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163 Cards in this Set

  • Front
  • Back
heroin in utero
IUGR, macrocephaly, SIDS, neonatal abstinence syndrome (irritability, high pitched cry, poor sleeping, tremors, seizures, sweating, sneezing, tachypnea, poor feeding, bomiting, diarrhea)
valproic acid in utero
neural tube defects, cardiac anomalies, dysmorphic facies, cleft lip, narrow bifrotnal diameter, midface hypoplasia, broad and depressed nasal bridge and long philtrum
phenytoin in utero
nail and digit hypoplasia, dysmorphic facies and MR, IUGR, hirustism, dev delay, cleft lip and palate
alcohol in utero
growth def, long smooth philtrum, small palpebral fissures and thin upper lip, MR
cocaine in utero
jitteriness, excessive sucking, hyperactive moro, behavior, attention and intelligence affected long term
myocarditis
dilated heart, increased end diastolic volume, CHF, fever, anorexia, lethary, irritability, resp dsieastress, holosystolic murmur due to dilation, functional mitral regurg, hepatomegaly, faituge, tachypnea, tachy, decreased perfusion, EKG low volt QRS and sinus tachy
kawasaki
med vasculitits,18-24m, fever >5d, extremity changes (erythema, edema, desquamation), conjunctivitits, rash on trunk, cervical lymphadenopathy, oral changes (erythema, fissured lips, strawberry tongue), increased CRP, ESP, ALT, thrombocytosis, leukocytosis, urethritis, orchitis, arthritis, hepatitis, induration of previous bcg site
which rash spreads from extremities inward to trunk?
rocky mountatin spotted fever
tx of kawasaki
IVIG and aspirin (high dose)
serious complication of kawasaki
coronary artery aneurysms, orchitis, hepatitis, myocarditis
intraventricular hemorrhage in infant
mainly due to prematurity, Low birth weight, cyanosis, pallor, focal neuro signs, seizures, hypotension, apnea, bradycardia
dermatomyositis
violaceous poikiloderma, periorbital edema, shawl sign, gottrons sign/ papules, internal malignancies, pul fibrosis
inflamm diseases assoc with aortic aneurysm
behcets, takayasu, ankylosing spondylitis, RA, psoriatic arthritis, relapsing polychronditis, and reiters
measles
leukopenia and thrombocytopenia (helped by vit A), high grade fever, rash, brick red maculopapular rash first on face
obstructive/ noncommunicating hydrocephalus
dandy walker- expanded 4th ventricle; chiari- protrusion of structures of posterior fossa through foramen magnum
causes of neonatal hyperbili (conjugated/direct)
biliary atresia, sepsis, endocrinopathy, nutritional hepatotox (galactosemia, tyrosinemia)
when to give varicella vaccine if exposed?
active immuzation first 3-5d after
when to give VZIG
immunocompromised, pregnant women, within 72-96 hrs
beckwith wiedemann
neonatal hypoglycemia, macroglossia, large size, visceromegaly, omphalocele and characteristic earlobe crease, prominent eyes, occiput, hyperplasia of pancreas, increased risk for wilms, hepatoblastoma, gonadoblastoma
prader willi
narrow bifrontal diameter, diamond shaped eyes, small downturned mouth, hypothalamic dysfunction, short statue, obesity, hypotonia, hyperphagia
medulloblastoma
usually in cerebellar vermis, mets through CSF tract, radiosensitive
eczema herpeticum
form of primary HSV infection, assoc with atopic dermatitis, need prompt acyclovir, umbilicated vesicles over area of healing atopic dermatitis
SCIDs
recurrent sinopul infections, oral candidiasis, persistent diarrhea, opportunistic infections, viral infections, absent lymph nodes, tonsils, lymphopenia, absent thyrmic shadow, abnl T and B and NK cell
common varible immunodef (acquired hypogammaglobuliemia)
decreased IgG,IgA,IgM, and IgE, no absence or decrease in B cells
