Pediatric Flashcards
most common type of tracheoesophageal fistula
esophageal atresia w/ distal TEF - unable to pass NG tube
things that are not contraindications to vaccination
mild illness and/or low grade fever, current abx therapy, and prematurity
tests to r/o shake baby syndrome
ophthalmologic exam, CT, and MRI
neonate has meconium ileus
cystic fibrosis
bilious emesis w/in hours after the first feeding
duodenal atresia
2 month old baby w/ non-bilious projectile emesis. Dx? Lab abnormality? Tx?
pyloric stenosis; hypocholermic, hypokalemic metabolic alkalosis; surgical correction - pyloromyotomy
most common primary immunodeficiency
selective IgA def
infant w/ high fever and onset of rash as fever breaks
roseola infantum (risk for febrile seizure)
chronic respiratory infections w/ - nitroblue tetrazolium test
chronic gramulomatosis dz
male child w/ eczema, thrombocytopenia (purpura/bleeding), and recurrent infections (otitis media). pts have high levels of IgA/IgE and decreased IgM
wiskott-aldrich syndrome (x-linked recessive only in males)
acute-phase tx of kawasaki dz
high-dose ASA for inflammation and fever and IVIG to prevent coronary artery aneurysms
tx for mild and severe unconjugated hyperbilirubinemia
phototherapy (mild) or exchange transfusions (severe)
sudden onset of mental status changes, emesis, and liver dysfunction after ASA intake
reye syndrome
child has loss of red light reflex (white pupil). Dx? child has increased risk of what cancer?
suspect retinoblastoma. increased risk of osteosarcoma
vaccinations at a 6 month well child visit
HBV, DTaP, Hibb, IPV, PCV, rotavirus
infection of small airways w/ epidemics in winter and spring
RSV bronchiolitis
cause of neonatal RDS? risk factors?
surfactant deficiency. risk factors include prematurity and maternal DM
condition associated w/ red “currant-jelly” stools, colicky abdominal pain, bilious vomiting, and a sausage-shaped mass in the RUQ
intussusception
congenital heart dz that causes secondary HTN
coarctation of the aorta (find decreases femoral pulses)
first line tx for otitis media
amoxicillin x 10 days
most common pathogen causing croup
parainfluenza virus type I
defect in x-linked syndrome w/ mental retardation, gout, self-mutilation, and choreoathetosis
lesch-nyhan syndrome (purine salvage problem w/ HGPRTase def)
newborn girl has continuous machine like murmur
PDA
tx to close PDA
indomethacin
newborn w/ posterior neck mass and swelling of the hands
turner’s syndrome
non-tender abdominal mass associated w/ elevated VMA and HVA
neuroblastoma
fixed, widely split S2
ASD
digeorge syndrome - CATCH 22
CATCH 22 cardiac abnormalities (transposition) abnormal facies thymic aplasia cleft palate hypocalcemia 22q11 deletion
trisomy ? - MR, flat face, upslanted eyes w/ epicanthal folds, simian crease, general hypotonia, atlantoaxial instabiligy, duodenal atresia, hirschsprung’s dz, and congenital heart defects
trisomy 21 (downs syndrome)
trisomy ? - rocker-bottom feet, low set ears, micrognthia (undersized chin), clenched hands (overlapping 4-5 digits)
trisomy 18 (edwards syndrome)
trisomy ? - microphthalmia, microcephaly, polydactyly
trisomy 13 (pataus syndrome)
male pt w/ testicular atrophy, eunuchoid body shape, tall stature, long extremities, gynecomastia, and female hair distribution. dx? tx?
klinefelter’s syndrome (47 XXY). tx w/ testosterone (prevents gynecomastia; improves secondary sexual characteristics)
second most common cause of MR (first is downs) + large jaw, testes and ears + autistic behaviors
fragile x syndrome (x-linked dominant)
cherry-red spot w/ hepatosplenomegaly. Dx? Path?
niemann-pick dz. due to def of sphingomyelinase
cherry-red spot but no hepatosplenomegaly. Dx? Path?
tay-sachs disease. due to absence of hexosaminidase
meckel’s rules of 2
2 yrs or less; 2 x more in males; 2 types of tissue (pancreatic and gastric); 2 in long; 2 feet from ileocecal valve; 2% of population
pneumatosis intestinalis on plain film is pathognomonic for…
NEC in neonates
b-cells make immunoglobulins and are responsible for immunity against?
extracellular bacteria
t-cells are responsible for immunity against?
intracellular bacteria, viruses, and fungi
features of kawasaki dz
fever greater than 104 for 5 days + 4 more criteria: conjunctivitis, rash, adenopathy (unilateral), strawberry tongue, erythema of hands and feet
most common cause of bronchiolitis
RSV
most common cause of croup
parainfluenza
x-ray findings: 1. double-bubble... 2. triple-bubble... 3. dilated loops of bowel... 4. pneumatosis intestinalis... dx for each?
