with low IQ, downturning of palpebral fissures, hypoplasia of maxilla, broad thumbs and toes, short stature?

Back 40

Rubinstein-Taybi syndrome

Front 41

What are the causes of noncyanotic congenital heart disease?

Back 41

VSD
ASD
PDA

Front 42

What are the causes of cyanotic congenital heart disease?

Back 42

Truncus arteriosis
Transposition of the great vessels
Tetrology of Fallot
Tricuspid Atresia
Total anomalous Pulmonary Venous Return
Pulmonary Atresia

Front 43

What is the MC congenital heart defect?

Back 43

VSD

Front 44

What other congenital illnesses predispose a pt to VSD?

Back 44

Apert's syndrome
Down Syndrome
fetal alcohol syndrome
cri-du-chat
Trisomies 13 and 18

Front 45

What heart sound is frequently heard in an infant with VSD?

Back 45

harsh holosystolic murmur best heard at the lower left sternal border

Front 46

What heart sound is heard in an infant with ASD?

Back 46

fixed widely split S2

Front 47

What other congenital illnesses predispose a child to ASD?

Back 47

Holt-Oram syndrome (absent radii, ASD, first-degree heart block)
Fetal alcohol syndrome
Down syndrome

Front 48

What are the risk factors for PDA?

Back 48

maternal first-trimester rubella infection
prematurity
female gender

Front 49

What are the physical exam findings of a child with PDA?

Back 49

wide pulse pressure, machine-like murmur best heard at the second left intercostal space on sternal border, loud S2, bounding peripheral pulses

Front 50

What is the treatment for PDA?

Back 50

Indomethacin or surgery if medication fails

Front 51

More than 2/3 of patients with coarctation of the aorta also have what anomaly?

Back 51

bicuspid aortic valve

Front 52

What is the typical clinical sign of a child with coarctation of the aorta?

Back 52

asymptomatic hypertension; systolic BP higher in the upper extremities than the lower

Front 53

What may be seen on the CXR of a child with coractation of the aorta?

Back 53

rib notching (2/2 collateral circulation through intercostal arteries)

Front 54

If severe coarctation presents in early infancy, what is the next step in management?

Back 54

PGE1 to keep ductus arteriosus open, followed by surgery

Front 55

What are the risk factors for Transposition of the Great Vessels?

Back 55

Diabetic mothers
DiGeorge syndrome

Front 56

What are the 6 signs of DiGeorge Syndrome?

Back 56

CATCH 22
Cardiac abnormalities
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia
22q11 deletion

Front 57

What is the MC cyanotic congenital heart lesion in newborns?

Back 57

Transposition of the Great Vessels

Front 58

Tran of the great vessels is incompatible with life unless what other congenital heart defect is present?

Back 58

PDA or septal defect

Front 59

What is the MC cyanotic congenital heart disease in children (vs newborns)?

Back 59

Tetrology of Fallot

Front 60

What are the 4 signs of Tetrology of Fallot?

Back 60

PROVe
Pulmonary stenosis
RVH
Overriding aorta
VSD

Front 61

What is the definitive treatment for both Tet and Trans of the Great Vessels?

Back 61

surgical correction

Front 62

For the following developmental milestones, state the age at which these normally occur:
Gross: lifts head/chest when prone
Fine: tracks past midline
Language: alters to sound; coos
Social: recognizes parent; social smile

Back 62

2 months

Front 63

For the following developmental milestones, state the age at which these normally occur:
Gross: rolls front to back; back to front
Fine: grasps rattle
Language: orients to voice; consonant sounds; razzes
Social: laughs; enjoys looking around

Back 63

4-5 months

Front 64

For the following developmental milestones, state the age at which these normally occur:
Gross: sits unassisted
Fine: transfers objects; raking grasp
Language: babbles
Social: stranger anxiety

Back 64

6 months

Front 65

For the following developmental milestones, state the age at which these normally occur:
Gross: crawls; pulls to stand
Fine: uses three-finger pincer
Language: mama/dada nonspecific
Social: waves bye-bye; plays pat-a-cake

Back 65

9-10 months

Front 66

For the following developmental milestones, state the age at which these normally occur:
Gross: Cruises; walks alone
Fine: uses two-finger grasp
Language: mama/dada specific
Social: imitates actions

