pediatrics Flashcards

1
Q

patient with spiral fracture what to rule out

A

child abuse

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2
Q

Left to right shunt 3 D

A

ASD
VSD
PDA

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3
Q

shunt in non cyanotic conditions

A

left to right

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4
Q

shunt in cyanotic conditions

A

right to left

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5
Q

Apert’s syndrome(2)

A

cranial deformities

fusion of fingers and toes

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6
Q

syndrome with VSD

A
Apert
Down
cri du chat
trisomies 13
trisomies 18
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7
Q

auscultation of ASD

A

fixed split of s2

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8
Q

quid of eisenmenger syndrome(3)

A

left to right shunt causes
pulmonary hyper tension
shunt reversal

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9
Q

xray and EKG in ASD(2)

A

increase pulmonary vascular markings

right axis deviation

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10
Q

secondary HTA in child

A

coarctation of oarta

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11
Q

disease with systolic BP higher in the upper extremities greater in the right arm than in the left

A

coarctation of aorta

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12
Q

advanced cases of coarctation of aorta(2)

A

well developped upper body

lower extremity wasting

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13
Q

xray of coarctation of aorta(2)

A

3 sign

rib notching

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14
Q

what is life sustaining in transposition of the great vessels(2)

A

PDA

Septal defect

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15
Q

why PDA and septal defect allow in case of transposition of great vessels

A

allow mixing of pulmonary and systemic blood flow

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16
Q

could a baby survive with transposition of the great vessels without a PDA or septal defect

A

it’s incompatible with life

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17
Q

transposition of the great vessels

A

eggshaped silhouette

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18
Q

quid of eggshaped silhouette(2)

A

narrow heart base

absence of of the main pulmonary artery segment

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19
Q

right to left shunt causes 5T

A
tetralogy
transposition of great vesels
truncus arteriosus
tricuspid atresia
total pulmonary venous return
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20
Q

tetralogy of fallot PROVE

A

pulmonary stenosis
RVH
overridding aorta
VSD

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21
Q

the most common cyanotic heart disease in infancy

A

transposition

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22
Q

the most common cyanotic heart disease in childhood

A

tetralogy

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23
Q

rx of tetralogy and transposition

A

PGE1

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24
Q

disease with tetralogy of fallot(3)

A

down
cri du chat
trisomie 13 and 18

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25
Q

xray of tetralogy de fallot

A

bootshaped

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26
Q

DTAP(5)

A
2 mois
4 mois
6 mois
15-18 months
4-6 ans
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27
Q

HIB vaccination(4)

A

2 mois
4 mois
6 mois
12-15 months

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28
Q

IPV vaccination(4)(inactivated polio vaccine)

A

2 mois
4 mois
6 mois
4-6 ans

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29
Q

PPV vaccination(4)

A

2 mois
4 mois
6 mois
12-15 mois

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30
Q

MMR

A

12-15 months

4-6 ans

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31
Q

Varicella

A

12-15 months

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32
Q

HAV

A

2 ans

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33
Q

age to take influenza vaccine

A

6 months

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34
Q

enfant with evidence of neglect and severe malnourishment CAT

A

hospitalisation

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35
Q

management of pulmonary manifestation sin cystic fibrosis(5)

A
chest physical activity
bronchodilators
antiinflammatory agents
antibiotics
DNASE
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36
Q

digestive management of cystic fibrosis(2)

A

pancreatic enzymes

fat soluble vitamins ADEK

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37
Q

patau syndrome

A

trisomy 13

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38
Q

clue for turner

A

pas de corps de Barr

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39
Q

quid of fabry disease

A

@ glycosidase deficiency

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40
Q

consequence of @ glycosidase deficiency

A

ceramide trihexoside

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41
Q

finding of Fabry

A

renal failure

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42
Q

heritance of Fabry

A

x-linked recessive

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43
Q

quid of krabbe disease

A

absence of galactosylceramide B galactoside

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44
Q

consequence of Krabbe disease

A

galactocerebroside in the brain

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45
Q

symptom of Krabbe(3)

A

atrophy
spasticity
early death

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46
Q

inheritance of krabbe

A

autosomal recessive

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47
Q

quid of gaucher disease

A

deficiency of B glucocerebrosidase

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48
Q

consequence of gaucher(3)