bruton's agammaglobulinemia (X linked agammaglobulinemia)
male asympt 6-9m, recurrent pyogenic infections, decreased IgG,IgA,IgM,IgE, absent or markedly decreased Bcells
wiskott aldrich
XR, ezcema, thrombocytopenia, recurrent infections with encapsulated germs, petechiae, bruises, bleeding from circumscision, bloody stools, low IgM, high IgA IgE,
chronic granulomatous disease
defect of phagocytic cells, dysfunction of NADPH oxidase, catalase + infections, lymphadenitis, abscesses of skin and vicera, nitro blue tetrazolium test,unexplained aspergillus, serrate marcescens and burkholderia cepacia, hepatomegaly, splenomegaly, anemia of chronic disease, chronic diarrhea, short, gingivitis, dermatitis, decreased cytochrome C, tx bone marrow txplant
transposition of great vessels
diabetic mothers, no murmur, single loud S2, prostaglandin E1 can be given IV to stabilize
tetralogy of fallot
S2 and systolic murmur from pul stenosis, cyanosis, present from birth to few yrs
apathy, weakness, hypotonia, large tongue, sluggish movement, and bloating ,umbilical hernia, jaundice, difficulty breathing ,noisy respiration, hypothermia, refractory macrocytic anemia
congenital hypothyroidism, from dysgenesis, hypoplasia or ectopic, errors of synthesis, or blocking antibodies, tx levothyroxine
what are the two causes of floppy baby
botulism or werdnig hoffman
rubella
maculopapular rash, posterior cervical and psoterior auricular lymphadenopathies and polyarthralgia
MENII
medullar ca of thyroid, hyperparathryoidism, pheo, serum calcitonin elevated in pts with medullary thyroid ca, hyperparathyroidism
SC patient with pain in hip, no fevers
avascular necrosis (other non traumatic causes: chronic corticosteroid therapy, SLE, alcoholism, gaucher's disease), supplied by ascending arteries and foveal artery (inside ligamentum teres)
schoolteacher, MCP,PIP, wrist and ankle jt pain, diarrhea, fatigue
parovirus infection, test for anti B19 IgM
acid base status in pyloric stenosis
hypok alkalosis
congenital deafness, QT prolongation
jervell lange nielson, tx BB +/- DDD pacemaker
risks of otitis media
6-36m, low SE status, use of formula, caretaker smoking, reps allergy, craniofacial anomalies, chronic middle ear effusion
bronchiolitis tx for high risk
ribavirin
previous seizure with DTaP, which part caused it
pertussis, give DT
craniopharyngioma
from epi remnant of rathke's pouch, calcifications and cystic, growth failure, headaches, nests of squamous cells in loose stroma, DI
HUS
hemolytic anemia, acute renal failure, thrombocytopenia
HBV tests
HBsAg unresolved disease, HBeAg infectious, HBsAb ONLY if immune (NEVER in chronic disease)
choanal atresia
better with crying, can't pass NG through nose
kernicteris
unconjugated bili, seizures flaccidity, poor feeding, opisthotonos, apnea
physiologic jaundice
term <12 peaks 2-3d, over in 2wks; premie <15 peaks 3-5d over in 3wks
tetrology of fallot
VSD, overriding aorta, PS, RVH
changes as infant is born
decreased pul vascular resistance, increased left sided heart pressure, closure of foramen oval, increased O2 shuts off prostaglandin --> close ductus arterioles
downs heart disease
ASD(fixed split S2, need abx prophylaxis), endocardial cushion defect (loud P2, L-->R shunt, pul HTN)
stroke in child?
sickle cell
papillary necrosis
analgesic overuse, DM, infection, UT obstruction, hemoglobinopathies, cirrhosis, CHF, shock, hemophilia
VSD tx
ACEI, diuretics
infant with reps farilure, fever, lethargy, poor feeding, hypotonia, seizures, bulging fontanel
meningitis, in newborn, most likely GBS
HSV in newborn
temporal lobes, focal neurologic deficits
listeria meningitis in newborn
also abscesses and pneumonia
before intubating pt with croup...
