UWorld peds Flashcards
What does vitamin E deficiency lead to?
hemolytic anemia and neurologic abnormalities
what heart sounds are associated with TOF?
- ULSB harsh systolic cresc decresc ejection murmur (due to RVOT obstruction - pulmonic stenosis/atresia)
- single S2 (due to pulmonic stenosis)
how does hypoplastic left heart syndrome usually present?
heart failure in the first few weeks of life; usually no murmur
what other defect (other than NTD) is elevated AFP associated with?
abdominal wall defect
treatment for gastroschisis after birth?
- sterile saline wrap
- NG tube to decompress bowel
- abx
- surgical repair w/ single stage closure
how is omphalocele different?
- often assoc w/ other congenital defects
- surgery with staged closure via silo
MCC osteomyelitis?
S. aureus
onset of gonococcal conjunctivitis and tx?
2-5 days after birth; IV or IM ceftriaxone or cefotaxime
onset of chlamydial conjunctivitis and tx?
5-14 days
oral erythromycin
unilateral tearing w/ minimal conj. injection. dx and tx?
nasolacrimal duct obstruction; tx w/ massage of nasolacrimal ducts
rash w/ sandpaper like texture? dx and tx
scarlet fever; tx w/ PCN V
- think circumoral pallor and strawberry tongue
what does abx after GAS infxn protect you from?
rheumatic fever
what do abx after GAS infxn NOT protect you from?
poststreptococcal glomerulonephritis
hip, groin or knee pain + antalgic gait in kid 4-10
Legg-Calvé-Perthes disease (idiopathic avascular necrosis of the femoral capital ephiphysis)
MC childhood myopathy?
duchenne muscular dystrophy -> proximal muscle weakness and calf pseudohypertrophy
what part of the bone does osteosarcoma target?
metaphyses (growth plate)
- codman’s triangle
what part of bone is Ewing’s sarcoma?
diaphyses
what is the hallmark of neonatal tetanus?
opisthotonus (diffuse hypertonicity)
- can also have feeding difficulty from trismus (lockjaw)
- risk for life-threatening stridor and resp failure
tx for tetanus?
supportive, abx (PCN), tetanus immune globulin
cyanotic infant with left axis deviation and small/absent R waves. suspected dx?
tricuspid valve atresia
- dec blood to RV -> underdev. of pulmonary vasculature
RF for congenital heart disease?
- congenital rubella syndrome
- down syndrome
- maternal diabetes
- FH of CHD
CXR: increased pulmonary markings and cardiomegaly
complete AV canal defect
- due to excessive pulmonary BF and biventricular volume overload
- assoc w/ DS
tall P waves, R axis deviation on EKG, cardiomegaly
Ebstein’s anomaly
- displacement of malformed tricuspid into RV -> severe triscupid regurg and R atrial enlargement
type of sz easily provoked by hyperventilation?
absence seizure
characterisitcs of absence sz?
- last 10-20 sec
- abrupt onset and resolution
- +/- automatisms
- 3Hz spike/wave on EEG
- easily provoked by hyperventilation
Lennox-Gastaut syndrome
- presents by age 5
- MR
- severe sz of varying types (atypical absence, tonic)
- EEG; slow spike wave pattern
management steps of caustic ingestion
- secure ABCs
- decontaminate (remove clothing, irrigate)
- CXR if resp symptoms
- endoscopy w/in 24 hrs
definition of primary amenorrhea?
absence of menarche by age 15
best way to confirm turner syndrome?
karyotype analysis (if neg, an high IOS do FISH for mosaicism) *buccal smear for barr bodies is outdated and unreliable screening test!
which RTA is commonly genetic and assoc w/ nephrolithiasis?
type 1 -distal (poor hydrogen secretion into urine)
alkalotic urine
which RTA is assoc w/ Fanconi syndrome?
type 2 - proximal
poor bicarb resorption
which RTA causes hyperkalemic hyperchloremic metabolic cidosis?
type 4 - defect in sodium/potassium exchange in distal tubule (aldosterone resistance)
typical labs of RTA
- low serum bicarb
- hyperCl
- normal anion gap metabolic acidosis
definition of precocious puberty
girls <8
boys <9
definition of infantile colic
excessive cry for >= 3 hrs daily >= 3 days/week for >= 3 weeks
- usually resolves by 4 months
suspected dx? symm swelling of hands and feet age 6mo-4yrs
dactylitis (handfoot syndrome); early manifestation of vaso-occlusion in SCD
how does rubella rash differ from measles?
- it does not darken
also lower fever, no koplik spots
what are some infectious complications of atopic dermatitis?
