HY Peds Shelf REview

96 flashcards · Other · by Jae ho Kim

zero recollection of waking up from bad dream What sleep disorder? mx?
sleep TERROR (N3) reassurance (for most parasomnias)
vivid recollections of dream which sleep disorder? mx?
Nightmare disorder (REM = beta waves) reassurance (for most parasomnias)
BOY recurrent sinopulmonary infections fam hx: men lymphoid HYPOplasia (eg "no cervical LAD, no evidence reactive hyperplasia) which immunodef syndrome?
bruton's agammaglobulinemia (failed differentiation of CD19 & 20+ cells)
Diagnostic test for CGD?
nitroblue tetrazolium/dihydrorhodamine tests
partial albinism recurr resp infections neuro problems "giant granules in cytosol" which immunodef syndrome?
Chediak Higashi LYST gene mutation
18 mo old hypertension, unilateral non-calcified abdominal mass diagnosis & pathophys?
Wilms tumor = tumor from embryonal renal cells (chr 11 on WT1 gene or PAX6 gene) classic triad: HTN, hematuria, flank mass (common on exams, rare in real life) ass'd WAGR complex -Wilms -Aniridia -GU anomalies -Mental Retardation dx = ultrasound first, then CT
posterior mediastinal mass +/- opsoclonus/myoclonus "sign" increase VMA & HVA in serum & urine what cells is the mass derived from? What is dx?
Neuroblastoma - from neural crest cells produce catecholamines CALCIFIED/often cross midline on CT)
hemihypertrophy hepatoblastoma wilms tumor macroglossia neonatal HYPOGLYCEMIA from islet cell hypertrophy = px as seizures which syndrome?
Beckwith-Wiedemann syndrome
Increased nuchal translucency on prenatal US = associated with _____ syndrome
Down syndrome
quad screen results for Down syndrome?
high = b-hcG + Inhibin A low = AFP + Estriol
symmetric IUGR = ass'd with which neonatal diagnosis? ________ ______
FAS TORCH infection (head & body = small)
Asymmetric IUGR = ass'd with ______
insults later in dvlpment eg maternal malnutrition....causes of uteroplacental insuff (normal head, small body)
Treatment of Kawasaki?
IVIG high dose aspirin
Treatment for Lyme disease in children <8 yo?
amoxicillin bc doxycycline = permanent tooth discoloration lyme cardiac/neuro disease tx = ceftriaxone
most common overal cause of osteomyelitis?
S. aureus
most likely bug? osteomyelitis in 16d old neonate
S. Agalactiae (GBS)
2 yo M w/left ankle osteomyelitis. Recent history of stepping on a “sharp substance”
P. Aeruginosa (remember association with footwear)
5 yo M w/right knee osteomyelitis. Spleen is not visible on abdominal imaging
Salmonella (remember the association with sickle cell disease)
-7 yo M with fevers for the past 6 weeks. -He has 2 febrile episodes/day. -PE is notable for diffuse cervical lymphadenopathy and hepatosplenomegaly. -Mom reports that he occasionally has a “salmon colored” skin rash that seems to appear with these febrile episodes. They disappear not too long after his fevers subside MOST LIKELY DIAGNOSIS?
systemic arthritis (Still's disease) 1 of 3 HY types of JIA key association: "salmon colored rash" "picket fence fevers" tx: NSAIDS
HY association for OLIGOARTICULAR JIA
anterior uveitis mx: slit lamp exam *also ANA positivity
7yo M had URI px: painful limp starting yesterday WBC = wnl ESR marginally elevated no joint erythema or effusion Dx?
transient hip synovitis tx: NSAIDs mx: arthrocentesis if 'joint findings'
4 yo F is brought to the ED by concerned parents. She is completely fine during the day but cries to sleep at night from bilateral calf pain. Her PE is completely normal. She runs around and plays with her friends during the day. dx?
Growing pains = classically pain @ night ...disappears during day if localized bone pain worse @ night relieved w/ analgesics = osteoid osteoma
kid has gradual onset severe hip pain >> trendelenburg pattern of ambulation buzzword: "antalgic gait" dx?
Legg Calve Perthes disease (avascular necrosis of femoral head()
neonate failed to pass meconium (FTPM) @ birth rectal prolapse as teen along w/ failure to thrive infertility. recurrent respiration infections DM. elevated PTH dx?
cystic fibrosis fat soluble vit def (vit D) = hypoCa = high PTH under 20 yo = S. aureus resp infection + 20 yo = P. Aeruginosa/Burkholderia super HY ass'd = nasal polyps/digital clubbing
wheeze = upper or lower resp tract infection?
