13.6.2013(pediatrics) 36 Flashcards
0
Q
Drug causing bronchiolitis obliterans
A
Penicillamine
1
Q
Causes of bronchiolitis obliterans
A
ADENO virus Influenza Measles Mycoplasma Pertussis AIM MP
2
Q
Connective tissue disorders causing bronchiolitis obliterans
A
SLE
RA
scleroderma
3
Q
Inhalants causing bronchiolitis obliterans
A
HCl
Sulphur di oxide
NO
4
Q
Causes of bronchiolitis obliterans
A
Infections Inhalants Connective tissue disorder Bronchopulmonary dysplasia Bone marrow transplant GERD Penicillamine
5
Q
Long term sequelae of bronchiolitis obliterans
A
Atelectasis
Bronchiectasis
Recurrent pneumonia
Swyer James syndrome
6
Q
Swyer James syndrome
A
Unilateral hyperlucent lung syndrome
7
Q
Atelectasis in bronchiolitis obliterans
A
RUL(young children)
LLL(older children)
8
Q
Most common cause of bronchiolitis
A
RSV
9
Q
Cause of acute coryza in children
A
Rhino virus
10
Q
Cause of ALTB
A
Parainfluenza virus type 1
11
Q
Cause of epiglottitis
A
H. Influenzae type B
12
Q
Cause of bronchopneumonia <2 yrs
A
RSV
13
Q
Cause of empyema in children <2yrs
A
Staphylococci
14
Q
IOC in bronchiolitis obliterans
A
Lung biopsy
15
Q
Antibiotic Rx in bronchiolitis
A
Ribavirin
If
CHF,chronic lung disease,immunodeficiency
16
Q
Mab against RSV
A
Palivizumab
17
Q
Follicular bronchitis
A
Rare in children
Fine crackles
Interstitial x ray pattern
restrictive PFT
18
Q
Sandstorm appearance in chest X ray
A
Pulmonary alveolar microlithiasis
19
Q
Features of croup
A
Barking cough worse at night
Hoarseness
Stridor
20
Q
X ray in ALTB
A
Steeple sign
21
Q
Rx of ALTB
A
- Dexamethasone
2. nebulised epinephrine
22
Q
Most common cause of airway obstruction in children btw 6mon-6yrs
A
Croup
23
Q
Stridor in epiglottitis
A
Aggravated in supine position
24
Rx of epiglottitis
Ceftriaxone
Ampicillin
Chloramphenicol
Dexamethasone and racemic epinephrine are contraindicated
25
Diagnosis of ADHD
Symptom onset before 12yrs of age
| Must be present for atleast 6 months
26
Symptoms of ADHD are divided into
Inattention
| Impulsivity/hyperactivity
27
First Line Rx of ADHD
Methylphenidate
| Dextroamphetamine
28
Drugs used in ADHD
Methylphenidate
Dextroamphetamine
Atmoxetine(second line)
29
Rx of refractory histiocytosis
```
Imatinib
Cladirabine
Cyclosporine
Antithymocyte globulin
Stem cell transplantation
```
30
Rx of single system histiocytosis
Low dose radiotherapy or curettage
31
Rx of multi system histiocytosis
Etoposide
| Vinblastine
32
Most common inherited childhood tumor
Retinoblastoma
33
TAR syndrome
```
Thrombocytopenia
B/L absent or hypoplastic radii
aplastic or hypoplastic ulnae
Thumb is normal
TOF,ASD
```
34
Syndromes associated with radial abnormalities
```
Holt oram
TAR
VATER
Fanconi Anemia
Baller gerold syndrome
```
35
AASE syndrome
Triphalangeal thumb
Radial hypoplasia
Hypoplastic Anemia
No thrombocytopenia
36
Holt oram disease
Ostium secundum ASD
conduction defects
Absent,hypoplastic or triphalangeal thumb
Blood counts are normal
37
Robert syndrome
```
Thrombocytopenia
Microcephaly
MR
B/L symmetric tetraphocomelia
Oligodactyly with thumb aplasia or hypoplasia
```
38
Congenital deficiency of erythroid precursors
AASE
diamond blackfan
Congenital dyserythropoietic Anemia
39
Cytoclastic agents
Cause chromosomal breakage in fanconi Anemia
Mitomycin-C
Diepoxybutane
40
Fanconi Anemia cells in the presence of clastogens arrest in