Back 66

12 months

Front 67

For the following developmental milestones, state the age at which these normally occur:
Gross: w follows two-step commands; walks backward
Fine: uses cup
Language: 4-6 words
Social: temper tantrums

Back 67

15 months

Front 68

For the following developmental milestones, state the age at which these normally occur:
Gross: runs; kicks a ball
Fine: 2-4 cube tower
Language: names common objects
Social: copies parents in tasks

Back 68

18 months

Front 69

For the following developmental milestones, state the age at which these normally occur:
Gross: walks up/down steps with help; jumps
Fine: 6 cube tower
Language: 2-word phrases
Social: follows two-step commands; removes clothes

Back 69

2 years

Front 70

For the following developmental milestones, state the age at which these normally occur:
Gross: rides trike; climbs stairs with alternating feet
Fine: copies a circle; uses utensils
Language: 3-word sentences
Social: brushes teeth with help; washes/dries hands

Back 70

3 years

Front 71

For the following developmental milestones, state the age at which these normally occur:
Gross: hops
Fine: copies a square
Language: knows colors and some numbers
Social: cooperative play; plays board games

Back 71

4 years

Front 72

For the following developmental milestones, state the age at which these normally occur:
Gross: skips; walks backwards for long distances
Fine: ties shoelaces; knows left and right; prints letters
Language: 5-word sentences
Social: domestic role playing; plays dress-up

Back 72

5 years

Front 73

At what age range do breast buds appear in girls?

Back 73

8-13 years

Front 74

At what age range does testicular enlargement begin?

Back 74

9-11 years

Front 75

For the following characteristics, state the disease and genetic abnormality:
-MCC of mental retardation and MC chromosomal d/o
-MR, flat facial profile, simian crease, epicanthal folds
-associated with duodenal atresia, Hirshprung's dz, congenital heart disease
-increased risk for ALL

Back 75

Down Syndrome
Trisomy 21 or translocation

Front 76

For the following characteristics, state the disease and genetic abnormality:
-severe MR
-rocker-bottom feet, low set ears, micrognathia, clenched hands
-may have horseshoe kidneys
-death within one year of birth

Back 76

Edwards' syndrome
Trisomy 18

Front 77

For the following characteristics, state the disease and genetic abnormality:
-severe MR, microphthalmia, microcephaly, cleft lip/palate, polydactyly
-Associated with congential heart disease
-death usually within one year of birth

Back 77

Patau's syndrome
Trisomy 13

Front 78

For the following characteristics, state the disease and genetic abnormality:
-Presence of a Barr body
-one of the MCC of hypogonadism in males
-testicular atrophy, eunuchoid body shape; tall, long extremities; gynecomastia; female hair distribution

Back 78

Klinefelter's syndrome
45,XXY

Front 79

For the following characteristics, state the disease and genetic abnormality:
-MCC of primary amenorrhea
-No Barr body
-short stature, ovarian dysgenesis, webbing of the neck, coarctation of the aorta

Back 79

45, XO

Front 80

For the following characteristics, state the disease and genetic abnormality:
-increased frequency among inmates in prison
-phenotypically normal; very tall with severe acne
-may have antisocial behavior (1-2%)

Back 80

Double Y males
47 XYY

Front 81

For the following characteristics, state the disease and genetic abnormality:
-screened for at birth
-tyrosine becomes essential and phenylalanine builds up excess ketones
-MR, fair skin, eczema, musty urine odor
-increased risk of heart disease

Back 81

PKU
-decrease in phenylalanine hydroxylase or decreased tetrahydrobiopterin cofactor

Front 82

For the following characteristics, state the disease and genetic abnormality:
-second MCC of genetic MR
-macro-orchidism; long face with large jaw; large everted ears; autism
-triplet repeat disorder that may show genetic anticipation

Back 82

Fragile X syndrome
-X-linked defect affecting the methylation and expression of the FMR1 gene

Front 83

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Fabry's Dz

Back 83

-deficiency of a-galactosidase A ->accumulation of ceramide trihexoside in the heart, brain and kidneys.
-renal failure, increased risk of stroke, MI
-X-linked

Front 84

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Krabbe's dz

Back 84

-Galactosylceramidase deficiency -> no galactosylceramide and galactoside-->accumulation of galactocerebroside int he brain
-optic atrophy, spasticity, early death
-AR