LOWER RSV = bronchiolitis, cough/rhinorrhea some days before PNA = consolidation, hyperresonance to percussion, incr tactile fremitus
stridor = upper or lower tract infection?
UPPER ! epiglottitis = super high fevers, acute onset respdistress/drooling/difficulty swallowing laryngotracheabronchitis (croup)
Epiglottitis arises from _____ obstruction ass'd w/ ___ sign on CXR
supraglottic thumbprint sign
-5 mo brought to ED "jerky" movements of head, arms, legs = 2 weeks multiple times per day over short time period hx: gross & fine motor developmental delay PE: hypopigmented macules ...more so on trunk......rest is wnl dx? mx/tx?
Tuberous Sclerosis w/ infantile spasms (aka West Syndrome) -hypsarrhythmia on EEG ACTH (or vigabatrin) = symptom control w/ proopiomelanocortin derivative
treatment for absence seizures?
ethosuximide 3 Hz spike & wave
tx for typical febrile seizure?
NSAID
atypical febrile seizure criteria?
focal seizures +15 min +1 neuro deficits in 24 hr period
-Mutation in chromosome 17. -PE is notable for iris hamartomas, freckles under the axilla, and multiple “growths” on the body. There are hyperpigmented macules on the skin. -The infant has a history of a tumor causing an afferent pupillary defect. diagnosis?
chr 17 mutation = Von Recklinghausen disease = Neurofibromatosis 1 -iris hamartomas (Lisch nodules) -freckles under axilla -multiple 'growths' on body = neurofibormas -hyperpigment macules on skin = cafe au lait spots tumor causing afferent pupillary defect = optic nerve gliomas
most likely cause of HTN? 15 yo F...recently became sexually active
OCPs
most likely cause of HTN? 16 yo M with an abdominal mass + episodic headache. Urinary metanephrines are elevated.
pheochromocytoma ass'd w/ MEN syndrome block alpha receptors FIRST to prevent unopposed HTN....then beta
most likely cause of HTN? 16 yo F with a normal plasma aldosterone/renin ratio. Plasma renin and aldosterone are elevated.
Fibromuscular dysplasia
most likely cause of HTN? 16 yo M w/ elevated plasma aldosterone/renin ratio plasma renin is extremely low
Conn's Syndrome (adrenal adenoma secreting aldosterone, give Spironolactone)
most likely cause of HTN? 11 yo F with elevated BP in her arms. Dorsalis pedis pulses are not palabale bilaterally
Coarctation of aorta
most likely cause of HTN? 5yo F w/ thoracic CT revealing posterior mediastinal mass
neuroblastoma
most likely cause of HTN? 12 yo F w/ atrial fibrillation
hyperthyroidism
most likely cause of HTN? 8mo F w/ ambiguous genitalia
Congenital Adrenal Hyperplasia (11-beta hydroxylase def) (21 hydroxylase def is most cc)
child w/ peripheral edema, hematuria, HTN ...FEW DAYS after upper resp (or ab infection consider _____ (renal dx)
IgA nephropathy (esp if proteinuria < 3.5 g /day)
** 2-6 WEEKS after URI **subepithelial humps ** 'tea colored urine' ** low C3, C4 also peripheral edema, hematuria, HTN
PSGN appropriate testing (ASO & anti-Dnase B antibodies) tetrad: -HTN -hematuria -edema -recent infection
name of condition when neonate's skin appears mottled due to low temperature (eg room temp 45)?
cutis marmorata (vasomotor instability in preemies)
grey macules over intergluteal cleft seen in 5day old neonate are called _____
Mongolian Spots common in AAs
rash on newborn's leg & ankle. PE notable for erythematous yellow white papules/pustules on involved extremities dx?
Erythema Toxicum Neonatorum (aka baby acne) mx: reassure mom (2wk resolution) super HY to know they contain eosinophils
RDS Decreased surfactant levels triggers... ________ in lung surface tension ________ in lung compliance ______ work of breathing ______ (lung collapse) reduction in __ available for gas exchange tx involves ___________
increase decrease increase atelectasis SA PPV w/ lung protective strategies (low tidal volumes w/ adequate PEEP) + deliver SA directly to airway
buzzwords: "tachypnea w/ no hypoxia" "perihilar streaking on CXR w/ 'fluid in fissures" resolution in few hrs which pulmonary condition?
transient tachypnea of newborn = delayed resorption of fetal lung fluid
HY risk factor for MAS (meconium aspiration syndrome) = ________ cxr ? ______ ______
post term delivery (+ 42 weeks) patchy atelectasis lung hyperinflation
'succussion splash' is code word on NBME for _______
gastric outlet obstruction (fluid/gas in obstructed organ)
HY RF for pyloric stenosis ?
