Peds Uworld Flashcards

1
Q

cyanosis and single S2 (most common cardiac defect of neonatal period)
in contrast, what is the most common after the neonatal period?
in contrast, what will present with shock due to impaired systemic perfusion?

A

transposition of the great vessels

after: ToF
shock: coarctation of aorta and hypoplastic left heart

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2
Q

recurrent skin/mucosal infections, periodontal disease, ^WBCs with neutrophil predominance, think?

A

Leukocyte adhesion deficiency

also: lack of umbilical cord separation and no pus

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3
Q

acute edema of face, extremities, genitals, trachea, abdominal organs without urticaria, think?

A

hereditary angioedemia
C1 inhibitor deficiency
leads to ^bradykinin and C2b (edema-producing factors)
most common cause of acquired form is from using ACE-i

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4
Q

recurrent infections with S. aureus, Serratia, Burkholderia, Aspergillus, think ? what test to get?

A

Chronic Granulomatous Disease: impaired oxidative burst, NADPH oxidase deficiency, get inf. with catalase+ organisms
think Cats Need PLACESS to Belch their Hairballs
Nocardia, Pseudomonas, Aspergillus, Candida, E. coli, Staph, Serratia, Burkholderia, H. pylori
get dihydrorhodamine 123 test (old: nitroblue)

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5
Q

partial albinism and recurrent cutaneous infections with S. aureus and S. pyogenes, think?

A

Chediak-Higashi

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6
Q

eczema, thrombocytopenia, recurrent infections, think?

A

Wiskott-Aldrich
impaired cytoskeleton changes in WBCs/plts
tx: SCT

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7
Q

fever, lethargy and signs of heart failure after a viral prodrome, think?
how to manage?

A

viral myocarditis
ICU due to risk of acute decompensation and fatal arrhythmia
+/- cardiomegaly and pulmonary edema on CXR

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8
Q

failure to thrive, recurrent infections of all types, low lymphocyte and T cell concentrations, think?
tx?

A

SCID

tx: stem cell transplant

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9
Q

recent Giardia infection and lobar pneumonia, consider ?

A

Bruton’s X-linked agammaglobulinemia: abnormal B lymphocyte maturation, at risk for sinopulmonary infections with encapsulated orgs (H. flu, S. pyogenes) due to imp. humoral response, and GI infections i.e. Giardia due to absence of IgA

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10
Q

contraindications to DTaP

A

anaphylaxis to vaccine ingredients

additional for pertussis part: progressive neurological disorder or encephalopathy within wk of vaccine

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11
Q

tx for long QT syndrome

A

B-blockers (propranolol) with pacemakers (except sotalol which blocks K+ channels)

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12
Q

electrolyte derangements that can cause prolonged QT

A

low Ca2+, low K+, low Mg2+

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13
Q

meds that can cause prolonged QT

A

macrolides, FQs, antipsychs, TCAs, SSRIs, methadone, oxy, zofran, quinidine, procainanmide, flecainide, amiodarone, sotalol

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14
Q

failure to thrive and recurrent respiratory and GI infections, chronic lung disease especially with Giardia, encapsulated bacteria, and enterovirus, think?

A

common variable immunodeficiency: B-cell differentiation impaired leading to decreased of all Igs

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15
Q

wheezing, coughing, dysphagia, biphasic stridor that improves with neck extension, think?
what improves with prone positioning?

A

vascular rings

prone: laryngomalacia (collapse of supraglottic structures during inspiration)

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16
Q

should you tx rheumatic fever with abx?

A

yes, PCN, as they are at risk of recurrent episodes and progression of rheumatic HD with repeated infection with GABS pharyngitis

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17
Q

what is never normal on neonatal EKG?

A

left axis deviation

if present + decreased pulmonary markings on CXR, think tricuspid atresia (hypoplasia of RV and pulm. outflow tract)

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18
Q

AOM orgs

A

S. pneumo, H. flu (not Hib), Moraxella

tx: amoxicillin, then augmentin

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19
Q

how to visualize laryngomalacia

A

direct laryngoscopy

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20
Q

tinea capitis tx

A

oral terbinafine, griseofulvin, itraconazole, fluconazole

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21
Q

bruise looking rash on minority kiddo

A

Mongolian spots, self-resolve during childhood

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22
Q

tinea corporis treatment

A

topical clotrimazole, terbinafine

2nd line: oral terbinafine, griseofulvin

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23
Q

hemangiomas in kiddos

A

strawberry (superficial)

vs cherry in adults

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24
Q

proptosis, ophthalmoplegia (pain with moving eyes), diplopia, think?
most common predisposing factor?

