pediatrics Flashcards

(135 cards)

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
xray of tetralogy de fallot
bootshaped
26
DTAP(5)
``` 2 mois 4 mois 6 mois 15-18 months 4-6 ans ```
27
HIB vaccination(4)
2 mois 4 mois 6 mois 12-15 months
28
IPV vaccination(4)(inactivated polio vaccine)
2 mois 4 mois 6 mois 4-6 ans
29
PPV vaccination(4)
2 mois 4 mois 6 mois 12-15 mois
30
MMR
12-15 months | 4-6 ans
31
Varicella
12-15 months
32
HAV
2 ans
33
age to take influenza vaccine
6 months
34
enfant with evidence of neglect and severe malnourishment CAT
hospitalisation
35
management of pulmonary manifestation sin cystic fibrosis(5)
``` chest physical activity bronchodilators antiinflammatory agents antibiotics DNASE ```
36
digestive management of cystic fibrosis(2)
pancreatic enzymes | fat soluble vitamins ADEK
37
patau syndrome
trisomy 13
38
clue for turner
pas de corps de Barr
39
quid of fabry disease
@ glycosidase deficiency
40
consequence of @ glycosidase deficiency
ceramide trihexoside
41
finding of Fabry
renal failure
42
heritance of Fabry
x-linked recessive
43
quid of krabbe disease
absence of galactosylceramide B galactoside
44
consequence of Krabbe disease
galactocerebroside in the brain
45
symptom of Krabbe(3)
atrophy spasticity early death
46
inheritance of krabbe
autosomal recessive
47
quid of gaucher disease
deficiency of B glucocerebrosidase
48
consequence of gaucher(3)
accumulation of glucocerebroside in brain liver and bone marrow
49
gaucher cells
crinkled paper enlarged cytoplasm
50
type 1 of gaucher
incompatible with life
51
inheritance of gaucher
autosomal recessive
52
quid of Nieman pick disease(2)
Deficiency of sphingomyelinase buildup of sphingomyelin cholesterol in reticuloendothelial and parenchymal cells and tissues
53
prognosis of nieman pick
death by age of 3
54
inheritance of nieman pick
autosomal recessive
55
Tay-Sachs disease
Absence of hexosaminidase A | GM2 ganglioside accumulation
56
Tay-Sachs disease prognosis
death by age of 3
57
symptom of tay sachs
Cherry-red spot visible | on macula.
58
people at risk Tay sachs disease
Carrier rate is 1 in 30 in Jews of European | descent (1 in 300 for others).
59
metachromatic leukodystrophy
Deficiency of arylsulfatase A accumulation of sulfatide in the brain, kidney, liver, and peripheral nerves.
60
inheritance of metachromatic leukodystrophy
Autosomal recessive
61
target organ in metachromatic leukodystrophy(5)
``` accumulation of sulfatide in the brain kidney liver peripheral nerves. ```
62
quid Hurler’s syndrome
Deficiency of α-L-iduronidase
63
inheritance of hurler syndrome
Autosomal recessive.
64
symptom of hurler(2)
corneal clouding | mental retardation
65
quid Hunter’s syndrome
Hunter’s syndrome Deficiency of iduronate sulfatase
66
hunter's syndrome symptom(2)
mild form of hurler without corneal clouding | mild mental retardation
67
transmission of hurler(2)
X-linked recesive | Hunters aim for the X.
68
quid of lysosomal storage disease(8)
``` Hurler hunter gaucher krabbe fabry nieman pick tay sachs Metachromatic leukodystrophy ```
69
most common cause of bowel obstruction in the first two years of life
intussuception
70
quid of Digeorge syndrome Catch 22
``` CATCH-22 Congenital heart disease Abnormal facies Thymic aplasia Cleft palate Hypocalcemia 22q deletion ```
71
B cell deficiency(3)
bruton(x linked agammaglobulinemia) common variable immunodeficiency Ig A deficiency
72
the most common immunodeficiency
IGA deficiency
73
common variable immunodeficiency clue
immunoglobulin levels drop at 20s et 30 s
74
infectious risk in brutons
pseudomonas
75
risk in common variable immunodeficiency
↑ pyogenic upper and lower respiratory infections | risk of lymphoma and autoimmune disease
76
t cell immunodefiency
digeorge syndrome
77
risk infectieux in digeorge(2)
PCP | fungi
78
rx of digeorge(2)
marrow transplantation | Intraveinous immunoglobulin
79
combined immunodeficiency(3)
Ataxia-telangiectasia severe combined immunodeficiency wiskott aldrich
80
symptom in ataxia telangiectasia(2)
Oculocutaneous telangiectasias | cerebellar ataxia.