firstborn male exposure to erythromycin in first few wks of life
Diagnosis method for intussusception ? PE notable for palpation of "sausage like mass"
contrast (barium) or air enema
Reye syndrome avoid aspirin for febrile viral infections in kids exception = kawasaki's disease
Necrotizing enterocolitis classic imaging = pneumatosis intestinalis (air in wall of bowel) HY RF = enteral feeding in preemie if abdominal perforation (free air under diaphragm) = do surgery
physiologic jaundice (T. bili mildly high ....low teens & mostly single digits) always unconjugated hyperbili....does not arise in first 24 hr life....resolves 2 wks 2/2 transient UGT def in newborns tx = supportive or photo therapy
jaundice in the first 24 hrs of life or conjugated hyperbilirubinemia in the newborn is ______ (normal or pathologic?)
always pathologic (eg HY dx = biliary atresia...fixed w/ Kasai procedure) low yield dx = choledochal cyst
does phototherapy work for Direct Hyperbilirubinemia?
no DH is always pathologic
_______ syndrome blueberry muffin rash RUQ mass b/L white reflex continuous machine like heart murmur abnormal otoacoustic emission testing mom = poor prenatal care & febrile illness @ 13 wks w/ rash baby is SGA
congenital rubella syndrome also in CMV....b/L cataracts 4.PDA 5.sensorineural HL
most likely dx? newborn w/ 'snuffles' = copious nasal secretions saddlenose deformity anterior bowing of lower limbs upward indentation of teeth frontal bossing of skull
congenital syphilis on NBME, snuffles is syphilils until proven otherwise! depressed nose bridge (also in granulomatosis w/ polyangiitis/wegener's) saber shins hutchinson's teeth
treatment for CF ? __________ = break secretions __________ = Pseudomonas coverage
dornase alpha/N-Acetylchysteine inhaled tobramycin also pancreatic enzyme replacement... fat soluble vitamin replenishment (ADEK)... chest physiotherapy
celiac disease is associated with which skin condition ?
dermatitis herpetiformis
most likely dx? 7yo F ....complains sore throat, high fevers, ab pain, vomiting ...began suddenly yesterday ....PE = tender anterior cervical LAD, redness of hard & soft palate ...'sandpaper rash' along upper extremity
strep pharyngitis via GAS = key*** abrupt onset tx = PCN, Amp/ Amoxicillin
most likely dx ? 3 yo M......sore throat started over 3 days ...persistent cough & rhinorrhea for past week
viral pharyngitis key** URI symptoms BEFORE sore throat begins
most likely dx? 5yo F......sore throat & watery eyes starting 3 d ago PE notable for severe conjunctival erythema & hyperemia
adenoviral conjunctivitis = conjuctivits + pharyngitis +/- abdominal pain
most likely dx? 6yo M.....sore throat & high fevers PE = erythematous lesions w/ central clearing on palms HEENT exam = ulcerative lesions (herpangina) in mouth
Coxsackie A pharyngitis
most likely dx? 14y F....sore throat & mild fevers....past 2 wks extremely tired & unable to participate in sports last 3 wks PE = generalized cervical LAD ...full palpation of LUQ of abdomen initial tx w/ amoxicillin triggered severe dermatologic skin rx which was discontinued
infectious mononucleosis = Ebstein barr virus tx = supportive care & avoid contact sports
most likely dx? 2yo M....brought by "enlightened parents" ....for sore throat HEENT exam = grayish 'pseudomembrane' in posterior pharynx (looks like bull's beck)
Corynebacterium Diptheriae consider as dx if parents immigrants & "enlightened parents"
Distinguish bw peritonsillar vs. retropharyngeal abscess
PTA = uvula devation & 'hot potato voice" RPA = toxic presentation/drooling ....+/- airway compromise....kids <5 on NBME
avoid honey til the child is ______
+1yo
How can you distinguish bw UC & Crohn's?