A

orbital cellulitis

bacterial sinusitis

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25
Q

neonate with blanching erythematous papules and pustules, think ?

A

erythema toxic neonatorum, benign and resolves within 2 weeks

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26
Q

bedwetting is normal before age ? and does not warrant treatment before this time

A

age 5

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27
Q

conjunctival injection, tarsal inflammation, pale follicles, think ?

A

trachoma: C. trachomatis A, B, C infection, leading cause of blindness worldwide
tx: oral azithromycin, may need eyelid sx

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28
Q

macrosomia, hypoglycemia, macroglossia, umbilical hernia/omphalocele, hemihyperplasia (1 side bigger than other) think ?
what to do next?

A

Beckwith-Wiedemann syndrome
get abdominal U/S to check for development of Wilms tumor or hepatoblastoma
in contrast, hypothyroidism may have macroglossia and umbilical hernia but not the other features

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29
Q

conjugated hyperbili + hepatomegaly in a newborn, think?
what to do next?
treatment?

A

biliary atresia

dx: abdominal U/S
tx: Kasai procedure

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30
Q

Reye syndrome:
presentation
labs
biopsy

A

n/v, encephalopathy, acute liver failure
^LFTs, ^PT, INR, PTT, ^NH3
microvesicular steatosis
in contrast: macrovesicular fatty changes are seen in alcoholic hepatitis and in NAFLD

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31
Q

1 month old with painless blood-streaked stools, eczema, regurgitating feedings, think?

A

milk- or soy-protein proctocolitis (enterocolitis/allergy)

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32
Q

intussusception dx? tx?

A

dx: U/S: “target sign”
tx: air or water-soluble contrast enema

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33
Q

see microcolon on contrast enema, think?

A
meconium ileus (at the level of the ileum), will also have thick "inspissated" meconium
in contrast, Hirschsprung will have a "transition point" on contrast enema (typically rectosigmoid)
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34
Q

infant supplement timeline

A

all exclusively breastfed infants: 400 IU vitamin D 1st mo
breastfed + preterm infants: iron at birth-1 yr
can introduce pureed foods at 6 mos
can introduce cow’s milk at 1 yr

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35
Q

vitamin B6 (pyridoxine) deficiency presents how?

A

cheilosis, stomatitis, glossitis + irritability, confusion, depression
in contrast, riboflavin (B2) def. presents with cheilosis, stomatitis, glossitis + normocytic anemia, seborrheic dermatitis

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36
Q

15 day old with bilious vomiting and no gas seen on abdominal XR, think ? what to do next?
treatment?

A

malrotation with volvulus (both meconium ileus and Hirschsprung will have some gas, those are dx with contrast enema)

dx: upper GI series (barium swallow), will see Ligament of Treitz on right side of abdomen (malrotation), and “corkscrew” pattern (volvulus)
tx: Ladd procedure (fix bowel)

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37
Q

why do newborns get jaundiced?

at what levels to do exchange transfusion?

A

^hgb turnover/bili production
decreased UGT activity (conjugating enzyme)
sterile gut cannot break down bili to urobilinogen

if total bilirubin exceeds 20-25 mg/dL, otherwise can use phototherapy

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38
Q

where it is worrisome for batteries to get lodged

A

esophagus: needs immediate endoscopic removal to prevent mucosal damage and esophageal ulceration
if distal, 90% pass uneventfully

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39
Q

8 yo: RUQ pain, dark urine, jaundiced, ^bili (D, T), ^amylase, ^lipase, +extrahepatic cyst on u/s, think?