A

accumulation of glucocerebroside in brain
liver and
bone marrow

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49
Q

gaucher cells

A

crinkled paper enlarged cytoplasm

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50
Q

type 1 of gaucher

A

incompatible with life

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51
Q

inheritance of gaucher

A

autosomal recessive

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52
Q

quid of Nieman pick disease(2)

A

Deficiency of sphingomyelinase
buildup of sphingomyelin
cholesterol in reticuloendothelial and parenchymal cells and tissues

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53
Q

prognosis of nieman pick

A

death by age of 3

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54
Q

inheritance of nieman pick

A

autosomal recessive

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55
Q

Tay-Sachs disease

A

Absence of hexosaminidase A

GM2 ganglioside accumulation

56
Q

Tay-Sachs disease prognosis

A

death by age of 3

57
Q

symptom of tay sachs

A

Cherry-red spot visible

on macula.

58
Q

people at risk Tay sachs disease

A

Carrier rate is 1 in 30 in Jews of European

descent (1 in 300 for others).

59
Q

metachromatic leukodystrophy

A

Deficiency of arylsulfatase A
accumulation of sulfatide in
the brain, kidney, liver, and peripheral nerves.

60
Q

inheritance of metachromatic leukodystrophy

A

Autosomal recessive

61
Q

target organ in metachromatic leukodystrophy(5)

A
accumulation of sulfatide in 
 the brain
kidney
liver
peripheral nerves.
62
Q

quid Hurler’s syndrome

A

Deficiency of α-L-iduronidase

63
Q

inheritance of hurler syndrome

A

Autosomal recessive.

64
Q

symptom of hurler(2)

A

corneal clouding

mental retardation

65
Q

quid Hunter’s syndrome

A

Hunter’s syndrome Deficiency of iduronate sulfatase

66
Q

hunter’s syndrome symptom(2)

A

mild form of hurler without corneal clouding

mild mental retardation

67
Q

transmission of hurler(2)

A

X-linked recesive

Hunters aim for the X.

68
Q

quid of lysosomal storage disease(8)

A
Hurler 
hunter
gaucher
krabbe
fabry
nieman pick
tay sachs
Metachromatic leukodystrophy
69
Q

most common cause of bowel obstruction in the first two years of life

A

intussuception

70
Q

quid of Digeorge syndrome Catch 22

A
CATCH-22
Congenital heart disease
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia
22q deletion
71
Q

B cell deficiency(3)

A

bruton(x linked agammaglobulinemia)
common variable immunodeficiency
Ig A deficiency

72
Q

the most common immunodeficiency

A

IGA deficiency

73
Q

common variable immunodeficiency clue

A

immunoglobulin levels drop at 20s et 30 s

74
Q

infectious risk in brutons

A

pseudomonas

75
Q

risk in common variable immunodeficiency

A

↑ pyogenic upper and lower respiratory infections

risk of lymphoma and autoimmune disease

76
Q

t cell immunodefiency

A

digeorge syndrome

77
Q

risk infectieux in digeorge(2)

A

PCP

fungi

78
Q

rx of digeorge(2)

A

marrow transplantation

Intraveinous immunoglobulin

79
Q

combined immunodeficiency(3)

A

Ataxia-telangiectasia
severe combined immunodeficiency
wiskott aldrich

80
Q

symptom in ataxia telangiectasia(2)

A

Oculocutaneous telangiectasias

cerebellar ataxia.

81
Q

physiopatho of ataxia telangiectasia

A

Caused by a DNA

repair defec

82
Q

risk in ataxia telangiectasia(2)

A

non-Hodgkin’s
lymphoma
gastric carcinoma

83
Q

Severe combined immunodeficiency

A

Severe lack of B and T cells

84
Q

risk in severe combined immunodeficiency(2)

A

opportunistic organism infection

PCP infection

85
Q

quid of wiskott aldrich(3)

A

eczema
low platelets
reccurent otitis media

86
Q

immunoglobulin in wiskott aldrich(2)

A

↑↑ IgE/IgA,

↓↓ IgM

87
Q

risk in wiskott aldrich(4)

A

atopic disorder
lymphoma
leukemia
infection

88
Q

bugs causing infection in wiskott

A

S pneumoniea
S aureus
H influenza type B

89
Q

Phagocytic problem causing immunodeficiency(2)

A

CGD

chediak higashi

90
Q

inheritance of CGD

A

x linked

91
Q

risk in CGD(3)