try epi
recent viral infection or trauma, limp and/or uni hip or knee pain, flexed, slightly abd and externally rotated, limitation of motion, pain with palpation or passive motion
transient synovitis tx: NSAIDs and rest, can see slight increased WBC, ESR
septic arthritis from transient synovitis
WBC >12, temp>39/102, ESR>40, refusal to bear weight
is breath holding spells assoc with anything (age 6-18m)
iron def anemia, no long term sequele,
PKU
def phenylalanine hydroxylase, increased serum phenylalanine and urinary excretion of phenyl acetic acid, irreversible MR, seizure d/o, psychotic episodes, blond hair, fair skin, blue eyes, musty or mousy odor, seborrheic lesions, eczematous rash, vomiting, failure to thrive, dev delay, met acidosis, phenyalanine >20, nl to low nl tyrosine, increased urinary levels of phenylalanine metabolites (phenylpyruvic and o hydroxyphenylacetic acids), nl tetrahydrobiopterin level
transient hyperphenylalaninemia
transient tyrosinemia of new bone- def oxidize tyrosine
tyrosinemia
def fumarylacetoacetate hydrolase, sym 2 wks, fever, irriability, vomiting ,hemorrhage, hepatomegaly, jaundice, elevated serum transaminases, epi of acute peripheral neuropathy, fanconi like synd, hypoglycemia, smell like boiled cabbage
alkaptonuria
def homogentisic acid oxidase, dark/ black urine, ochronosis, arthritis, homogentisic acid in urine
chediak higashi
decreased granulation, chemotaxis and granulopoiesis, mild coagulopathy, peripehral and cranial neuropathy, HSM, pancytopenia, partial oculocutaneous albinism, frequent bacterial infections, progressie lymphoprolif synd,giant lysosomes tx: TMP SMX and ascorbic acid
leukocyte adhesion defect
defective tethering, adhesion, targeting of myeloid leukocytes to sites of microbial invasion, neutrophilia w/o polymorphs, delayed separation of umbilical cord, recurrent bacterial infections, necrotic skin lesions, severe gingivitis, periodontitis, alveolar bone loss --> early loss of deciduous and permanent teeth
atlantoaxial instability
10-15% down synd, excessive laxity in posterior transverse ligament, increased mobility btw C1 and C2, compression of spinal cord, behavioral changes, torticollis, urinary incontinence, vertebrobasilar sympt, dizziness, vertigo, diplopia, leg spasticity, hyperreflexia, positive babinski, clonus
metatarsus adductus
type I: overcorrects passively and actively- no tx; type II: feet that correct to neural tx orthosis or corrective shoes; type III rigid feel do not correct- serial casts
febrile seizures that need to be worked up
focal lasts >15min, or multiple with total >30min
neonatal tetanus
muscle spasms, hypertonicity, poor suckling, fatigue, opisthotonus, death from apnea and septicemia
ADHD
6m, persistent since age 7, in more than one setting, poor conc, distractibility, elevated motor activity, impulsivity
signs of hydrocephalus
poor feeding, irritability, committing ,decreased activity, scalp veins may appear prominent with shiny, tight skin overlying the vessels, cranial sutures may be widely spaced, CT brain, tx: shunt
tx of DDH
pavlik harness <6m, reduction and spica cast 6m-2yrs, open reduction after 2yo, no correction after 8yo
when to prophylactic pin contralateral side in slipped apical femoral epiphysis
hypothyroidism
x linked gammaglobulinemia/ bruton's agammaglobulinemia
tx: IVIG; recurrent bacterial infections early in life
cause of exudate
increased capillary permeability
what causes effusion in liver failure
transudate- decreased plasma oncotic pressure
what causes transudative effusion in CHF
elevated hydrostatic pressure
cretinism
poor feeding, lethargy, large fontanelles, thick tongue, constipation, umbilical hernia, poor growth, hypotonicity, dry skin, hypothermia, jaundice, decreased T4, increased TSH, prolonged jaundice
difference between pseudo precocious puberty and true?