A

biliary cyst: cause cholestasis, palpable mass, +/- pancreatitis
tx: surgical resection
f/u: cholangiocarcinoma

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40
Q

“lead points” in intussusception

A

most common: hypertrophy of Peyer patches from recent illness
others: Meckel’s, HSP, celiac, tumor, polyps
other risk factors: rotavirus vaccine

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41
Q

intussusception tx

A

U/S guided air contrast enema or saline enema

barium enema not done due to risk of peritonitis from barium leakage if intestine perforates

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42
Q

rehydration for dehydrated kiddos

A

mild-mod: oral

mod-severe: IV NS, (dextrose-containing fluids may be used in maintenance, but not resuscitation)

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43
Q

weight loss, iron-def anemia, vesicular skin rash, T1 DM, think?

A

celiac

also associated with IgA deficiency, Downs, AI thyroiditis

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44
Q

constitutional growth delay

A

normal birth weight/height
then drop to 5th/10th % btw 6mo-3yrs, remains low until growth spurt, should reach normal adult height
giving GH will NOT help, they are not deficient

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45
Q

3 mo old: hypoglycemic, lactic acidosis, hyperuricemia, hyperlipidemia
doll-like face, thin extremities, hepatomegaly

A

Glucose-6-phosphatase deficiency (type 1 glycogen storage disease, von Gierke disease)
cannot convert glycogen to glucose
in contrast, glucocerebrosidase deficiency (Gaucher) presents with bone pain, cytopenias, hepatosplenomegaly, but NOT hypoglycemia and lactic acidosis

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46
Q

Turners hormone levels

A

low estrogen, progesterone (“streak ovaries”)

^FSH, LH (disinhibited feedback)

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47
Q

most common cause of congenital hypothyroidism ww

A

thyroid dysgenesis

in contrast, transplacental passage of TSH-rec Abs leads to hyperthyroidism

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48
Q

when to remove testes in AIS

A

after puberty so chica gets to full height but reduces risk of malignancy

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49
Q

lead poisoning tx

A

moderate 45-69: DMSA

severe 70+: DMSA + EDTA

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50
Q

precocious puberty with low LH levels that do not ^after GnRH stim test

A

nonclassic CAH (21-hydroxylase deficiency)

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51
Q

primary amenorrhea w/u

A

FSH if no breast development
if FSH decreased: pituitary MRI (prolactinoma)
if FSH increased: karyotype

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52
Q

abdominal pain, shock, hematemesis, metabolic acidosis + radio opaque areas on XR think ?
tx?

A

iron poisoning, tx with deferoxamine

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53
Q

airborne vs droplet vs contact diseases

A

airborne: measles
droplet: influenza, RSV
contact: MRSA, rotavirus

54
Q

most common causes of acute, u/l LAD in kids

A

S. aureus, S. pyogenes

55
Q

Lyme tx for kids <8

A

amoxicillin PO or cefuroxime

56
Q

impetigo tx

A

topical mupirocin

57
Q

posterior oropharyngeal vesicles/ulcerations, fever, pharyngitis, think?

A

herpangina (coxsackie virus)

tx: supportive

58
Q

all nonimmune, asymptomatic pts older than 1 yr with varicella exposure should be treated how?

A

post-exposure ppx with varicella vaccine

if immunocompromised get VZIG

59
Q

Cat-scratch vs cat bite abx

A

Cat-scratch: azithromycin

Cat bite: augmentin (Pasteurella)

60
Q

osteomyelitis tx

A

nafcillin/oxacillin or cefazolin
if suspect MRSA: vancomycin or clindamycin
if SCD: same as above + ceftriaxone/cefotaxime

61
Q

most common predisposing factor for acute bacterial sinusitis

A

viral URI

62
Q

prophylaxis for close contacts of pertussis?

A

macrolides for all, regardless of age, immunization status, or symptoms

63
Q

most common cause of sepsis in SCD pt

A

S. pneumo (encapsulated, N. men and Hib are less common due to vaccinations)
Salmonella in osteomyelitis, not sepsis

64
Q

hydroxyurea SE

A

myelosuppression

65
Q

ALL markers

A

Tdt+, PAS+, no peroxidase + granules

66
Q

pure red cell aplasia + webbed neck, short stature, cleft lip, shield chest, thumb anomalies, think?