A

Chronic pulmonary, GI, and urinary infection
osteomyelitis
hepatitis

92
Q

bugs causing infection in CGD

A

catalase + organism

93
Q

dx test for CGD

A

Nitroblue tetrazolium test

94
Q

problem in chediak higashi

A

defect in neutrophils chemotaxis

95
Q

syndrome in chediak(4)

A

oculocutaneous
albinism,
neuropathy
neutropenia

96
Q

bugs in chediak higashi(3)

A

S. pyogenes,
S. aureus,
Pseudomonas spp

97
Q

complement deficiency(2)

A

C1 esterase deficiency( angioneurotic hereditary edema)

terminal complement deficiency (c5-c9)

98
Q

inheritance of chediak

A

autosomal recessive

99
Q

symtom in c1 esterase deficiency

A

recurrent episodes of angioedema in stress and trauma

100
Q

inheritance of C1 esterase deficiency

A

autosomal dominant

101
Q

complication of C1 esterase inhibitor

A

airway edema

102
Q

dx of C1 esterase deficiency

A

total hemolytic complement( CH50)

103
Q

prophylactic rx in c1 esterase deficiency

A

danazol

104
Q

problem in terminal complement deficiency

A

inability to form membrane attack complex

105
Q

consequence of terminal complement deficiency(4)

A

recurrent meningocal
gonoccocal infection
lupus
glomerulonephritis

106
Q

Mesure a prendre in case of terminal complement deficiency

A

meningoccal vaccines

107
Q

Kawasaki disease

symptoms—CRASH AND BURN”

A
Kawasaki disease
symptoms—
“CRASH AND BURN”
Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hands and feet (red,
swollen, flaky skin)
AND
Burn = fever > 40°C for
≥ 5 days
108
Q

fisr cause of bronchiolitis

A

RSV

109
Q

criteria to hospitalize brochiloitis(9)

A
marked respiratory distress, 
O2 saturation of
< 92%, 
toxic appearance, 
dehydration/poor oral feeding, 
a history of prematurity (< 34 weeks), 
age < 3 months, 
underlying cardiopulmonary disease
 unreliable parents.
110
Q

RSV prophylaxis

A

poly or monoclonal antibodies

111
Q

example of poli or mono clonal antbodies(2)

A

respigam

synagis

112
Q

first bug involved in croup

A

PIV 1(para influenza type 1)

113
Q

bugs causing tracheitis

A

s aureus

114
Q

xray in croup

A

“Steeple sign” on AP film

115
Q

xray in epiglotitis

A

“Thumbprint sign” on lateral film

116
Q

xray in tracheitis

A

Subglottic narrowing

117
Q

indication of racemic epinephrine

A

croup

118
Q

what to not do in epiglotitis and why

A

throat examination

laryngospasm and airway obstruction

119
Q

bug in epiglotitis

A

h influenza

120
Q

quid of direct hyperbilirubinemia

A

always pathologic in baby

121
Q

quid of erythma infectyosum 5th disease(2)

A

slapped cheek

worse with fever and sun exposure

122
Q

bug causing erythema infectyosum

A

parvovirus B19

123
Q

bugs causing roseola infantum

A

HHV-6

124
Q

rash in roseola infantum(3)

A
Maculopapular rash appears as fever
breaks 
begins on the trunk and quickly
spreads to the face and extremities
last< 24 h
125
Q

tear drop vesicules

A

varicella

126
Q

bug causing hand fot and mouth disease

A

cocksakie A

127
Q

rash in hand foot mouth syndrome

A

Oral ulcers
maculopapular vesicular rash
on hands feet and sometimes buttocks

128
Q

associated defect with esophageal atresia(VACTERL)

A
VACTERL
Vertebral 
cardiac
anal
tracheal 
esophageal
renal 
limb
129
Q

is gastrocisis emergent

A

surgical emergency

130
Q

rule of 2 for meckel(4)

A

2 times as many males affected;
2 feet from the ileocecal valve (most common);
2 types of mucosa (gastric, pancreatic)
2% of people affected

131
Q

failure to pass meconium in the first 24 hours of life

A

hirshprung disease

132
Q

jaundice in neonate witn abnormal vital signs

A

workup for sepsis

133
Q

Patients with complex febrile may require what?

A

chronic

anticonvulsant therapy.

134
Q

Top childhood

cancers:(4)

A
  1. Leukemia (ALL)
  2. CNS tumors
  3. Lymphoma
  4. Neuroblastoma
135
Q

association of wilms tumor(2)

A

aniridia

hemihypertrophy