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Pediatrics > UWorld > Flashcards

UWorld Flashcards

1
Q

loss of motor milestones, hypotonia, HSM, protuberant abdomen, areflexia, cherry red macula

A

niemann pick disease- sphingomyelinase deficiency

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2
Q

loss of motor milestones, hypotonia, cherry red macula, hyperreflexia, no HSM

A

tay sachs- beta hexosaminidase

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3
Q

early infancy: developmental regression, hypotonia, areflexia; no cherry red macula or organomegaly

A

krabbe disease- galactocerebrosidase

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4
Q

anemia, thrombocytopenia, HSM
no loss of milestones or cherry red macula
this is an IEM

A

gaucher disease- glucocerebrosidase deficiency

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5
Q

coarse facial features, inguinal or umbilical hernias, corneal clouding, HSM at 6 months-2years

A

hurler syndrome- mucopolysaccharidosis (lysosomal storage disorder)

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6
Q

how to dx hereditary spherocytosis

A

acidified glycerol lysis test

eosin-5-maleimide binding test

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7
Q

erythroderma

A

bright red patches coalesce and gradually peel

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8
Q

long arms and legs, sparse facial and body hair, gynecomastia, cryptorchidism, infertility

A

klinefelter’s (XXY)

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9
Q

rare but life threatening hepatic encephalopathy that develop in children using aspirin during influenza or varicella infections

A

reye syndrome

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10
Q

where do you see adenoma sebaceum

A

tuberous sclerosis

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11
Q

risk factors for RDS

A 
PREMATURITY
MALE SEX
PERINATAL ASPHYXIA
MATERNAL DIABETES
C SECTION WITHOUT LABOR
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12
Q

what heart issue do you see most often in trisomy 18 (Edwards)

A

VSD

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13
Q

what heart issue do you see with congenital rubella

A

PDA

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14
Q

normal baby at birth –> apathy, weakness, hypotonia, large tongue, sluggish movement, abdominal bloating, umbilical hernia

A

congenital hypothyroidism

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15
Q

mild coagulopathy, peripheral and cranial neuropathy, HSM, pancytopenia, partial oculocutaneous albinism, frequent bacterial infection

  • neutropenia, giant lysosomes in neutrophils
  • tx with daily bactrim and vitamin C
A

chediak-higashi syndrome

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16
Q

neutrophilia w/o PMNs in the infected tissue/pus

A

leukocyte adhesion defect

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17
Q

chronic pruritic dermatitis, recurrent staph skin and respiratory tract infections, elevated serum IgE, eosinophilia, coarse facial features

A

hyper-IgE (Job’s) syndrome

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18
Q

3 main causes of nephrotic syndrome

A

minimal change disease
FSGS- crescent formation
membranous glomerulonephritis- thickened basement membrane, subepithelial spikes

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19
Q

_____ hypercellularity is suggestive of membranoproliferative GN (nephritic syndrome)

A

mesangial

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20
Q

child with self mutilation and gout

A

lesch nyhan syndrome

deficiency in hypoxanthine-guanine phosphoribosyl transferase (HPRT)- involved in purine metabolism

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21
Q

hydrophobia and aerophobia

A

pathognomonic for rabies

feeling of water or air triggers involuntary pharyngeal muscle spasms

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22
Q

common cardiac issues in turners

A

bicuspid aortic valve
coarctation of aorta
aortic root dilation

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23
Q

how to dx DMD

A

genetic testing

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24
Q

how to tx high lead levels

A

mild: rpt levels in 1 month
moderate (45-70): DMSA
severe (>70 or encephalopathy): dimercaprol + EDTA

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25
Q

hematuria

isosthenuria- impairment in concentrating ability -> nocturia, polyuria

A

sickle cell trait

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26
Q

fever, urticarial rash, polyarthralgia, LAD 1-2 weeks after abx

A

serum sickness-like reaction

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27
Q

how to tx GC and chlamydial conjunctivitis

A

GC- IM cefotaxime

chlamydia- oral erythromycin

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28
Q

how to ppx GC conjunctivitis in newborns

A

topical erythromycin

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29
Q

blood stained eye discharge in a neonate is characteristic for ______

A

chlamydial conjunctivitis

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30
Q

apgar score < ____ requires further evaluation and resuscitation

A

7

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31
Q

APGAR- what is involved?