Front 85

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Gaucher's dz

Back 85

-deficiency of galactocerebrosidase -> accumulation of galactoceribroside in the brain, liver, spleen and bone marrow; "crinkled paper" of enlarged cytoplasm
-hepatosplenomegaly, anemia, thrombocytopenia
-AR

Front 86

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Niemann-Pick dz

Back 86

-sphingomyelinase deficiency -> build up of sphingomyelin cholesterol in reticuloendothelial and parenchymal cells
-pts with Type A die by age 3
-AR

Front 87

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Tay-Sachs dz

Back 87

no hexosaminidase -> GM2 ganglioside accumulation
-weakness begins at 3-6 months of age, development slows and regresses, exaggerated startle response, death by age three, cherry red spot on macula
-carrier in 1 in 30 Ashkenazi Jews

Front 88

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Metachromatic leukodystrophy

Back 88

Deficiency of arylsulfatase A -> accumulation of sulfatide in brain, kidney, liver and peripheral nerves
-AR

Front 89

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Hurler's syndrome

Back 89

deficiency of a-L-iduronidase
-corneal clouding and MR
-AR

Front 90

For the following Lysosomal Storage Disease state the etiology, clinical findings and mode of inheritance:

Hunter's syndrome

Back 90

Deficiency in iduronate sulfatase
-mild Hurler's with no corneal clouding, mild MR
-X linked

Front 91

What is the most common severe genetic disease in the US and what causes it?

Back 91

CF
caused by mutations in the CFTR gene (chloride channel) on Chr 7
widespread exocrine gland dysfunction

Front 92

What pulmonary infections are most common in pts with CF?

Back 92

Pseudomonas and S. aureus

Front 93

15% of infants with CF present with what illness?

Back 93

Meconium illeus

Front 94

What is the test for diagnosis of CF?

Back 94

Sweat chloride test > 60 mEq/L in pts < 20 yo
> 80 mEq/L in adults

Front 95

What is the MCC of bowel obstruction in the first two years of life?

Back 95

Intussusception (telescoping of bowel into an adjacent segment, usually proximal to the IC valve)

Front 96

What are the signs/symptoms of intussusception?

Back 96

-colicky abdominal pain in an otherwise healthy child
-flexed knees and vomiting
-blood per rectum; blood mucus in stools ("currant jelly")
-palpable sausage-shaped RUQ mass

Front 97

What is the treatment for intussusception?

Back 97

-correct volume/electrolyte abnormalities
-abdominal plain films/US
-air-contrast barium enema is diagnostic and often curative
-surgical resection of gangrenous bowel

Front 98

What is pyloric stenosis?

Back 98

hypertrophy of the pyloric sphincter leading to gastric outlet obstruction

Front 99

What are the clinical signs of pyloric stenosis?

Back 99

NB emesis beginning around 3 weeks of age; projectile emesis after almost every feeding; palpable olive-shaped, NT epigastric mass on PE

Front 100

What diagnostic tests are used to find pyloric stenosis?

Back 100

Ab U/S showing hypertrophic pyloris is diagnostic

Barium studies will show pyloric narrowing (string sign) or a pyloric beak

Front 101

What is the treatment for pyloric stenosis?

Back 101

surgical correction with pyloromyotomy

Front 102

What ist he MC congenital abnormality of the small intestine?

Back 102

Meckel's Diverticulum

Front 103

What is the "Rule of 2's" in regards to Meckel's Diverticulum?

Back 103

1. Most common in children < 2 yo
2. 2x as common in males
3. 2 types of tissue (pancreatic and gastric)
4. 2 inches long
5. within 2 ft of the IC valve
6. 2% of the population

Front 104

What is the MC clinical presentation of Meckel's Diverticulum?

Back 104

typically asymptomatic and found incidentally; can present with painless rectal bleeding

Front 105

Abdominal pain in a child with Meckel's diverticulum suggests what?

Back 105

complications such as diverticulitis, volvulus, and intussusception

Front 106

What is the diagnostic tool for Meckel's?

Back 106

Meckel scintigraphy

Front 107

What is the treatment for Meckel's?

Back 107

excision of the diverticulum together with the adjacent intestinal segment

Front 108

What is Hirschprung's Disease?