- impetigo (s. aureus, s. pyogenes)
- eczema herpeticum(HSV1)
- molluscum contagiosum (poxvirus)
- tinea corporis (trichophyton rubrum)
painful non-pruritic pustules w/ honey crusted adherent coating
impetigo
painful vesicular rash w/ punched out erosions and hem. crusting
eczema herpeticum
pruritic circular patch w/ central clearing and raised, scaly border
tinea corporis
where does atopic dermatitis present on body in infant vs. child/adult?
infant: extensor surfaces/elbows/trunk/knees/cheeks
child/adult: flexor surfaces: dorsal ankles, popliteal fossae, volar wrists, antecubital fossae, neck
erythematous plaques and/or yellow greasy scales on scalp, face, umbilicus, diaper. dx and tx?
seborrheic dermatitis
tx: 1) emollients, nonmedicated shampoos
2) topical antifungals, low potency steroids
when do you expect to see seborrheic dermatitis?
- peaks 1st year of life AND adulthood
glossitis, dermatitis, GI complaint, diarrhea, mental status changes. vit def?
B3, niacin (condition=pellagra)
vit def: angular cheilosis, stomatitis, glossitis, normocytic anemia, seborrheic dermatitis
B2 (riboflavin)
vit def: cheilosis, stomatits, glossitis, irritability, confusion, depression
B6 (pryidoxine)
vit def: punctate hem, gingivitis, corkscrew hiar
vit C (ascorbic acid) def = scurvy
vit def: peripheral neuropathy and heart failure
B1 (thiamine) def = beriberi
dry = peripheral neuropathy
wet = dilated cariomyopathy
toxicity: neuropsych sx and cerebral edema
vit A toxicity
what is the other major symptom of Lesch-Nyhan syndrome besides self mutilation?
dystonia - hypotonia, choreoathetosis, spasticity
recurrent sinopulm and GI infxns after age 6months. dx?
x-linked agammaglobulinemia (abnormal B lymphocyte maturation)
aka Bruton agammaglobulinemia - deftect in tyrosine kinase
tx: IVIG, abx ppx
how do you dx X-linked agammaglobulinemia?
dec Ig and B cells.
normal T cell amt
no response to vaccinations!
(also will see no/low lymphoid tissue on exam)
severe recurrent viral, fungal, and bacterial infxns + FTT
ADA def (SCID) **autosomal recessive**
tx: BM xplant, gene therapy
recurrent skin and pulm infections w/ catalase pos orgs
CGD (impaired oxidative burst)
- defective NADPH oxidase
tx for hereditary spherocytosis?
- folic acid supp
- blood txfusion
- splenectomy
hereditary spherocytosis
- dx and triad?
- AD, esp. No. Europe
- hemoloytic anemia, jaundice, splenomeg
- dx: inc MCHC, spherocytes on smear, neg coombs, pos osmotic fragility (acidified glycerol lysis test), abn eosin-5-maleimide binding test
complications of hereditary spherocytosis?
- pigment gallstones
- aplastic crises from parvovirus B19
hemolytic anemia, cytopenia, thrombosis. dx?
Paroxysmal nocturnal hemoglobinuria
with what kind of anemia might glucocorticoid therapy be helpful?
warm-agglutinin anemia (low Hb, reticulocytosis, coombs +)
precocious puberty, cafe au lait spots, bone defects. dx?
McCune-Albright
- AD
- often oassoc w/ other endocrine disorders
3 P’s of McCune Albright
- precocious puberty
- pigmentation
- polyostotic fibrous dysplasia
how do you confirm a dx of pyloric stenosis?
abdominl U/S => thick, elongated pylorus
when might you see metabolic acidosis when the patient is also vomiting? (vomit usually -> alkalosis)
when pt also has diarrhea -> sig. loss of bicarb in stool
why is tetany and sz due to hypocalcemia unlikely after the NB period?
compensatory hyperplasia of existing parathyroid tissue
how do migraines present in kids compared to aduls?
- often bifrontal and of shorter duration
indications for neuroimaging in a child w/ headache:
- occipital headache
- hx of coordination problems
- numbness, tingling, FND
- awakens from sleep w/ HA
- increasing freq
what is seen w/ Trendelenberg sign and what is the cause?