A

diamond-blackfan anemia

67
Q

medulloblastoma location/symptoms

A

posterior fossa, from cerebellar vermis
truncal/gait ataxia
obstructive hydrocephalus and s/s of ^ICP (near 4th ventricle)

68
Q

kid with a stroke, think ? what to do next?

A

SCD, get Hgb electrophoresis

most common cause ped. stroke

69
Q

labs in splenic sequestration

A

low Hgb, ^retics, low plts

in contrast, aplastic crisis will have normal plts but low retic count

70
Q

limitation of upward gaze, think?

A

Parinaud syndrome, occurs with pinealoma

in contrast, craniopharyngiomas (suprasellar) can cause visual field deficits

71
Q

s/s of polycythemia in neonate

A

respiratory distress, hypoglycemia, neurological manifestations

72
Q

craniopharyngioma vs pituitary tumor

A

both can cause visual field deficits and hormone derangements
craniophayngiomas have calcification and light up on CT/MRI

73
Q

anemia of prematurity

A

low EPO, low Hbg, HCT, and retic count

74
Q

poor growth, macrocytic anemia, congenital marrow failure, short stature, other physical anomalies (skin, digits), think?

A
Fanconi anemia (AR)
DNA repair defect causing chromosomal breaks
75
Q

aplastic anemia vs aplastic crisis

A

anemia: pancytopenia due to BM failure (congenital or acquired)
crisis: just anemia, typically in SCD pt exposed to Parvovirus B19 (arrest of EPO, low retic)

76
Q

kiddo develops isolated thrombocytopenia (low plts) and petechia after viral infection, think ? how to manage?

A

immune thrombocytopenia (ITP)
kids typically recover spontaneously in 6 mos, only need observation (regardless of platelet count)
BUT if bleeding should receive IVIG or steroids
in contrast, adults with plts less than 30,000 or bleeding need IVIG/steroids

77
Q

nuclear remnants within RBCs in asplenic pts
ribosomal precipitates with thals or lead
small inclusions seen in G6PD def.

A

Howell-Jolly bodies
Basophilic stippling
Heinz bodies

78
Q

iron def labs

A

low MCV, ^RDW, low iron, low transferrin sat, ^TIBC, low RBC count
in contrast, thalassemia has a normal RDW and RBC cell count

79
Q

sepsis in a SCD kid, think?

A

pneumococcal, ppx with conjugate capsular polysaccharide vaccine and PCN

80
Q

SCD can lead to what vitamin deficiency?

A

folate (^reticulocytosis)

81
Q

what is hemophilic arthropathy seen in hemophiliacs caused by?

A

iron/hemosiderin deposition leading to synovitis and fibrosis in the joint
(prolonged PTT)

82
Q

dx of ALL

A

+25% lymphoblasts on bone marrow biopsy

83
Q

most common complications of bacterial meningitis

A

hearing loss!

others: intellectual injury, CP, learning disabilities, seizure disorder

84
Q

absence seizure disorder is associated with

A

ADHD

85
Q

cherry red macula, hepsplenmeg, areflexia, feeding difficulties (2-6 mos), think?

A

sphingomyelinase deficiency: Niemann-Pick

IN CONTRAST, Tay-Sachs has same presentation but HYPERREFLEXIA and NO hepslenmeg

86
Q

Krabbe disease?
Gaucher?
Hurler?

A

Krabbe: galactocerebrosidase deficiency (hypotonia, areflexia, regression)
Gaucher: GLUCocerebrosidase deficiency (anemia, low plts, hepsplenmeg)
Hurler: lysosomal hydrolase deficiency (coarse features, hernias, corneal cloud, hepsplenmeg)

87
Q

Marfan vs Homocystinuria

A

Marfan: AD, NORMAL intellect, aortic root dilation, UPWARD lens dislocation, Fibrillin-1 gene
Homo: AR, intellectual disability, +thrombosis, DOWNWARD lens dislocation, meg. anemia, fair skin

88
Q

fever, HA, FNDs (classic triad)

+/- seizure in kiddo with congenital HD and recurrent sinusitis, think?

A

brain abscess
^risk for direct spread: OM, mastoiditis, sinusitis, dental infection
^risk blood spread: cyanotic HD (bypass lung), other infected sites (seed GW-J)

89
Q

CGG repeats, think?