Back 108

Congenital lack of ganglion cells in the distal colon leading to uncoordinated peristalsis and decreased motility

Front 109

What are the risk factors for Hirschprung's?

Back 109

male gender
Down syndrome
Waardenburg's syndrome
MEN, Type 2

Front 110

What is the clinical presentation of Hirschprung's?

Back 110

Delayed passage of meconium in newborns
Bilious vomiting
FTT
can present as chronic constipation in older children with less severe dz
explosive discharge of stool following rectal exam

Front 111

What is the diagnostic test for Hirschprung's?

Back 111

plain films will reveal proximally distended loops of bowel followed by constricted, obstructed aganglionic segment

Front 112

What is the treatment for Hirschprung's?

Back 112

surgical excision of the affected bowel segment to prevent MEGACOLON

Front 113

What is malrotation with volvulus?

Back 113

congenital malrotation of the midgut resulting in abnl positioning of the small bowel (cecum in the R hypochondrium) and formation of fibrous bands --> obstruction and constriction of blood flow

Front 114

What are the clinical signs of malrotation with volvulus?

Back 114

often presents in newborn period with bilbious emesis, abdominal tenderness and distension

Front 115

What is the diagnostic study of choice for volvulus?

Back 115

Upper GI, if patient is stable

Front 116

What is the treatment for volvulus?

Back 116

1. NG tube to decompress the intestine
2. IVF hydration
3. surgical repair

Front 117

What are the general signs and treatment of a B-cell deficiency?

Back 117

Most common (50%)
Present after 6 months of age
Recurrent sinopulmonary, GI, UTI with encapsulated organisms (H. flu, Strep pneumo, Nesseria men)
Treat with IVIG

Front 118

What are the general signs of a T-cell deficiency?

Back 118

Present at 1-3 months
Opportunistic and low-grade fungal, viral and intracellular bacterial infections (e.g., mycobacteria)
May also see secondary B-cell def.

Front 119

What are the general signs of a phagocyte deficiency?

Back 119

Mucous membrane infections
Abscesses
Poor wound healing
Infections with catalase + organisms (S. aureus), fungi, and gram (- )enteric organisms

Front 120

For the following B-cell deficiency give a description, infection risk/type and diagnosis/treatment:

X-linked agammaglobulinemia (Bruton's)

Back 120

-Boys only, B-cells only
-life-threatening; encapsulated Pseudomonas, S. pneumo, Haemophilus infections
-Quant Ig levels; may have absent tonsils; treat with prophylactic antibx and IVIG

Front 121

For the following B-cell deficiency give a description, infection risk/type and diagnosis/treatment:

Common variable immunodeficiency

Back 121

-Ig levels in the 20's and 30's; usually combined B and T-cell
-increased pyogenic URI/LRI; increased risk of lymphoma and autoimmune dz
-quant Ig; tx=IVIG

Front 122

For the following B-cell deficiency give a description, infection risk/type and diagnosis/treatment:

IgA deficiency

Back 122

-Mild; most common
-usually asymptomatic; recurrent infections; may have anaphylactic transfusion reaciton
-Quant IgA; treat infections

Front 123

For the following T-cell deficiency give state a description, infection risk/type and diagnosis/treatment:

Thymic Aplasia (DiGeorge syndrome)

Back 123

-CATCH22: cardiac abnormalities, abnormal facies, thymic aplasia, left palate, hypocalcemia, 22q11 deletion
-highly increased infections with fungi and P. jiroveci pneumo
-absolute lymphocyte count, delayed hypersensitivity skin testing
-treat with bone marrow transplant and IVIG; PCP prophylaxis; possible thymus transpant

Front 124

For the following combined immunodeficiency give state a description, infection risk/type and diagnosis/treatment:

Ataxia-telangiectasia

Back 124

-oculocutaneous telangiectasias and progress cerebellar ataxia. Defect in DNA repair
-increased incidence of non-hodgkin's lymphoma, leukemia and gastric carcinoma
-No specific treatment; may require IVIG depending on severity of hte Ig deficiency

Front 125

For the following combined immunodeficiency give state a description, infection risk/type and diagnosis/treatment:

Severe Combined Immunodeficiency (SCID)

Back 125

-Severe lack of B and T cells
-severe, frequent bacterial infections, chronic candidiasis, and opportunistic infections
-Treat with bone marrow transplant or stem cell transplant and IVIG. NEEDS PCP PROHYLAXIS