true causes testicular or ovarian enlargement
diamond blackfan syndome/ congential hypopalstic anemia
defect ofintrinsic defect of erythroid progenitor cells, macrocytosis, pallor, web neck, cleft lip, shielded chest, triphalangeal thumbs, palse mucous membranes tx:steroids
fanconi's anemia
AR, pancytopenia and macrocytosis, cae au lait, microcephaly, microphthalia, short, horseshoe kidney, absent thumbs
neuroblastoma vs wilms
neuroblastoma crosses midline and have catecholamine metabolites in urine, wilms DOES NOT cross midline and has hematuria
congential syphillis
lesions on plasm and soles, HSM, jaundice, anemia, rhinorhea, metaphyseal dystrophy, periostitis
ricketts
open fontanels, pingpong ball over occiput, enlargement of costochondral junctions, thickening of wrists and ankles, cupping and fraying of distal ends of radius and ulna, horizontal depression along lower border of chest, LOW calcifediol level, increased Alk phos,
chondrodystrophy
irregular concave outlines of distal ends of bone
stridor, diff breathing, sitting up, leaning forward, drooling,
epiglottitis, need to intubate- H flu and s. pyogenes
depression and dexamethasone suppression test
abnl in 50%
newborn hand paralysis and horners
klumpkes
laryngomalacia/ congenital flaccid larynx
hold baby up for 30 mins after feeding, don't feed supine, rolling epiglottis side to side, noisy inspiration, subsides by 18m
AFP
from yolk sac and fetal liver, increased in neural tube defect, multi gestations, wrong gestational age, abd wall defect
stranger anxiety
normal till 3yo
causes of neonatal shock
gastroenteritis, GBS, CAH, CF, gi obstruction, galactomsemia
allergic interstitial nephritis
from many drugs, along with arthralgias and rash, WBC casts and eos in urine, caused by sulfa, penicillin, cephalosporin rifampicin
vaccinations for pre term babies given according to...
chronological age, except no hep b till >2kg
wet the bed at least 2 x/wk for 3 months
behavior mod --> desmopressin --> TCA (imipramine)
overproduction uric acid, behavioral problems, neurologic disability, def of HPRT
nesch nyhan
what tests of get on BPH pt?
U/A and Cr
hip is externally rotated, won't walk on it, extreme pain
septic arthritis -- tx drainage immediately
HUS
e.coli 0157H7, shigella, salmonella, yersinia, campylobacter, preceded by URI, GI bleeding, purpura, htn, microangiopathic hemolytic anemia, acute renal failure, fever, oliguria, thrombocytopenia, increased LDH, indirect bili, retic count
androgen insensitivity
primary amenorrhea, b/l inguinal masses, breast development w/o pubic or axillary hair
hyperca, single lytic lesion in head of humerus in 6yo
langerhans cell histiocytosis, benign and treated conservatively
newborn, cyanotic, left axis deviation
tricuspid atresia
lennox gastaut synd
seizures and MR
most common brain tumor in children
astrocytoma, usually infratentorial
what is in excess with 21 hydroxlase def?
17 alpha hydroxyprogesterone--> hirsutism or virilization
molluscum
in child prob not sexual abuse
tx of rosacea
metronidazole or tetracycline
increased gastric residual first few days of life, abd distention
necrotizing entercolitis
restrictive lung disease
all lung volumes go DOWN except FEV1/FVC
test amniotic fluid for lung maturity
L/S>2 or phosphatidylglycerol present
tx of CF
deoxyribonuclease, pancreatic enzymes
what children get RDS?
premature and diabetic mother
CF
rectal prolapse, esophageal varices, pancreatic insufficency, infertility,
erythema toxicum
red halo surrounding lesions, presence of eosinophils in pustules
tx of get spell
put knee to chest, fluids, morphine, propranolol
staph scalded skin syndrome
perioral, flexures, nikolsky sign is positive, target desmoglein 1, resolve 1-2 wks, cultures of skin are sterile (caused by toxin)
medial longitudinal fasciculus lesion (MS)
left eye nystagmus when looking left and right eye nystagmus when looking right
ALL
children, >25% lymphoblasts in bone marrow, HSM, petechiae, lymphadenopathy
what is meconium aspiration common in?
post mature and SGA infants, flattenign of diaphragm, hyperinflation, coarse streaking and patch opacities over both lung fields
complications of meconium aspiration
pneumothorax, infection, pul HTN, residual lung problems, neuro problems from asphyxia
rubella
posterior cervical lymphadenopathy, patchy erthyema throat and palate, 3 day measles
atypical measles
inactivated vaccine, atypical rash, arthritis, hepatitis, lung involvement, edema of hands and feet
scarlet fever
punctate or finely papular texture, gray white tonsilar exudates, chills, abd pain, etc, tx: pen V or erthyromycin, clinda
diabetic mother... increased risk for...
