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AIIMS > 13.6.2013(pediatrics) 36 > Flashcards

13.6.2013(pediatrics) 36 Flashcards

0
Q

Drug causing bronchiolitis obliterans

A

Penicillamine

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1
Q

Causes of bronchiolitis obliterans

A 
ADENO virus
Influenza
Measles
Mycoplasma
Pertussis
AIM MP
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2
Q

Connective tissue disorders causing bronchiolitis obliterans

A

SLE
RA
scleroderma

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3
Q

Inhalants causing bronchiolitis obliterans

A

HCl
Sulphur di oxide
NO

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4
Q

Causes of bronchiolitis obliterans

A 
Infections
Inhalants
Connective tissue disorder
Bronchopulmonary dysplasia
Bone marrow transplant 
GERD 
Penicillamine
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5
Q

Long term sequelae of bronchiolitis obliterans

A

Atelectasis
Bronchiectasis
Recurrent pneumonia
Swyer James syndrome

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6
Q

Swyer James syndrome

A

Unilateral hyperlucent lung syndrome

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7
Q

Atelectasis in bronchiolitis obliterans

A

RUL(young children)

LLL(older children)

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8
Q

Most common cause of bronchiolitis

A

RSV

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9
Q

Cause of acute coryza in children

A

Rhino virus

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10
Q

Cause of ALTB

A

Parainfluenza virus type 1

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11
Q

Cause of epiglottitis

A

H. Influenzae type B

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12
Q

Cause of bronchopneumonia <2 yrs

A

RSV

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13
Q

Cause of empyema in children <2yrs

A

Staphylococci

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14
Q

IOC in bronchiolitis obliterans

A

Lung biopsy

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15
Q

Antibiotic Rx in bronchiolitis

A

Ribavirin
If
CHF,chronic lung disease,immunodeficiency

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16
Q

Mab against RSV

A

Palivizumab

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17
Q

Follicular bronchitis

A

Rare in children
Fine crackles
Interstitial x ray pattern
restrictive PFT

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18
Q

Sandstorm appearance in chest X ray

A

Pulmonary alveolar microlithiasis

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19
Q

Features of croup

A

Barking cough worse at night
Hoarseness
Stridor

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20
Q

X ray in ALTB

A

Steeple sign

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21
Q

Rx of ALTB

A
  1. Dexamethasone

2. nebulised epinephrine

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22
Q

Most common cause of airway obstruction in children btw 6mon-6yrs

A

Croup

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23
Q

Stridor in epiglottitis

A

Aggravated in supine position

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24
Q

Rx of epiglottitis

A

Ceftriaxone
Ampicillin
Chloramphenicol

Dexamethasone and racemic epinephrine are contraindicated

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25
Q

Diagnosis of ADHD

A

Symptom onset before 12yrs of age

Must be present for atleast 6 months

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26
Q

Symptoms of ADHD are divided into

A

Inattention

Impulsivity/hyperactivity

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27
Q

First Line Rx of ADHD

A

Methylphenidate

Dextroamphetamine

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28
Q

Drugs used in ADHD

A

Methylphenidate
Dextroamphetamine
Atmoxetine(second line)

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29
Q

Rx of refractory histiocytosis

A 
Imatinib
Cladirabine
Cyclosporine 
Antithymocyte globulin
Stem cell transplantation
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30
Q

Rx of single system histiocytosis

A

Low dose radiotherapy or curettage

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31
Q

Rx of multi system histiocytosis

A

Etoposide

Vinblastine

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32
Q

Most common inherited childhood tumor

A

Retinoblastoma

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33
Q

TAR syndrome

A 
Thrombocytopenia
B/L absent or hypoplastic radii
aplastic or hypoplastic ulnae
Thumb is normal
TOF,ASD
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34
Q

Syndromes associated with radial abnormalities

A 
Holt oram
TAR
VATER
Fanconi Anemia 
Baller gerold syndrome
35
Q

AASE syndrome

A

Triphalangeal thumb
Radial hypoplasia
Hypoplastic Anemia
No thrombocytopenia

36
Q

Holt oram disease

A

Ostium secundum ASD
conduction defects
Absent,hypoplastic or triphalangeal thumb
Blood counts are normal

37
Q

Robert syndrome

A 
Thrombocytopenia
Microcephaly
MR
B/L symmetric tetraphocomelia
Oligodactyly with thumb aplasia or hypoplasia
38
Q

Congenital deficiency of erythroid precursors

A

AASE
diamond blackfan
Congenital dyserythropoietic Anemia

39
Q

Cytoclastic agents

A

Cause chromosomal breakage in fanconi Anemia
Mitomycin-C
Diepoxybutane

40
Q

Fanconi Anemia cells in the presence of clastogens arrest in

A

G2M phase

41
Q

Chromosomal breakage syndromes

A

Fanconi Anemia
Ataxia telangiectasia
Bloom
Xeroderma pigmentosum

42
Q

Specific leukodystrophies

A 
Metachromatic leukodystrophy
Krabbe
Canavan
Alexander
Adrenoleukodystrophy
Pelizaeus merzhacher disease
43
Q