- stand on one leg, drooping of contralateral pelvis
- due to weakness of gluteus medius/minimus (superior gluteal nerve), hip abduction on side of STANDING LEG
how is neonatal polycythemia defined and what causes it?
hematocrit >65% in term babies;
cause: inc erythropoiesis from intrauterine hypoxia (maternal db, maternal htn, smoking, iugr) or erythrocyte xfusion (delayed cord clamping, twin-twin transfusion)
clinical prsentation and tx of neonatal polycythemia?
sx: ruddy skin, dec BG, resp distress, cyanosis, apnea/irritiable/jittery, abd distention
tx: partial exchange xfusion (remove blood, infuse NS) **IF asx: hydration by feeding or IVF
pathophys behind cystic hygroma of the neck?
severe obstruction of lymphatic vessels -> congenital lymphedema
what is the pathophys of refeeding syndrome?
carb ingestion -> insulin secretion -> cellular uptake of P, K, Mg -> dec. serum P, K, Mg -> inc Na/water retention -> CHF
low phos-> low ATP
low K, Mg->arrhythmia
inheritance pattern of myotonic dystrophy?
AD, CTG repeat expansion
problems assoc w/ myotonic dystrophy
- cataracts
- testicular atrophy
- baldness
(in addition to myotonia, facial weakness, foot drop, dysphagia and heart conduction abn)
how does squatting affect TOF?
increased systemic vascular resistance (afterload) => dec. R to L shunting across VSD => improves cyanosis and Increased murmur intensity (increased flow thru RVOT)
How do you distinguish CVID?
- less severe, presents later, normal B and T cell counts
acute lymphoblastic leukemia
MC childhood cancer, male > female, age 2-5
rf: down syndrome
- can have dec or inc. WBC
>25% lymphoblasts on BM smear is diagnostic
until what age is intermittent strabismus considered normal (due to ocular instability of infancy)
<4 months
MCC intussusception before age 2?
viral illness -> Peyer patch hypertrophy (lead point)
MCC recurrent intussusception (older kids)
- Meckel’s diverticulum
dx w/ technetium-99m scan
tx w/ sx
dx and tx of choice for fb in esophagus?
flexible endoscopy
asx scattered erythematous macules, papules & pustules throughout body of NB <2 weeks old
erythema toxicum neonatorum; completely benign, no tx needed
- dx supported by sterile pustule and inc. eosinophils
tx for HUS?
supportive: fluid/elec management, blood xfusions, dialysis
hyper IgM syndrome
due to X-linked genetic defect in CD40 ligand;
tx: abx ppx
IVIG prn
gold standard for dx Hirschsprung?
rectal suction bx
serum sickness like reaction
MCC = abx
fever, urticaria, polyarthralgia 1-2 wks after 1st exposure (less common: HA, edema, LAD, splenomegaly)
- type III HSR
when does UGT reach adult levels?
2 weeks after birth. asian nb have dec UGT activity espeically (jaundice)
features of absence sz
- occur during all activities
- length <20 s
- lack of response to vocal/tactile stim
- simple automatisms present (eyelid fluttering, lip smacking)
- usually age 4-10
- can provoke w/ hyperventilation
- EEG: 3hz spike wave
tx: ethosuximide
Fanconi anemia
- pancytopenia
- genetic defect in genes for dna repair
- congen anomalies: hyperpigment/cafe au lait, short stature, upper limb abn, hypogonad, skel anom, eye/eyelid changes, renal malformations
- onset 4-12 yrs old: dec plt=> dec polys => dec rbcs
Diamond blackfan anemia
- congenital pure red cell aplasia
- presents first 3 months of life w/ pallor & poor feeding
- normo/macro anemia w/ dec. retic
- normal WBC and plt
transient erythroblastopenia of childhood
- acquired red cell aplasia
- onset 6mo-5 yrs
- normocytic normochrom anemia w/ dec retic
sturge weber
acquired
- unilat cavernous hemangioma of trigem distribution (nevus flammeus)
- sz
- intracranial calc
tuberous sclerosis
AD mutation
- sz
- adenoma sebaceum
- facial angiofribromas
- ash leaf spots
- hamartomas
HSP
IgA vasculitis of small vessels
- palp purpura on LE
- arthralgias
- abd pain
- renal disease
how is HIV dx in infancy?
DNA PCR
- persistence of HIV ab after 18 months
within what time frame should transient synovitis resolve?
1-4 weeks
what muscle movements are limited in LCP?
internal rotation and abduction of the hip
Tourette’s dx
multiple motor AND one or more vocal tics before age 18, occurs many times a day, nearly every day for at least a year
what comorbid conditions are assoc w/ tourette’s?
ADHD and OCD
dx and tx of biliary atresia?