UC = continuous, does not touch terminal ileum Crohn's = nukes terminal ileum, B12 def, affects anywhere along GI tract, skip lesions, non-caseating granulomas
breast milk/most other formula products have energy value of ____ kcal/ounce
20 kcal/ounce
Elfin facies short upward turned nose long philtrum _____ syndrome
Williams syndrome (also cardiac problems)
tricuspid valve displaced downward, RV hypoplasia ____ toxicity
Lithium Ebstein's anomaly
branchial cleft cyst is derived from which germ layer?
ectoderm
hypopigmented macules identified w/ Wood's lamp shagreen patches adenoma sebaceum (face) infantile spasms subependymal nuldes in brain cardiac rhabdomyosarcoma renal angiomyolipoma dx?
tuberous sclerosis
severe fasting hypoglycemia lactic acidosis hepatomegaly gout +/- kidney sx since it also does gluconeogenesis no muscle sx give cornstarch which enzyme def disease?
von gierke (GSD 1, G-6-P'ase gone)
GSD associated with heart failure & death at <2 yo
pompe's GSD 2, lysosomal acid maltase gone)
mild hypoglycemia hepatomegaly muscle sx no lactic acidosis
Cori GSD3 , debranching enzyme gone
no lactic acidosis muscle weakness no hepatomegaly
McArdle's GSD 5 , muscle glycogen phosphorylase gone
4yo immigrant has 102F, cough, b/L ocular erythema, & runny nose PE = whitish spots on buccal mucosa & palate. red maculopapular rash on face that spreads down to trunk 5d after supportive care, rash resolves in same distribution most likely diagnosis?
measles (rubeola) 3C = cough, coryza, conjunctivitis HY: vit A is helpful if child starts presenting w/ neuro prob (eg seizures) = consider SSPE (subacute sclerosing panencephalitis
most likely dx? day 1: 2yo has low grade fevers & does not feel well day 5: posterior auricular & subocciptal LAD can be appreciated rash starts on head & moves downward
German measles (rubella)
think of _____ as the dx in a 'playful' kid w/ high fevers that precede a 'sudden appearing' rash
roseola/hhv 6 (exanthem subitum)
nbsim
next best step in management
edema from lesions CROSSES suture lines = dx? NBSIM?
caput succedaneum (scalp swelling 2/2 barotrauma from going thru birth canal) completely benign, resolves within few days
scalp edema does NOT cross suture lines dx? NBSIM?
cephalohematoma = subperiosteal bleed resolves weeks-months incr risk: anemia/jaundice as blood resorbs
most likely dx? 15 yo M....3mo hx of fevers & pain jus below femoral head pain initially well controlled w/ acetaminophen but progressively gotten worse & unbearable over past 4 wks labs = incr ESR, mild leukocytosis femur imaging = moth eaten appearance, 'concentric whorling' periosteal bone pattern bx = small blue cells
ewing sarcoma "onion skinning' 11:22 translocation (EWS - FLI) potential tx w/ dactinomycin
most likely pediatric cardiac anomaly? newborn w/ multiple facial defects & hypocalcemic seizures transported to NICU for persistent cyanosis. cardiac auscultation is notable for single S2 heart sounds
Truncus Arteriosus = failed aorticopulmonary septum to seperate truncus arteriosus into pulmnary artery & aorta very common in DiGeorge
most likely pediatric cardiac anomaly? 3hr old newborn in NICU for persistent cyanosis cardiac auscultation = wide, fixed split of 2nd heart sound holosystolic murmur at LLSB diminished S1 CXR = dcr pulm vascular markings EKG = left axis deviation (LAD)
tricuspid atresia ** only congenital cyanotic defect w/ LAD initial tx = PGE1 analog
hyperhomocysteinemia vs marfan syndrome?
HH = marfanoid habitus, mental retardation, lens displaced DOWNWARDS, high serum titers: autoantibody against parietal cells (antiphospholipid) marfan syndrome = marfanoid habitus, no MR, lens displaced UPWARDS, MVP, AR, pectus issues, fibrillin-1 mutation)
Galactosemia = ____ deficiency 3 key findings?
GALT HSM diarrhea, cataracts in newborn sx begin with milk administration = avoid lactose & galactose cc death = e. coli sepsis
kid <1yo.....weird startle response.....cherry red spot on macula.....no HSM = ____ disease
tay sachs hexosaminidase def
kid <1yo.....weird startle response.....cherry red spot on macula.....kid DOES have HSM = ____ disease
Niemann pick (sphingomyelinase def)
hemangioma that sequesters platelets leading to thrombocytopenia = ______ syndrome
Kasabach Meritt syndrome
low plts in setting of skeletal deficits (like absent radius) = _____ syndrome
TAR syndrome (thrombocytopenia with absent radius
Which one has corneal clouding? Hurler or Hunter
corneal clouding = hurler no cc, w/ coarse facial features & x-linked = Hunter's
kid w/ progressive flaccid paralysis ...<1 yo ...muscle weakness, tongue fasciculations, ...ultimately death from resp insuff dx?
Werdnig hoffman disease (aka Spinal Muscular atrophy auto recess SMN1 gene
super HY association of Chiari 2 with _____
lumbosacral myelomeningocele