A

Fragile X: MCC inherited intellectual disability

prominent jaw, large ears, macroorchidism

90
Q

stroke s/s after fall with toothbrush in mouth, think?

A

carotid artery dissection or thrombus formation

91
Q

FTT, b/l cataracts, jaundice, hypoglycemia, think?

A

galactosemia: galactose-1-phosphate uridyl transferase deficiency
may lead to MR, cirrhosis, ^risk E. coli sepsis
tx: eliminate galactose
mild (only cataracts) if galactokinase deficiency

92
Q

kids with Downs are at ^risk what malignancy

A

ALL

93
Q

LE weakness more than UE

A

Werdnig Hoffman (spinal muscular atrophy)

94
Q

infant botulism is from ?
how to tx?
in contrast to food-borne

A

C. botulinum spores (environment)
tx: human-derived botulism Ig
IN CONTRAST, food-borne is from ingestion of preformed toxin, tx: equine-derived antitoxin
BOTH have descending flaccid paralysis

95
Q

LOC, post-octal state, transient hemiplegia (1-sided paralysis), think?

A

Todd paralysis
transient, focal weakness after focal/generalized seizure
r/o other causes with CT/MRI if no clear hx of preceding seizure

96
Q

most common brain tumor in kids

A

astrocytoma

97
Q
brain tumor presentations
supretentorial: 
posterior fossa:
brainstem:
SC:
A

supretentorial (astrocytoma, craniopharyngioma): ^ICP, seizures, sensory changes
posterior fossa (medulloblastoma, ependymoma): ^ICP, ataxia, clumsiness
brainstem: ataxia, clumsiness, CN palsies
SC: back pain, weakness, abnormal gait

98
Q

risk factors for IVH

A

premies, LBW

99
Q

if brief loss of consciousness, HA, and vomiting after head injury, what to do?

A

get head CT without contrast as it is a mild TBI

100
Q

cavernous hemangioma along trigeminal nerve distribution + intra-cranial calcifications that resemble a train-track

A

Sturge-Weber

101
Q

Uncoordinated and limited voluntary movements, think? What are the most common risk factors?

A

Cerebral palsy
Prematurity, intrauterine growth restriction, alcohol consumption, multiple gestation, tobacco use
Comorbidities: epilepsy strabismus scoliosis

102
Q

A two-year-old should have how many words in their vocabulary?

A

More than 50 and be able to combine them into two word phrases

103
Q

Delayed muscle relaxation, facial weakness, foot drop, dysphasia, cardiac conduction anomalies, cataracts, testicular atrophy, baldness, think?

A

Myotonic muscular dystrophy, autosomal dominant expansion of the CTG repeat
Later onset than other dystrophies, age 12 to 30

104
Q

Wilms tumor versus neuroblastoma origins

A

Wilms tumor: metanephros, precursor of the renal parenchyma

Neuroblastoma: neural crest cells, precursor of the sympathetic chains and adrenal medulla

105
Q

Wide-based, unsteady gait, weakness and lower limbs, decreased vibratory in position sense in the extremities, atrophy of the cervical spine accord, T-wave inversion’s on EKG, think?

A

Friedreich ataxia, autosomal recessive, GAA nucleotide repeats, leads to myocarditis and cardio myopathy, arrhythmias, CHF
Genetic counseling is recommended

106
Q

Amenorrhea, short stature, anosmia, delayed development, low FSH and LH, normal uterus and ovaries, think?

A

Kallmann syndrome

46, XX

107
Q

Tourette’s is associated with increased risk of?

Tourette’s treatment?

A

ADHD and OCD

Treatment: antipsychotics: Risperidone, alpha 2 adrenergic receptor agonist: clonidine, Guanfacine

108
Q

Gold standard for muscular dystrophy diagnosis

A

Genetic studies

Supportive diagnostic tests: elevated CK, aldolase; fibrosis and fatty infiltration on calf muscle biopsy

109
Q

Precocious puberty, pigmentation, polyostotic fibrous dysplasia think?

A

McCune Albright syndrome

Pigmentation: café au lait spots

110
Q

Edema in Turner’s syndrome is from?