Front 126

For the following combined immunodeficiency give state a description, infection risk/type and diagnosis/treatment:

Wiskott-Aldrich Syndrome

Back 126

-X-linked disorder with less-severe B- and T-cell dysfunction; patients have eczema, increased IgE/IgA and decreased IgM and thrombocytopenia; classic presentation involves bleeding, eczema and recurrent otitis media
-increased risk of atopic disorders and encapsulated organisms
-supportive treatment IVIG antibx

Front 127

For the following phagocytic deficiency give state a description, infection risk/type and diagnosis/treatment:

Chronic Granulomatous Disease

Back 127

-X-linked or AR with deficient superoxide production by PMNs and macrophages; anemia, lymphadenopathy, hypergammaglobulinemia
-chronic skin, pulmonary, GI and UTIs
-Nitroblue tetrazolium test is diagnostic for CGD.

Front 128

For the following phagocytic immunodeficiency give state a description, infection risk/type and diagnosis/treatment:

Leukocyte adhesion deficiency

Back 128

-defect in chemotaxis of leukacytes
-recurrent skin, mucosal and pulmonary infections. Delayed separation of the umbilical cord
-no pus with minimal inflammation in wounds; high WBCs in blood
-Bone marrow transplant is curative

Front 129

For the following phagocytic immunodeficiency give state a description, infection risk/type and diagnosis/treatment:

Chediak-Higashi syndrome

Back 129

-AR defect in neutrophil chemotaxis; oculocutaneous albinism, neuropathy and neutropenia
-increased incidence of overwhelming S. pyogenes, S. aureus and Pseudomonas
-Bone marrow transplant

Front 130

For the following complement immunodeficiency give state a description, infection risk/type and diagnosis/treatment:

C1 esterase deficiency

Back 130

-AD with recurrent episodes of angioedemalasting 2-72 hours and provoked by stress or trauma
-can lead to life-threatening airway edema
-total hemolytic complement (CH50) to assess quantity and function; FFP prior to surgery

Front 131

For the following complement immunodeficiency give state a description, infection risk/type and diagnosis/treatment:

terminal complement deficiency (C5-C9)

Back 131

-inability to form the membrane attack complex
-recurrent meningococcal or gonococcal infections; rarely lupus or glomerulonephritis
-Meningococcal vaccine and antibx

Front 132

Complement deficiencies have what presentation in children?

Back 132

asplenia or splenic dysfunction; recurrent bacterial infections by encapsulated organisms

Front 133

What are the symptoms of Kawasaki dz?

Back 133

CRASH and BURN
conjunctivitis
rash
adenopathy
strawberry tongue
hands and feet (swollen)
Burn (fever > 40 for >/= 5 days)

If the patient has had a high fever for greater than 5 days, mark Kawasaki and move on!

Front 134

Untreated children with Kawasaki are at risk for what sequelae?

Back 134

aneurysmal expansion and MI

Front 135

What is the treatment for Kawasaki Dz?

Back 135

1. High dose aspirin and IVIG
2. Low dose aspirin for 6 weeks; may need anticoags
3. Corticosteroids in IVIG refractory cases - not routine
4. Consult with pediatric cardiologist

Front 136

What is the MC pathogen causing Croup?

Back 136

Parainfluenza virus

Front 137

What is the MC pathogen causing epiglottitis?

Back 137

H. flu

Front 138

What is the MC pathogen causing tracheitis?

Back 138

S. aureus

Front 139

What is the MC pathogen causing retropharyngeal abscess?

Back 139

GAS is most common followed by S. aureus and Bacteroides

Front 140

What is the MC pathogen causing Peritonsillar abscess?

Back 140

GAS most common; folloed by S. aureus, S. pneumo and anaerobes

Front 141

For the following disease state the virus, characteristics and complications:
Erythema Infectiosum (fifth disease)

Back 141

Virus: Parvo B19
Characteristics: slapped cheek; can start on arms and spread to trunk and entire body; worse with fever and sun
Complications: arthritis, hemolytic anemia; congenital infection is associated with fetal hydrops and death.