- U/S - absent/abn GB
- failure of liver to excrete tracer into SI on scintigarphy
- cholangiogram in OR (def. dx)
tx: 1. kasai procedure (hepatoportoenterostomy) 2. liver xplant
how does biliary atresia present?
kid initially well then over 1-8 weeks: - jaundice - acholic stool/dark urine - hepatomegaly - conj. hyperbili - mild elev. LFTs
how does breast milk jaundice present?
- 2nd week of life
- indirect hyperbilirubinemia
Crigler-Najjar vs. Gilbert’s syndrome
both are inherited def of UDP-glucuronyl xferase -> unconj. hyperbili
- CN = absent enzyme, need liver xplant
- Gilbert: mild
alloimmune hemolytic disease (erythroblastosis fetalis)
- unconj hyperbili
- Coombs + HA
- mismatch b/w infant and maternal blood types (Rh, ABO, or minor blood group antigens)
timeline for physiologic jaundice?
unconj. hyperbili appears after first 24 hours, resolves within the first week
age range of most breath holding spells?
6mo-2yrs
reye syndrome
microvesicular fatty infiltration and hepatic mitochondrial dysnfunction secondary to peds aspirin use in setting of flu/varicella infxn
- acute liver failure
- encephalopahty
- labs: inc LFTS, inc PT, PTT, ammonia
breastfeeding failure jaundice
- inadequate stooling/signs of dehydration
- dec. bili elim and inc. enterohepatic circ of bili
how many wet diapers should an infant have a day in the first week?
wet diapers per day = infant’s age in days
how does breast milk jaundice differ?
NO signs of dehydration or feeding problems
how should NB breastfeed in first months?
8-12x a day (q 2-3 hours) for >=10-20 minutes per breast
what is the threshold for phototherapy in a full term infant?
bili >=20 mg/dL
when to do exchange xfusion for jaundice?
> = 25 mg/dL or bili-induced neuro dysfxn
trisomy 18
Edwards syndrome
- micrognathia
- prom. occiput
- low set ears
- clenched hands w/ overlapping fingers
- heart defects (VSD = MC)
- renal defects
- limited hip abduction
- rocker bottom feet
management of ITP in kids vs adults?
KID asx: observe
KID bleed: IVIG OR steroids
ADULT
plt 30K w/o bleed: observe
plt <30k OR bleed: IVIG OR steroids
metatarsus adductus
- flexible positioning
- medial deviation of forefoot
- neutral position of hindfoot
tx: reassure!
(MC congenital foot deformity!)
congenital clubfoot
- rigid position
- medial/upward dev of forefoot and hindfoot
- hyperplantar flexion of foot
tx: serial manip and casting; sx if refractory
what alarm symptoms differentiate orbital cellulitis from preseptal cellulitis?
- ophthalmoplegia
- pain w/ EOM
- proptosis
- vision impairment
tx for cat scratch?
azithromycin
Langerhans cell histiocytosis
- casues solitary lytic long bone lesion
- may be painful/swell/cause path fx
- locally destructive but resolve spontaneously
esophageal atresia with TEF
- excessive drooling and choking, coughing, regurg w/ initial feeding attempts
- can have polyhdramnios
- enteric tube in prox esophagus
- inability to pass feeding tube into stomach
- abd distention, resp distress
- aspiration pna is a complication
- assoc w/ VACTERL
presentation of congenital diaphragmatic hernia
- cyanosis, resp distress after birth
- can have polyhydramnios
- scaphoid abdomen, displaced cardiac silhouette, bowel in thorax, gasless abdomen
presentation of duodenal atresia
- polyhydramnios and vomiting w/ initial feeds
- NO resp distress
- nondistended abd, no intestinal gas on XR
- can insert gastric tubes w/o resistance
- air in stomach and prox duodenum “double bubble sign”
what is the other name for osgood schlatter dz?
traction apophysitis of tibial tubercle
(occurs where patellar tendon inserts)
- tenderness over proximal tibia
presentation of patellar tendonitis?
anterior knee pain after exercise
- point tenderness at inferior pole of patella
patellofemoral stress syndrome
- common in runners
- anterior knee pain worse when going down steps/hills
- pain in patella
should presumed bacterial pharyngitis be treated empirically without further confirmatory testing?
NO, must avoid abx if viral
- centor criteria is only for adults! (fever, tender, ant cervical LAD, tonsillar exudates, no cough)
How is centor criteria used in adults?
0-1: no testing/tx
2-3: RST, tx if positive
4: empiric abx or RST
MC brain tumor in kids?
low grade pilocytic astrocytoma
how do ependymomas present in kids?