A

Abnormal development of the lymphatic system

111
Q

Hypotonia, hyperphagia, obesity, think? What genetic abnormality?

A

Prader Willi syndrome, loss of paternal copy of 15q11-q13
Loss of maternal copy results in Angelman syndrome, (paternal uni parental disomy): short stature, intellectual disability, abnormal smiling/laughter, hand flapping, ataxia, seizures

112
Q

Ataxia, scoliosis, cardio myopathy, Think? Most common cause of death?

A

Friedrich ataxia, most common cause of death is cardiomyopathy or respiratory complications

113
Q

Risk factors for RDS

A

Prematurity, maternal diabetes, C-section

Hi levels of insulin antagonize cortisol and block surfactant production

114
Q

Diagnosis of minimal change disease

A

Not required, highly responsive to steroids

115
Q

Toddler with the firm, smooth, unilateral abdominal mass and hemateria, think?

A

Wilms tumor, the most common pediatric renal malignancy, peaks age 2 to 5, Does not cross midline
In contrast, neuroblastoma happens more commonly in the first year of life, affects the adrenal gland, crosses the midline and has systemic symptoms

116
Q

African-American boy with asymptomatic hemateria think?

A

Renal papillary necrosis due to Sickle cell trait

117
Q

If subcutaneous emphysema, get? To rule out?

A

Get chest x-ray to rule out pneumothorax

May occur secondary to severe coughing

118
Q

2 to 24 month old with febrile UTI, get? To evaluate?

If recurrent UTIs also get? To evaluate for?

A

Get Renal ultrasound to evaluate for anatomic abnormalities

If recurrent, get a VCUG to evaluate for vesicoureteral reflux

119
Q

First line medical treatment for enuresis if behavioral an alarm therapy fails

A

Desmopressin

TCAs: imipramine, are just as effective but have more serious side effects

120
Q

First steps If newborn with respiratory compromise and suspected diaphragmatic hernia

A

Endotracheal intubation, gastric tube to decompress the stomach and bowel
Bag mask ventilation can exacerbate respiratory decline
Get CXR when stable

121
Q

Nephrotic syndrome in kid with hepatitis B think?

A

Membranous nephropathy

122
Q

Complications of bronchiolitis

A

Apnea and respiratory failure

123
Q

CF kid with pneumonia, treated with Tamiflu, cefepime and ? for ?

A

Vancomycin for S. aureus, most common cause of bacterial pneumonia in young kids with cystic fibrosis especially with coexisting influenza
In contrast, Cipro would be appropriate for adults with CF to treat Pseudomonas

124
Q

Upper motor neuron findings in a kid with down syndrome, think?

A

Atlantoaxial instability

125
Q

Kawasaki disease is a vasculitis characterized by fever for at least five days and 4/5 of the following:

A

Conjunctivitis, extremity changes, cervical LAD, oral mucosal changes (strawberry tongue), rash

126
Q

Migratory Polyarthritis, pink rash with sharp edges,fever, elevated ESR, think?

A

Acute rheumatic fever

127
Q

Transient Cinnabay this versus Legg-Calve-Perthes disease

A

Transient synovitis should self resolve in 1 to 4 weeks

128
Q

Solitary, painful,lytic bone lesion plus Hypercalcemia in a kid, think?

A

Think neoplastic process: Langerhans cell histiocytosis

In contrast, hyperparathyroidism typically occurs in patients over 50

129
Q

Fever, rash, joint pain 1 to 2 weeks after exposure to penicillins or sulfa drugs, think?

A

Serum sickness like reaction
Type 3 hypersensitivity
Treatment: remove offending agent, supportive care, steroids if severe
In contrast, penicillin in the setting of EBV causes a rash that spares the extremities and does not have joint pain

130
Q

think compartment syndrome if?

A

Six P’s:

Pain, pallor, poikilothermia, paresthesias, and late findings of pulselessness, paralysis

131
Q

Isolated polyarthralgia in a kid, think?

Lab values?

A

Juvenile idiopathic arthritis

Elevated ESR, CRP, elevated ferritin, elevated IgG, elevated platelets, anemia