- duodenal atresia (stomach + doudenum)
- jejunal atresia (stomach + duodenum + jejunum)
- hirschsprung dz
- NEC
classic findings in congenital rubella syndrome
classic triad of cataracts, PDA, and sensorineural hearing loss. may also have IUGR and purpura
differentiate neonatal gonococcal vs chlamydia conjunctivitis
gonococcal = copious purulent ocular drainage and eyelid swelling in 2-5 days post birth chlamydia = milder symptoms in 5-14 days post birth
young child w/ increased intracranial pressure (ie. HA, vomiting), bitemporal hemianopsia, and a calcified lesion above the sella. dcx?
craniopharyngioma
tx for pt w/ B-cell def (pts that present after 6 months w/ recurrent sinopulmonary, GI, and urinary tract infections w/ encapsulated organsisms (SHiN - Strep, H. influ, Neisseria)?
IVIG (except for selective IgA def, as can lead to the production of anti-IgA antibodies)
x-linked recessive B-cell def found only in boys. symptoms begin after 6 months when maternal IgG (transferred across placenta) is no longer present. pt may have absent tonsils or other lymphoid tissue
bruton’s congential agammaglobinemia
pt w/ combined B and T-cell def. normal B-cell numbers w/ decreased plasma cells –> usually all Ig levels are low and symptoms usually present later in life (15-30 yo)
common variable immunodef (CVID)
tx for DiGeorge?
treat w/ BM transplant and IVIG for antibody def
pt w/ progressive cerebellar ataxia and oculocutaneous telangiectasias caused by DNA repair defect
ataxia-telangiectasia
severe lack of B and T-cells due to defect in stem cell maturation and decreased adenosine deaminase
severe combined immunodef (SCID)
x-linked or autosomal recessive dz w/ anemia, lymphadenopathy, hypergammaglobulinemia, and + nitrobule tetrazolium test
chronic granulomatous disease (def of superoxide production by PMNs and macrophages)
pt w/ recurrent skin, mucosal, and pulmonary infections. infant w/ delayed separation of the umbilical cord. no pus w/ minimal inflammation in wounds
leukocyte adhesion def (defect in chemotaxis of leukocytes)
autosomal recessive disorder that leads to defect in neutrophil chemotaxis/microtubule polymerization –> partial oculocutaneous albinism, peripheral neuropathy, and neutropenia. may have giant granules in neutrophils. dx?
chediak-higashi syndrome
defect in neutrophil chemotaxis –> FATED: coarse Faces, Abscesses, retained primary Teeth, hyper-igE, Dermatologic (severe eczema)
job’s syndrome
autosomal dominant disorder w/ recurrent episodes of angioedema lasting 2-72 hrs and provoked by stress or trauma
C1 esterase def (hereditary angioedema)
inability to form membrane attack complexes (MAC) due to terminal complement def (C5-C9) leads to recurrent infections of?
neisseria infections
pedi cancer - differentiate wilms tumor vs neuroblastoma
wilms tumor - unilateral mass w/ hematuria that is otherwise asymptomatic, and occurs in older children
neuroblastoma - mass that crosses the mildline, has systemic symptoms, and occurs in younger children
association of anomalies in newborns - VACTERL
vertebral, anal atresia, cardiac, tacheoesophageal fistula, renal, and limb
obese adolescent (11-14 yo) boy has pain in the left anterior thigh for 2 months. typically have painful limp. may have inability to bear weight. limited passive flexion and internal rotation of hip. x-ray w/ frog leg view reveals posterior/inferior displacement of femoral head. dx? tx?
SCFE (separation of proximal femoral epiphysis through the growth plate leading to inferior/posterior displacement of the femoral head to femoral neck). tx is surgery.
4-10 yo boy presents w/ hip pain and/or knee pain. has painless limp, has limited abduction or internal rotation of the hip. dx? tx?
legg-calve-perthes disease (idiopathic AVN of the femoral head). tx is observation, if severe may need to brace
16 yo boy complains of R knee pain. exam shows signficant tenderness and swelling over the tibial tuberosity. otherwise healthy. dx? tx?
osgood-schlatter dz (due to chronic microtrauma to the tibial tuberosity secondary to overuse of the quads). tx is rest, restriction of activities, and/or brace
pt w/ recurrent fx, blue sclera, hearing loss. dx? cause?
osteogenesis imperfecta. caused by a mutation in type 1 collagen (m/c autosomal dominant)
pt w/ leukocoria. dx?
retinoblastoma until proven otherwise. emergent to ophthalmology
pt w/ combo of neurologic (ataxia, dysarthria), skeletal (scoliosis, feet deformities), and cardiac (concentric hypertropic cardiomyopathy). dx?
friedreich ataxia (most common cause of death is cardiomyopathy and respiratory complications)
3 P’s of mccune albright syndrome (defect in G-protein cAMP-kinase function)
- precocious puberty
- pigmentation (cafe au lait spots)
- polyostotic fibrous dysplasia
rheumatic fever is a preventable complication of strep pharyngitis. what is non-preventive complication.?
post-strep glomerulonephritis
rheumatic fever - clinical diagnosis based off of JONES criteria
joints (migratory), heart (carditis), nodules (subQ), erythema marginatum, sydenham chorea
child w/ fever, oral vesicles on the bucca mucosa, tongue, and small, tender cutaneous lesions on the palms and soles. dx? cause? tx?