4th ventricle in posterior fossa -> CSF obs and inc ICP
- weakness/sz less common
medulloblastoma
- posterior fossa
- arise from cerebellar vermis
- vomit, HA, ataxia
neuroblastoma
arise from symp ganglion cells
- usually abd mass
- mets: bone, liver (brain if late)
how does pediatric viral myocarditis present?
- viral prodrome (adeno or coxsackie b), then Heart Failure
- rsp distress from acute LHF and pulm edema
- holosystolic murmur (dilated cardiomyopathy -> mitral regurg)
- hepatomegaly (RHF)
dx: hypodense lesion in prox femur, worse at night, better w/ NSAIDs
and tx:
osteoid osteoma
- serial exams/xray every 4-5 months
MCC proteinuria in kids?
TRANSIENT (from fever, exercise, seizures, stress,vol depletion)
- must repeat dipstick testing on 2 subsequent occasions
Cow’s milk amount and timing?
do not start before age 1,
kids should consume <24 oz/day
what is the cut off for managing neonatal wt loss?
7%
what do you do for loss <7%
continue exclusive BF
- f/u age 10-14 days to check that infant regained birth weight
what do you for loss >= 7%
assess for oromotor dysfxn, assess for lactation failure, daily wts, consider formula supplementation
what are the “pink stains” in neonatal diapers?
uric acid crystals, commonly seen during 1st week
primary dose limiting SE of hydroxyurea?
myelosuppression
macrocytic anemia, low retic count, congenital anomalies (short stature, webbed neck, cleft lip, shield chest, triphalangeal thumbs). dx?
diamond blackfan syndrome (congenital hypoplastic anemia)
>90% dx w/in 1st year of life, avg age = 3months
pure red cell aplasia WITHOUT macrocytosis. dx?
transient erythroblastopenia of childhood
- usually dx after 1 year of age
- no assoc congen anomalies
progressive pancytopenia and macrocytosis. ~8 yrs old. assoc w/ cafe au lait spots, microcephaly, microphthalmia, short staure, horseshoe kidneys, absent thumbs
Fanconi’s anemia
AR disorder
when do you do bx for MCD?
- kid >10 w/ nephrotic syndrome
- any MCD unresp to steroids
biggest RF for cerebral palsy?
prematurity
how is CP characterized?
nonprogressive motor dysfxn
spastic, dyskinetic, or ataxic
MCC bacterial lymphadenitis in children
S. aureus
tx of bacterial meningitis in children age >1 month
IV vanc &ceftriaxone (OR cefotaxime)
+ dexamethasone if H flu
how do carotid injuries present in kids?
mech: penetrating trauma, fall w/ object in mouth, neck manipulation
pres: gradual onset hemiplegia, aphasia, neck pain, “thunderclap HA”
dx: CT or MRA
who is at risk for NEC besides premies?
very LBW infants or infants w/ reduced mesenteric perfusion from CHD
androgen insensitivity syndrome
xlinked mut of androgen receptor \+ breast dev. no uterus/upper vagina; cryptorchid testes minimal/absent axillary & pubic hair karyotype: 46 XY Inc: T, E, LH
mullerian agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome)
hypoplastic or absent mullerian duct system
+ breast dev
absent/rudimentary uterus & upper vagina, normal ovaries
normal pubic hair
46XX
transverse vaginal septum
malformation of urogenital sinus and mullerian ducts
+ breast dev
normal uterus and ovaries, abnormal vagina
normal hair
46xx
turner syndrome
complete/partial absence of 1 x chromosome breast dev variable normal uterus/vagina streak ovaries normal hair 45x Inc: LH, FSH
what should be done w/ cryptorchid testes in “female” child w/ androgen insensitivity syndrome?
perform gonadectomy after puberty
what is an atypical presentation of HSP?
scrotal pain and swelling as initial symptoms
known complications of HSP?
GI hem or intussuscpetion (from bowel wall edema and localized hem = lead points)
**intuss w/ HSP are MC ileo-ileal (instead of the usual ileocolic)
how to distinguish congenital toxo from CMV?
toxo = intracranial calcifications CMV = periventricular calcifications, deafness
both: blueberry muffin rash, chorioretinitis
congenital varicella syndrome
- limb hypoplasia
- cataracts
- distinctive skin lesions (scarring)
congenital syphilis
- hepatomgegaly
- nasal discharge (snuffles)
- osteoarticular destruction
- maculopapular rash
congenital absence of GnRH secretion
Kallman syndrome
5a reductase deficiency
AR, 46XY can't convert T to DHT - ambiguous genitals until puberty, then increased T causes masculinization/inc growth ext. gen - normal T/E - LH normal or inc - normal internal genitals
how do you distinguish niemann-pick from tay-sachs?