Leukodystrophy associated with macrocephaly

A

CANAVAN
Alexander
Metachromatic leukodystrophy(enlarges late)

44
Q

Canavan disease,enzyme deficient

A

N-aspartoacylase

45
Q

Leukodystrophy with early onset

A

Canavan disease

46
Q

Enzyme deficient in adrenoleukodystrophy

A

Acyl coA synthetase

47
Q

X linked recessive leukodystrophy

A

Adrenoleukodystrophy
Pelizaeus merzhacher disease

All others are autosomal recessive

48
Q

Enzyme deficient in metachromatic leukodystrophy

A

Arylsulfatase A

49
Q

Enzyme deficient in krabbe leukodystrophy

A

Beta galactosidase

50
Q

Seizures are common in which leukodystrophy

A

Krabbe

51
Q

Leucodystrophy with thalamic hyperintensity and low density areas in white matter

A

Krabbe

52
Q

Metachromatic Leucodystrophy CT

A

Diffuse symmetric attenuation of cerebellar and cerebral white matter

53
Q

Leucodystrophy which occurs sporadically

A

Alexander

54
Q

Brain biopsy findings in Alexander disease

A

Rosenthal fibres

55
Q

Alexander disease is due to

A

Mutation in glial fibrillary acidic protein

56
Q

CT in Alexander disease

A

White matter degeneration more prominent in frontal lobe

57
Q

CT in Canavan disease

A

White matter degeneration in cerebral hemisphere

58
Q

Crumpled tissue paper appearance of macrophages

A

Gaucher

59
Q

Enzyme deficient in Farber disease

A

Ceraminidase

60
Q

Enzyme deficient in fabry disease

A

Alpha galactosidase

61
Q

Enzyme deficient in gaucher

A

Glucocerebrosidase(beta glucosidase)

62
Q

Most common lysosomal storage disease

A

Gaucher

63
Q

Types of gaucher

A

Type1(nonneuropathic)
Type2(acute infantile neuropathic)
Type3(subacute neuropathic)

64
Q

Most common type of gaucher

A

Type 1

65
Q

Most common symptom of gaucher

A

Splenomegaly

66
Q

Enzyme defect in hurler

A

Alpha-1- iduranidase

67
Q

Enzyme defect in hunter

A

Iduronate sulfatase

68
Q

Enzyme replacement in gaucher

A

Cerezyme

69
Q

Triad in neurologic gaucher

A

Retroflexion of head(Opisthotonos)
Trismus
Strabismus

STR

70
Q

X- ray of long bones in gaucher

A

Erlenmeyer flask deformity

Femur most commonly affected

71
Q

Defect in GLUT2

A

Fancon/ Bickel disease

72
Q

Type XI glycogen storage disease

A

Fancon/ Bickel

73
Q

Most common congenital anomaly in turner

A

Bicuspid aortic valve

74
Q

CVS anomalies in turner

A

1.Bicuspid aortic valve
2.Coarctation of aorta
MVP
TAPVD

75
Q

Unique features of trisomy 13

A 
Ectodermal scalp defects(cutis aplasia)
Hare lip
Cleft palate
Microphthalmia
Hypertelorism
Flexed fingers with polydactyly
76
Q

Unique features of Edward

A 
Elongated skull
Simple arches in all digits
Rocker bottom foot
Closed fists with index finger overlapping 3rd and 5th digit overlapping 4th
Micrognathia
77
Q

Triple test in down

A

Alphafetoprotein decreased
Beta hCG increased
Estradiol decreased

78
Q

Quadruple test in down

A

Inhibin A increased

79
Q

PAPPA

A

Pregnancy associated plasma protein

Levels decreased in down

80
Q

Screening test for down in first trimester

A

Combined test
PAPPA+HCG
Integerated test
Combined test+increased nuchal fold thickness

81
Q

Screening test for down in second trimester

A

Triple
Quadruple
Integrated

82
Q

Ductus venous blood flow

A

Forward biphasic pulsatile flow(normal)

In down it is abnormal

83
Q

USG markers of down in second trimester

A 
Choroid plexus papilloma
Absent nasal bone
Atrioventricular canal defect 
Duodenal atresia
Hyperechoic bowel 
Short femur
Pyelectasia(dilation of renal pelvis)
84
Q

USG marker of down in first trimester

A

Nuchal translucency
Abnormal ductal flow velocity
Absent nasal bone

AIIMS (13 decks)

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