Front 142

For the following disease state the virus, characteristics and complications:
Measles

Back 142

Virus: Paramyxovirus
Characteristics: Prodrome: cough, coryza, conjunctivitis; KOPLIK SPOTS (red spots with central gray specks) appear on buccal mucosa after 1-2 days; Rash is maculopapular erythematous that spreads from head toward feet.
Complications: Otitis media, pneumo, laryngeotracheitis

Front 143

For the following disease state the virus, characteristics and complications:

Rubella

Back 143

Virus: Rubella virus
Characteristics: erythematous tender maculopapular rash starts on face and spreads distally. Only have a low grade fever, so not appear ill (compared to measles)
Complications: encephalitis, thrombocytopenia, congenital infection = congenital abnormalities

Front 144

For the following disease state the virus, characteristics and complications:

roseola infantum

Back 144

Virus: HHV-6
Characteristics: aucte onset of high fever (>40) with no other sx for 3-4 days; a maculopapular rash appears with fever on trunk and spreads to face and extremetiies; lasts less than 24 hours
Complications: febrile seizures

Front 145

For the following disease state the virus, characteristics and complications:

Varicella

Back 145

Virus: VZV
Characteristics: mild fever, anorexia, malaise precede the rash by 24 hrs; generalized, pruritic, teardrop vesicular periphery, lesions at different stages of healing
Complications: progresive varicella with meningoencephalitis and hepatitis in immunocomp people.

Front 146

For the following disease state the virus, characteristics and complications:

Varicella zoster

Back 146

Virus: VZV
Characteristics: pain along a sensory nerve; teardrop vesiclular rash in a dermatomal distribution
Complications: encephalopathy, aseptic meningitis, TTP, G-B, cellulitis, arthritis

Front 147

For the following disease state the virus, characteristics and complications:

Hand-foot-and-mouth disease

Back 147

Virus: Coxsackie A
Characteristics: fever, anorexia, oral pain; oral ulcers, maculopapular vesicular rash on hands and feet, sometimes buttocks
Complications: none; self-limitied

Front 148

What does the APGAR score measure?

Back 148

Appearance (color)
Pulse
Grimace (reflex ability)
Activity (muscle tone)
Respiratory effort

Front 149

What are some of the causes of conjugated hyperbilirubinemia in a neonate?

Back 149

ALWAYS PATHOLOGIC
-extrahepatic cholestasis (biliary atresia, cholodochal cysts)
-intrahepatic cholestasis (neonatal hepatitis, inborn errors of metabolism)
-ToRCHeS infections

Front 150

What are some of the causes of unconjugates hyperbili in a neonate?

Back 150

-physiologic jaundice
-hemolysis
-breast milk jaundice
- increased enterohepatic circulation (GI obstruction), disorders of bilirubin metabolism, sepsis

Front 151

What is the MC presentation of kernicterus?

Back 151

lethargy, poor feeding, high-pitched cry, hypertonicity and seizures

Front 152

What is the MCC of respiratory failure in preterm infants?

Back 152

RDS

Front 153

What is the pathophys of RDS?

Back 153

surfactant deficiency -> poor lung compliance and atelectasis

Front 154

What are risk factors for RDS?

Back 154

maternal DM, male gender, second born of twins

Front 155

What will you see on the CXR of a neonate with RDS?

Back 155

ground glass appearance and air bronchograms

Front 156

What is the treatment for RDS in neonates?

Back 156

CPAP or intubation and mechanical ventilation; artificial surfactant to decrease mortality

Front 157

What is the treatment to prevent RDS in mother's?

Back 157

corticosteroids

Front 158

What measure is used to evaluate fetal lung maturity?

Back 158

lecithin-to-sphingmyelin ratio and phosphatidylglycerol

Front 159

What is the MC movement disorder in children?

Back 159

Cerebral palsy

Front 160

What is thought to be the cause of cerebral palsy?

Back 160

perinatal neurologic insult

Front 161

What type of gait is common in children with cerebral palsy?

Back 161

tow walking or scissor gait

Front 162

What is used to treat the spasticity in cerebral palsy?

Back 162

diazepam, dantrolene or baclofen

Front 163

What is the MC childhood malignancy?

Back 163

Acute lymphocytic leukemia (ALL)

Front 164

What is a chloroma?

Back 164

greenish soft-tissue tumor on the skin or spinal cord; seen in association with AML

Front 165

What is a neuroblastoma?

Back 165

embryonal tumor of neural crest origin