Tay Sachs = hyperreflexic
(B hexosaminidase A def)
NP = areflexic, HSM
(sphingomyelinase def)
both: AR, Jewish heritage, onset 2-6 months, loss of motor milestones, hypotonia, feeding difficulties, cherry rad macdul
Krabbe disease: deficiency?
galactocerebrosidase deficiency
Krabbe disease
rare AR LSD
early infancy: develpment regression, hypotonia, areflexia
(NO cherry red macula or organomegaly)
Gaucher disease deficiency:
glucocerebrosidase deficiency
Gaucher dz
anemia, thrombocytopenia, HSM
NO loss of milestones, cherry red macula
Hurler synrome
MPS, LSD
lysosomal hydrolase def
- presents age 6 mo-2 years: coarse facial features, inguinal/umbilical hernias, corneal clouding, HSM
what hereditary condition can cause edema of the intestines -> colicky abd pain
hereditary angioedema (C1 inhib def) => bradykinin - low Cr4 levels noninflamm edema of face, limbs, genitas - laryngeal edema - intestingal edema - NO urticaria
how can you test for diff b/w acquired and hereditary angioedema?
hereditary: normal C1q levels
acquired: depressed C1Q
what does aldolase B def cause?
hereditary fructose intolerance due to accum of F1P
what cells express TdT
pre B and pre T lymphoblasts
tx for impetigo?
limited: mupirocin topical abx
extensive: oral abx (keflex, diclox, clinda)
best dx test for kids w/ inc. Head Circ (hydrocephalus)?
CT brain
tx: shunt
MCC viral meningitis
non-polio enteroviruses like coxsackie and echovirus
what is the only exception to scheduling vaccines by chronologic age?
hep B: pt must weigh >= 2 kg
all other vaccines are okay to give to preemies on same schedule as normal kids!
what is the physiologic effect of a val salva maneuver?
decreases preload
increases HOCM murmur
what maneuvers increase afterload?
sustained hand grip, squatting (also inc. preload), passive
dec HOCM murmur
what maneuver increases preload?
passive leg raise
how does val salva maneuver affect murmurs?
louder: HCM (decreases LV volume, increase gradient)
MVP (dec LV volume)
softer: all others (dec flow through stenotic valve)
how does standing affect murmurs?
(dec venous return)
same affect as val salva
squatting affect on murmurs?
inc venous return (preload) inc afterload
louder: AR, MR, VSD
softer: HCM MVP
how does handgrip affect murmurs?
inc afterload
louder: AR, MR, VSD
softer: HCM, AS (dec pressure gradient)
Friedrich ataxia
AR, sx before age 22: neuro (gait ataxia, falls, dysarthria) from degen of spinal tracts
non neuro: concentric hypertrophic cardiomyopathy, db, skeletal deformities (hammer toes, scoliosis)
MC predisposing factor for orbital cellulitis in kids?
bacterial sinusitis
when do you get a CT head (without contrast!) in a kid 2-18 w/ head trauma?
- FND
- skull fx/basilar skull fx
- sz
- persistent AMS (agitated, lethargic, slow response)
prolonged LOC
when do you NOT need a head CT for head trauma?
minor head trauma: GCS of 15 w/ non severe MOI, no vomiting, HA, LOC, or fx
when is it acceptable to either observe for 4-6 hrs or do head CT?
GCS 15 but any one of the following:
- vomit
- HA
- questionable/brief LOC
- high risk MOI
- severe MOI
what abnormality can give neuro sx in a kid with downs?
atlantoaxial instability
what is atlantoaxial instability?
excessive laxity in posterior transverse ligament (dec mobility bw C1 and C2)
sx rare; from compression of cord: behavior change, torticollis, urinary incont, vertebrobasilar (dizzy, vertigo, diplopia)
UMN signs
dx: w/ lateral spinal XR
tx: cervical fusion
what are the typical sx of cerebellar astrocytoma vs medulloblastoma?
medulloblastoma (vermis) : truncal/gait ataxia
astrocytoma (lat hem): fine motor, dysmetria, intention tremor, dysdiadochokinesia
Parinaud syndrome
pressure on pretectal midbrain -> eyelid retraction, limitation of upward gaze, light-near dissoc
- assoc w/ pineal tumors
1st steps for NB with bilious emesis?
stop feeds, NG decompress, IVF
then abdominal XR
bilious emesis + KUB w/ free air, hematemesis, VS unstable?
surger
bilious emesis w/ KUB: showing dilated loops of bowel?
get contrast enema:
microcolon = meconium ileus
rectosigmoid transition zone = Hirschsprung dz
bilious emesis, KUB: NG misplaced in duodenum?
upper GI series: ligament of treitz on R side of abdomen: malrotation
bilious emesis KUB: double bubble
duoudenal atresia
when is MC to have imaginary friend?