caudal regression synd, transposition of great vessels, duodenal atresia and small elft colon, anencephaly and neural tube defects
polycythemia in newborn (HCT>65)
from delayed clamping of umbilical cord, find plethora, lethargy, irritability, jitterienss, seizures, resp distress, tachypnea, cyanosis
lead levels and tx
>10 recheck serum lead, if <20 can recheck in 1 mon, if >45 chelation
cystathionine synthase def
elevations of homocysteine and methionine tx: high dose Vit B6 --> restrict methionine with supplementation of cysteine
cholesteatoma
secondary to chronic middle ear infection, continuous drainage despite abx, granulation and skin debris; complications hearing loss, CN palsies, vertigo, brain abscess, meningitis
prophylaxis for malaria
mefloquine
suspect congenital diaphragmic hernia
Orogastric tube placement
best predictor of extent of TCA overdose
QRS interval
infant that drinks a lot of cows milk? dx? tx?
iron def anemia, tx oral iron w/o testing
complications of aortic dissection
stroke, ARF, aortic regurg, cardiac tamponade
what dev of secondary sex characteristic is worrisome for CNS disorder
pubic hair before 8yo (pubarche), okay to have adrenarchy (axillary hair-premature adrenal activation) or thelarche
type 1 gaucher
acid beta glucosidase, fatigue, easy bruise, thrombocytopenia, bone pain, path fractures, erlenmeye fast of femur, wrinkled paper on bone marrow study
patients with RA are increased risk of what other bone d/o
osteopenia, osteoporosis
osteitis fibrosis cystic
brown tumors, increased osteoclast, hyperparathyroidsism causes it
osteomalacia
d/o of bone mineralization from decreased fit D, ca, from parathyroidectomy and rental tubular acidosis
acute renal trxplant rejection
heavy lymphocyte infiltration, vascular involvement with swelling of intima
tx of tet spells
increase systemic vascular resistance--> improved pul blood flow
hyperIgM
recurrent sinopul infections, pneumocystis jiroveci,
bulous myingitis
bullae on tympanic membrane in patients with otitis media, more severe pain, assoc mycoplasma pneumoniae
childhood disintegrative disorder
in men, nl 2 yrs, loss of skills in receptive/ expressive language, social skills, bowel or bladder control, play and motor skills, severely disabled for life
rett syndrome
usually female, hand movements, nl for 6 m, decal head growth, poor coordination, seizure, ataxia, MR, diminished social interactions
c. botulism
2wk-9m with constipation --> hypotonia, weakness, loss of DTR, CN abnl, respiratory difficulty, symptoms from organism
parinaud's synd
paralysis of vertical gaze, assoc with pupillary disturbances, eyelid retraction, can disturb hypothal --> increased beta hog --> increase leydig cell stimlation
milk protein intolerance
can be intolerant to cows milk or breast, stool has RBCs and dos,
acute unilateral lymphadenopathy
usually bacterial, Staph A,
tularemia
francisella tularenis, acute uni cervical lymphadenopathy, headache, fever, malaise, infected animal
peptostreptococcus
periodontal disease, acute uni lymphadenopathy,
child with cerebellar signs
fredreich's ataxia, arnold chiari, dandy walker, tumor, ataxia telangiectasia
prostate cancer
abnl nodules, sympts of BPH, osteoblastic lesions, acid phosphatase increased if broke through capsule
bunch of grapes out of vagina
sarcoma botryoides
unicameral bone cyst
lytic benign, prox humerus, pathologic fx possible
MCC of precocious puberty in boys
adrenal hyperplasia
hyaline membrane disease of newborn
60-80% <28 wks, tachypnea, grunt, retractions, grunt, dusky, fine reticularity of lung parenchyma on xray
painless melena
meckels
fredreich's ataxia
necrosis and degeneration of cardiac muscle fibers leading to myocarditis, cardiac arrhythmias, CHF, wide gait, cervical spinal cord and cerebellar atrophy, high plantar arches, from tocopherol abnl
NF1
cafe au lait, macrocephaly, short stature, feeding difficulties, learning disabilities
risks for RDS in infant
male, maternal diabetes, premature, c/s w/o labor, perinatal asphyxia
obesity hypoventilation syndrome
weight loss, ventilator, O2, progestins
reye's synd
vomiting, aggitation irritation, --> restless, lethargy, stupor; see convulsions, hyperammonium, increased bili, alk phos, PTT, AST,ALT, LD,hypoglycemia, microvesicular steatosis
risks SGA
hypoglycemia, polycythemia, hypoxia, meconium, hypocalcemia, hypothermia