A 
appearance/color
pulse
grimace/reaction 
activity/muscle tone 
respiration
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32
Q

quantitative pilocarpine iontophoresis is a test for what

A

cystic fibrosis

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33
Q

strawberry vs. cherry hemangioma

which one do you find in kids?

A

strawberry

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34
Q 
shapes you can copy and at what age 
age 3
age 4
age 5
age 6
A

3- cross, circle
4- square, rectangle
5- triangle
6- diamond

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35
Q

dubin johnson causes _______ hyperbili

galactosemia causes ______ hyperbili

A

conjugated

conjugated

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36
Q

CATCH22 of ______

A 
DiGeorge
conotruncal cardiac defects
abnromal facies
thymic aplasia
cleft palate 
hypoCa
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37
Q

when you suspect DiGeorge, which two things do you order?

A

serum Ca

echo

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38
Q

pappenheimer bodies seen on smear suggest this dx

A

sideroblastic anemia

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39
Q

how to tx pinworm (enterobiasis)

A

albendazole or mebendazole

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40
Q

cirrhosis and periportal eosinophilic inclusion bodies in the liver

A

alpha 1 antitrypsin

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41
Q

microvesicular steatosis, elevated transaminases, coagulopathy, hyperammonemia

A

reye syndrome (ASA + flu or varicella in kids)

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42
Q

what is caroli syndrome

A

congenital disorder- intrahepatic dilation of bile ducts

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43
Q

by 12 months, a child’s weight should ______ and height should ______

A

triple

increase by 50%

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44
Q

gold standard for diagnosing malrotation

A

upper GI

-you would see ligament of treitz on the right side of the abdomen

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45
Q

upper GI finding of volvulus

A

corkscrew pattern

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46
Q

which 3 electrolytes are low in refeeding syndrome

A

K
Mg
phosphorus
also decreased thiamine

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47
Q

scaphoid/concave abdomen, barrel chest in newborn with respiratory distress

A

congenital diaphragmatic hernia

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48
Q

how to manage congenital diaphragmatic hernia

A

intubate

then gastric tube to decompress the stomach and bowel

49
Q

which vitamins should a premie baby who is exclusively breast feeding take?

A

iron

vitamin D

50
Q

testicles that haven’t descended by age _____ should be operated on

A

6 months

51
Q

2 causes of viral myocarditis

A

coxsackie B

adenovirus

52
Q

causes of intussusception

  • viral illness –> hypertrophy of peyers patches
  • inspissated stool
  • small bowel diverticulum
  • small bowel wall hematoma
A
  • kids < 2 years
  • CF kids
  • Meckel’s diverticulum** pretty common
  • HSP manifestation
53
Q

angular cheilosis, stomatitis, glossitis, normocytic anemia, seborrheic dermatitis

A

B2 riboflavin deficiency

54
Q

dermatitis, diarrhea, dementia, glossitis

A

B3 niacin deficiency

55
Q

cheilosis, stomatitis, glossitis, irritability, confusion, depression

A

B6 pyridoxine deficiency

56
Q

beriberi- peripheral neuropathy, heart failure

wernicke korsakoff

A

B1 thiamine deficiency

57
Q

classic triad: cataracts, deafness, cardiac defects

A

congenital rubella

58
Q

classic triad: chorioretinitis, hydrocephalus, intracranial calcifications

A

congenital toxo

59
Q

reasons to operate on an umbilical hernia

A 
persists to age 3-4 years
exceeds 2 cm in diameter
causes symptoms
becomes strangulated
enlarges progressively after age 1-2 years
60
Q

how to manage meconium ileus after you’ve excluded perf on xray

A

water soluble contrast enema

61
Q

how to manage ITP in kids

A

skin only: observe

bleeding: IVIG or steroids

62
Q

how to manage ITP in adults

A

plts > 30,000 w/o bleeding: observe

plts < 30,000 OR bleeding: IVIG or steroids

63
Q

HSM, hydrocephalus, chorioretinitis, intracranial calcifications

A

congenital toxo

64
Q

sensorineural hearing loss, cataracts, heart defects, HSM, microcephaly, thrombocytopenia purpura (blueberry muffin)