- age 3-6
but can be anytime during school age
this is NORMAL, and can help w/ real relationships
differentiate Hirschspung from meconium ileus
assoc disorder:
H = DS
M = CF
level of obs:
H:rectosigmoid
M: ileum
mec consistency
H: normal
M: thick (“inspissated)
squirt sign
H: pos
M: neg
until what age should infant be breasted exclusively?
0-6 months
when to introduce pureed foods?
6 months
when to introduce cows milk?
1 yr
MC benign vasculr tumor in children?
superficial infantile hemangioma: capillaries separated by connective tissue (aka strawberry hemangiomas)
- blanch w/ pressure
infantile hemangiomas
- appear first days/weeks after birth
- grow rapidly during 1-2 years
- regress spontaneously
- rarely disabling or lifethreatening but if so: give B blockers
cavernous hemangiomas
mass of dilated sinusoidal type blood vessels primarily located in brain (can also appear on SC and skin)
- spongy red blue mass
cherry hemangioma
MC benign vasc prolif in ADULTS
- usually widespread on trunk and inc w/ age
blanchable pink-red patch MC on eyelid, glabella, midline nape of neck
nevus simplex
- present at birth, regress spont by age 1-2
seconds in big ekg box?
0.2 s
seconds in little ekg box?
0.04s
DOC for QT prolongation syndrome?
propranolol (add pacemaker if symptomatic or having syncope)
how does nonclassic CAH present?
late onset: late childhood w/ androgen excess: premature adrenarche/pubarche, severe cystic acne, accel. linear growth, advanced bone age
- NORMAL lytes
- LH low, not responsive to GnRH
risk factors for RDS?
#1 = premie others: male sex, perinatal asphyxia, maternal db (delays maturation of surfactant production), C section w/o labor
what factors decrease the risk for RDS?
intrauterine stress stimulates early fetal lung maturity:
IUGR, maternal HTN, prolonged ROM
ratios for exudative effusions
pleural protein/serum protein ration >0.5
pleural LDH/serum LDH >0.6
pleural LDH> 2/3 upper limit normal for serum LDH
types of exudative effusions
empyema (neutrophils, +gram stain/cx)
chylothorax (milky white, inc TG)
malignancy (abn cytology)
tb (+AFB)
MC predisposing factor for acute bacterial sinusitis?
viral URI;
tx: amox clauv
when is it okay to observe an ingested battery for spontaneous expulsion?
if it has already passed beyond the esophagus
what is the cause of bleeding diathesis in pts with CF?
vit K def -> inc Pt and INR with normal PTT
iron poisoning
sx w/in 30 min to 4 days: abd pain, hematemesis, melena/diarrhea, hypotensive shock, AGMA
w/in 2 days: hepatic necrosis
w/in 2-8 wks: pyloric stenosis
*radiopaque pills
differential for T wave inversion?
MI, myocarditis, old pericarditis, myocardial contusion, digitoxin toxicity
how to distinguish homocystinuria from marfan syndrome?
homocystinuria is AR, has MR, thrombosis, downward lens disloc, megaloblastic anemia, fair complexion
(Marfan = AD, aortic root dilatation, upward lens)
deficiency in homocystinuria? and tx
cystathionine synthase
tx: vit B6, folate, B12, anticoag
fabry dz
a-galactosidase def
- angiokeratomas, periph neuropathy, asx corneal dystrophy
- poss renal/heart failure and thromboembolism
how can you distinguish herpangina (coxsackie) from herpetic gingivostomatitis (HSV)?
herpangina: age 3-10, summer/early fall, gray vesicles/ulcers
HSV: 6mo-5yrs, erythematous gingiva, small vesicle clusters on anterior orophrynx
vascular ring
presents in infants, biphasic stridor that improves w/ neck extension
laryngomalacia
inspiratory stridor, worse when supine, improves when prone
pain jaundice and palpable mass in child <10
biliary cyst
dx: U/S or ERCP
tx: sx
dx tx for pertussis?
dx: cx or PCR; lymphocyte-predom leukocytosis
macrolides
MCC syndromic gynecomastia?