hand, foot, mouth dz. caused by coxsackie. self-limiting, therefore supportive care
effects of epi given during anaphylactic rxn?
epi = beta2 and alpha 1 receptor agonists –> bronchial smooth muscle relaxation and constriction
young infant w/ upper respiratory symptoms, wheezing/crackles, and respiratory distress. dx?
bronchiolitis most commonly 2/2 RSV
pt w/ rapid-onset edema of the face, acral extremities, genitals, trachea, and abdominal organs w/o urticara. dx? cause?
hereditary angioedema. due to def or dysfunction of C1 inhibitor –> elevated levels of the edema producing factors C2b and bradykinin
pt w/ inspiratory stridor that worsens in the supine position and w/ crying that peaks around 4-8 months. dx? workup to confirm dx? tx?
larynogomalacia. confirm w/ flex laryngoscopy, which shows collapse of the supraglottic structures during inspiration. tx is none, as usually resolves by 18 months
abx of choice for tx and post-exposure prophylaxis of pertussis
macrolide abx
infant w/ lateral traction on neck during delivery –> abduction of army, lateral rotation, and flexion, supination. dx? risk factor? injured nerve roots?
erb palsy w/ increased risk due to fetal macrosomia - injury at C5-C6 roots
infant w/ upward force on army during delivery –> total claw hand deformity. dx? injured nerve roots?
klumpke palsy - injury at C8-T1
neonatal in first few days of life presents w/ irritability, high-pitched cry, poor sleep, tremors, seizures, sweating, sneezing, tachypnea, poor feeding, vomiting, and diarrhea. dx? cause?
neonatal abstinence syndrome caused by infant withdrawal from opiates (morphine, heroin, etc)
contraindications to breast feeding due to mother? due to baby?
mother - active, untreated TB, HIV, and active illicit drug use (hep B and C are not considered contraindications)
baby - galactosemia
ddx of solitary, painful, lytic bone lesions w/o overlying swelling and hyperCa in a child
langerhans cell histiocytosis and neoplasms
pt w/ palpable purpura on the LE, arthralgias, abdominal pain, and renal dz. dx? cause?
HSP. caused by IgA mediated vasculitis
well appearing infant w/ intermittent cyanosis and distress during feeding that is relieved by crying. dx? confirm dx?
choanal atresia. failure to pass a catheter through the nose into the oropharynx is suggestive of the diagnosis and CT shows narrowing at the level of the pterygoid plate in the posterior nasal cavity
maternal imprinting - deletion of paternal copy of chromo 15. dx? features?
prader-willi - features include hyperphagia, obesity, intellectual disability, hypogonadism, and hypotonia
paternal imprinting - deletion of maternal copy of chromo 15. dx? features?
angelman syndrome - features include inappropriate laughter/smiling, seizures, ataxia, severe intellectual disability
pt w/ macrocytic pure red aplasia + several congential anomalies such as short stature, webbed neck, cleft lip, shielded chest, and triphalangeal thumbs. dx?
diamond-blackfan syndrome
associated cardiovascular co-morbidities w/ turner syndrome
bicuspid aortic valve, coarctation of the aorta, and aortic root dilation
early cyanosis - blue babies are R–>L shunts (5 T’s)?
- truncus arteriosus
- transposition
- tricupsid atresia
- tetralogy of fallot
- TAPVR
4 features of tetralogy of fallot?
- pulmonary stenosis
- RVH
- overriding aorta
- VSD
late cyanosis - blue kids are L–>R shunts (3)
- VSD
- ASD
- PDA
pt w/ bounding peripheral pulses, wide pulse pressure, and continuous machine like murmur. dx?
PDA
most common early cyanotic congenital heart condition in newborns? risk factors? CXR finding? tx?
transposition of great vessels. risk factors include maternal DM and DiGeorge syndrome. CXR finding is an “egg-shaped silhouette.” tx includes IV PGE to maintain PDA and surgery
most common early cyanotic congential heart condition in childhood? key PE finding? CXR finding? tx?
tetralogy of fallot. key PE finding is children will squat for relief during hypoxemic episodes (tet spell) –> increased SVR. CXR finding is a “boot-shaped heart” tx includes IV PGE to keep PDA open and surgery
pt w/ FTT, easy fatigue, and heart failure w/ pansystolic murmur heard loudest at the L LSB and a diastolic rumble at the apex due to increased flow across the mitral valve
large VSD
pt w/ EKG that shows shortened PR interval, delta wave, and widened QRS complex
wolff-parkinson-white syndrome
allergic contact derm is what type of HSR
HSR type IV - delayed type - T-cell mediated
pt w/ self-mutation, neuro features, gouty arthritis, and tophus formation. dx? cause?
lesch-nyhan syndrome. caused by def in HGPRT (hypoxanthine-guanine phosphoribosyl transferase)
pt w/ nocturnal vulvar itching. dx? confirm dx? tx?
pinworms. confirm by scotch tape test. tx w/ mebendazole