A

congenital rubella

65
Q

IUGR, HSM, petechiae or purpura, microcephaly, chorioretinitis, sensorineural hearing loss, periventricular calcifications

A

congenital CMV

66
Q

HSM, cutaneous lesions, jaundice, anemia, rhinorrhea in new born, metaphyseal dystrophy, periostitis

A

congenital syphilis

67
Q

fair complexion, intellectual disability, seizures, musty/mousy body odor
what is it and how to screen

A

PKU

  • newborn screen- tandem mass spec
  • quantitative AA analysis shows increased phenylalanine levels
68
Q

aldose B deficiency is what

A

hereditary fructose intolerance

69
Q

______ are the most common cause of urinary tract obstruction in newborn boys

A

posterior urethral valves

-cause oligohydramnios –> lung hypoplasia and Potter sequence

70
Q

grey colored pseudomembrane formation in the mouth of a unvaccinated child

A

diphtheria

71
Q

how to manage gastroschisis and omphalocele

A

wrap the bowel up
OG tube for decompression
get IV access
then surgery for definitive tx

72
Q

how to dx wilms tumor

A

abd ultrasound then CT

73
Q

______ (wilms tumor/neuroblastoma) crosses the midline

A

neuroblastoma crosses the midline

74
Q

which abx for sinusitis

A

augmentin

75
Q

how to tx long QT

A

propranol and pacemaker

76
Q

in infants with meningococcemia, watch out for this cause of mortality

A

waterhouse friderichsen sydrome

-sudden vasomotor collapse and skin rash due to adrenal hemorrhage

77
Q

______ deficiency presents with cataracts only

A

galactokinase

78
Q

newborn with FTT, bilateral cataracts, jaundice and hypoglycemia
what does it put you at risk for

A

galactosemia- galactose 1 phosphate uridyl transferase deficiency
E. coli neonatal sepsis

79
Q

low C1q levels are associated with ______ angioedema

A

acquired

hereditary angioedema is due to C1 inhibitor deficiency

80
Q

low IgM, high IgA and IgE

A

wiskott aldrich syndrome

81
Q

difficulty making facial expressions, dysphagia, ptosis, temporal wasting, thin cheeks, emaciated extremities, atrophy of thenar and hypothenar eminences, inverted V shaped upper lip, testicular atrophy, delayed relaxation on contraction of thenar and hypothenar muscles
what is it and how do you get it?

A

myotonic muscular dystrophy

-autosomal dominant

82
Q

inheritance of BMD and DMD

A

X linked recessive

83
Q

how to give shots to premies

A

via chronological age

only exception: weight should be > 2 kg for first hep B shot

84
Q

MCC sepsis in SS disease

A

strep pneumo

-vaccinate and give pcn ppx before age 5 years

85
Q

macrosomia, macroglossia, hemihyerplasia, umbilical hernia/omphalocele, hypoglycemia
what is it, what are you at risk for, what chromosome ?

A

beckwith wiedemann syndrome

  • risk for wilms tumor or hepatoblastoma
  • chromosome 11p15 (imprinted gene dysregulation)
86
Q

brick dust/red stains in neonatal diapers is ______

A

uric acid crystals- high at birth and commonly seen while mother’s milk is coming in

87
Q

normal neonates lose _____ of birth weight in the first 5 days

A

up to 7%

88
Q

follicular conjunctivitis and pannus (neovascularization) formation in the cornea is consistent with _____
how to dx and tx?