Klinefelter (XYY)
Beckwith-Wiedemann syndrome
path: dysreg of imprinted gene exp in chrom 11p15
exam: hemihyperplasia, macroglossia, ompahlocele, fetal hyperinsulinism
complications: wilms tumor, hepatoblastoma
surveillance: serum AFP, abd/renal U/S
triple bubble sign?
jejunal atresia; due to vascular accident in utero -> necrosis and resorption of fetal intestine
what is craniotabes (ping pong skull) associated with?
vit D deficient rickets
also: delayed fontanel closure, frontal bossing, costchondral joints (rachitic rosary) lg bone joints (wrist widening)
genu varum
how do patients with glucose 6 phosphatase deficiency present?
type I glycogen storage disease, von Gierke
- impaired conversion of glycogen to glucose
- age 3-4 mo: hypoglycemia-> sz, lactic acidosis
- hyperuricemia, hyperlipidemia
doll-like face w/ rounded cheeks, thin ext, short stature, protuberant abd (hepatomegaly)
what is the reason behind what happens with Wiskott-Aldrich?
impaired cytoskeleton changes in leukocytes, platelets
what should growth be like by age 12 months?
weight should triple, height increase by 50%
at what ages is universal screening for dyslipidemia recommended?
age 9-11
age 17-21
cutis aplasia and microphthalmia. dx?
Patau (trisomy 13)
cutis aplasia = lack of epidermis over skull; also assoc w/ holoprosecnephaly and omphalocele
IUGR, microcephaly, VSD, closed fists w/ overlapping fingers, micrognathia, rocker bottom feet, prom. occiput
Edwards syndrome (trisomy 18)
what are Turner syndomre patients at risk for later in life?
osteoporosis (lack of estrogen = lack of bone protection)
tx w/ ERT for sexual maturation and dec. osteporosis risk
pathognomonic sign of abusive head trauma?
retinal hem
how to screen for dev dysplasia of hip from age 0-12 months
Barlow/Ortolani positive -> refer to ortho
if negative but asymmetry:
age 2 weeks -6 mos: hip U/S
>4-6 mos: hip XR
if less than 2 weeks, don’t image, won’t show up on imaging
DDH tx?
if <6 mo: Pavlik harness
if older: reduction under anesthesia
MC complication of mumps?
aseptic meningitis
MC bacteria assoc w/ CF by age?
<20: S. aureus
>20: pseudomonas
features of septic arthritis of hip in kids:
- fever >38.5 (101)
- can’t bear wt
- WBC >12,000
- ESR >40
- CRP>2
up until what age is the thymus normally visible on CXR?
<3 years
3 pathognomonic facial dysmorphisms for FAS?
- small palpebral fissures
- smooth philtrum
- thin vermilion border (upper lip)
until what age should you give ppx abx for rheumatic fever?
RF without carditis: 5 years or until 21 years old (whichever is longer)
RF w/ carditis: 10 years or until 21
RF w/ carditits and persistent heart/valvular dz: 10 years or until 40 years old
how is abx ppx for RF dosed?
IM benzathine PCN G q 4 weeks
how is measles transmitted?
airborne
leukocyte adhesion def
WBCs cannot extravasate -> neutrophilia
skin infxn (cellulitis, abscess, omphalocele)
mucosal infxn (periodontal)
- no pus
comorbities of absence sz?
ADHD, anxiety
findings w/ complete AV septal defect>
onset ~6wks
-diaphoresis/dyspnea w/ feeds
- crackles
loud S2 (pulm HTN), systolic ejection murmur (increased flow across pulm valve from ASD), holosystolic murmur ASD
what is the coffee bean sign associated with?
sigmoid volvulus (usually occurs in elderly, not kids)
lab values with thalassemia minor
smear: target cells and teardrop cells
normal RBC count, normal RDW
low MCV
may have slight reticulocytosis
causes of unilateral acute cervical adenitis in kids
S. aureus, S. pyogenes (erythema)
anaerobes ie prevotella (dental caries, periodontal disease)
B. henselae (papular nodular at site of cat scratch/bite)
Mycobacterium avium (gradual onset, nontender)
causes of bilateral acute cervical adenitis in kids?
adenovirus (pharyngoconjunctivitis)
EBV/CMV (mono)
what is spondylolisthesis?
dev disorder: forward slip of vertebrae (MC L5 over S1)
displacement -> chronic back pain, neuro dysfxn
exam: palpable stepoff
best way to evaluate for malrotation/volvulus after bilious emesis?
(NG tube, IVF, cessation of feeds, and abdominal XR have already been done)
upper GI series (barium swallow)
ligament of treitz on R side = malrotation
corkscrew pattern of contrast = volvulus