81
physiopatho of ataxia telangiectasia
Caused by a DNA | repair defec
82
risk in ataxia telangiectasia(2)
non-Hodgkin’s lymphoma gastric carcinoma
83
Severe combined immunodeficiency
Severe lack of B and T cells
84
risk in severe combined immunodeficiency(2)
opportunistic organism infection | PCP infection
85
quid of wiskott aldrich(3)
eczema low platelets reccurent otitis media
86
immunoglobulin in wiskott aldrich(2)
↑↑ IgE/IgA, | ↓↓ IgM
87
risk in wiskott aldrich(4)
atopic disorder lymphoma leukemia infection
88
bugs causing infection in wiskott
S pneumoniea S aureus H influenza type B
89
Phagocytic problem causing immunodeficiency(2)
CGD | chediak higashi
90
inheritance of CGD
x linked
91
risk in CGD(3)
Chronic pulmonary, GI, and urinary infection osteomyelitis hepatitis
92
bugs causing infection in CGD
catalase + organism
93
dx test for CGD
Nitroblue tetrazolium test
94
problem in chediak higashi
defect in neutrophils chemotaxis
95
syndrome in chediak(4)
oculocutaneous albinism, neuropathy neutropenia
96
bugs in chediak higashi(3)
S. pyogenes, S. aureus, Pseudomonas spp
97
complement deficiency(2)
C1 esterase deficiency( angioneurotic hereditary edema) | terminal complement deficiency (c5-c9)
98
inheritance of chediak
autosomal recessive
99
symtom in c1 esterase deficiency
recurrent episodes of angioedema in stress and trauma
100
inheritance of C1 esterase deficiency
autosomal dominant
101
complication of C1 esterase inhibitor
airway edema
102
dx of C1 esterase deficiency
total hemolytic complement( CH50)
103
prophylactic rx in c1 esterase deficiency
danazol
104
problem in terminal complement deficiency
inability to form membrane attack complex
105
consequence of terminal complement deficiency(4)
recurrent meningocal gonoccocal infection lupus glomerulonephritis
106
Mesure a prendre in case of terminal complement deficiency
meningoccal vaccines
107
Kawasaki disease | symptoms—CRASH AND BURN”
``` 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
fisr cause of bronchiolitis
RSV
109
criteria to hospitalize brochiloitis(9)
``` 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
RSV prophylaxis
poly or monoclonal antibodies
111
example of poli or mono clonal antbodies(2)
respigam | synagis
112
first bug involved in croup
PIV 1(para influenza type 1)
113
bugs causing tracheitis
s aureus
114
xray in croup
“Steeple sign” on AP film
115
xray in epiglotitis
“Thumbprint sign” on lateral film
116
xray in tracheitis
Subglottic narrowing
117
indication of racemic epinephrine
croup
118
what to not do in epiglotitis and why
throat examination | laryngospasm and airway obstruction
119
bug in epiglotitis
h influenza
120
quid of direct hyperbilirubinemia
always pathologic in baby
121
quid of erythma infectyosum 5th disease(2)
slapped cheek | worse with fever and sun exposure
122
bug causing erythema infectyosum
parvovirus B19
123
bugs causing roseola infantum
HHV-6
124
rash in roseola infantum(3)
``` Maculopapular rash appears as fever breaks begins on the trunk and quickly spreads to the face and extremities last< 24 h ```
125
tear drop vesicules
varicella
126
bug causing hand fot and mouth disease
cocksakie A
127
rash in hand foot mouth syndrome
Oral ulcers maculopapular vesicular rash on hands feet and sometimes buttocks
128
associated defect with esophageal atresia(VACTERL)
``` VACTERL Vertebral cardiac anal tracheal esophageal renal limb ```
129
is gastrocisis emergent
surgical emergency
130
rule of 2 for meckel(4)
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
failure to pass meconium in the first 24 hours of life
hirshprung disease
132
jaundice in neonate witn abnormal vital signs
workup for sepsis
133
Patients with complex febrile may require what?
chronic | anticonvulsant therapy.
134
Top childhood | cancers:(4)
1. Leukemia (ALL) 2. CNS tumors 3. Lymphoma 4. Neuroblastoma
135
association of wilms tumor(2)
aniridia | hemihypertrophy