A

trachoma infection
dx with giemsa stain examination of conjunctival scrapings
tx with topical tetracycline or oral azithromycin

89
Q

_____ : gray vesicles on the tonsillar pillars and posterior oropharynx that progress to fibrin-coated ulcerations

_____: vesicles in the anterior oropharynx and lips

A

coxsackie causing herpangina –> supportive tx

HSV causing herpetic gingivostomatitis –> acyclovir

90
Q

umbilical stump infection, poor suckling and fatigue, rigidity, spasms, opisthotonus

A

neonatal tetanus

91
Q

bedwetting is normal before age _____

A

5

92
Q

patients with ______ are predisposed to anaphylactic transfusion reactions

A

IgA deficiency

93
Q

precocious puberty, cafe au lait spots (coast of maine), multiple bone defects

A

mccune albright

3 Ps: precocious puberty, pigmentation, polyostotic fibrous dysplasia

94
Q

how to tx TCA overdose

A

Na bicarb to correct acidosis and narrow the QRS complex

benzos for seizures

95
Q

PAS positive staining happens in this childhood leukemia

A

ALL

96
Q

how to tx eczema herpeticum

A

acyclovir

97
Q

small palpebral fissures
smooth philtrum
thin upper lip

A

fetal alcohol syndrome

98
Q 
flat facial profile
slanted palpebral fissures
small low set ears
single transverse palmar crease
clinodactyly 
large space between first two toes
A

Down syndrome

99
Q 
long narrow face
prominent forehead and chin
large ears
macrocephaly
marcoorchidism
A

fragile X

100
Q

hypoplastic fingers/nails and cleft lip/palate are commonly found in fetal _____ syndrome

A

hydantoin

  • phenytoin during pregnancy
  • ppx with vitamin K in 3rd trimester
101
Q

with HOCM, what increases murmur and what decreases it?

A

increases: valsalva, standing
decreases: hand grip, squatting and leg raise

102
Q

breathholding spell… what do you need to check?

A

CBC and serum ferritin b/c it may be assoc with iron deficiency anemia

103
Q

little girl who voids normally but is always wet with urine

A

low implantation of ureter

104
Q

teen boy who drinks for the first time and has flank pain

A

ureteropelvic junction obstruction

105
Q

if rapid strep is positive, then _____

if rapid strep is negative, then ______

A

treat without culture

get cx to r/o strep infection

106
Q

wiskott aldrich pts (thrombocytopenia, recurrent infections, eczema) associated with this malignancy

A

NHL

107
Q

if worried about child abuse with fx and bruises, what to do?

A

funduscopic exam

108
Q

basophilic stippling is seen in ____ and _______

A

thalassemia and lead poisoning

109
Q

FEP in lead poisoning

A

high

110
Q

abx against pseudomonas PNA in CF patient

A

ceftazidime and tobramycin (2 abx active against pseudomonas)

111
Q

burning pain, erythema, swelling minutes after being exposed to the sun
no blistering or scarring; thickened skin with erythema and swelling

A

erythropoietic protoporpyria

112
Q

rash spares the wrist

A

staph toxic shock

113
Q

chronic mucocutneous candidiasis, hypoparathyroidism, addison disease

A

polyglandular deficiency type I

114
Q

parathyroid adenomas with hypercalcemia, pancreatic islet cell tumors, pituitary adenomas

A

MEN I

115
Q

medullary carcinoma of thyroid, pheo, parathyroid adenomas

A

MEN II

116
Q

adrenal insufficiency, thyroid disease, DM

A

polyglandular deficiency syndrome type II

117
Q

Bruton’s agammaglobulinemia: what organisms might you be susceptible to?

A

h flu, strep pneumo, mycoplasma

118
Q

2 causes of secretory diarrhea in infants

A

rotavirus, ETEC

119
Q

most common non ocular tumor in kids with retinoblastoma

A

osteosarcoma

Pediatrics (14 decks)

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