G2M phase
41
Chromosomal breakage syndromes
Fanconi Anemia
Ataxia telangiectasia
Bloom
Xeroderma pigmentosum
42
Specific leukodystrophies
```
Metachromatic leukodystrophy
Krabbe
Canavan
Alexander
Adrenoleukodystrophy
Pelizaeus merzhacher disease
```
43
Leukodystrophy associated with macrocephaly
CANAVAN
Alexander
Metachromatic leukodystrophy(enlarges late)
44
Canavan disease,enzyme deficient
N-aspartoacylase
45
Leukodystrophy with early onset
Canavan disease
46
Enzyme deficient in adrenoleukodystrophy
Acyl coA synthetase
47
X linked recessive leukodystrophy
Adrenoleukodystrophy
Pelizaeus merzhacher disease
All others are autosomal recessive
48
Enzyme deficient in metachromatic leukodystrophy
Arylsulfatase A
49
Enzyme deficient in krabbe leukodystrophy
Beta galactosidase
50
Seizures are common in which leukodystrophy
Krabbe
51
Leucodystrophy with thalamic hyperintensity and low density areas in white matter
Krabbe
52
Metachromatic Leucodystrophy CT
Diffuse symmetric attenuation of cerebellar and cerebral white matter
53
Leucodystrophy which occurs sporadically
Alexander
54
Brain biopsy findings in Alexander disease
Rosenthal fibres
55
Alexander disease is due to
Mutation in glial fibrillary acidic protein
56
CT in Alexander disease
White matter degeneration more prominent in frontal lobe
57
CT in Canavan disease
White matter degeneration in cerebral hemisphere
58
Crumpled tissue paper appearance of macrophages
Gaucher
59
Enzyme deficient in Farber disease
Ceraminidase
60
Enzyme deficient in fabry disease
Alpha galactosidase
61
Enzyme deficient in gaucher
Glucocerebrosidase(beta glucosidase)
62
Most common lysosomal storage disease
Gaucher
63
Types of gaucher
Type1(nonneuropathic)
Type2(acute infantile neuropathic)
Type3(subacute neuropathic)
64
Most common type of gaucher
Type 1
65
Most common symptom of gaucher
Splenomegaly
66
Enzyme defect in hurler
Alpha-1- iduranidase
67
Enzyme defect in hunter
Iduronate sulfatase
68
Enzyme replacement in gaucher
Cerezyme
69
Triad in neurologic gaucher
Retroflexion of head(Opisthotonos)
Trismus
Strabismus
STR
70
X- ray of long bones in gaucher
Erlenmeyer flask deformity
Femur most commonly affected
71
Defect in GLUT2
Fancon/ Bickel disease
72
Type XI glycogen storage disease
Fancon/ Bickel
73
Most common congenital anomaly in turner
Bicuspid aortic valve
74
CVS anomalies in turner
1.Bicuspid aortic valve
2.Coarctation of aorta
MVP
TAPVD
75
Unique features of trisomy 13
```
Ectodermal scalp defects(cutis aplasia)
Hare lip
Cleft palate
Microphthalmia
Hypertelorism
Flexed fingers with polydactyly
```
76
Unique features of Edward
```
Elongated skull
Simple arches in all digits
Rocker bottom foot
Closed fists with index finger overlapping 3rd and 5th digit overlapping 4th
Micrognathia
```
77
Triple test in down
Alphafetoprotein decreased
Beta hCG increased
Estradiol decreased
78
Quadruple test in down
Inhibin A increased
79
PAPPA
Pregnancy associated plasma protein
| Levels decreased in down
80
Screening test for down in first trimester
Combined test
PAPPA+HCG
Integerated test
Combined test+increased nuchal fold thickness
81
Screening test for down in second trimester
Triple
Quadruple
Integrated
82
Ductus venous blood flow
Forward biphasic pulsatile flow(normal)
| In down it is abnormal
83
USG markers of down in second trimester
```
Choroid plexus papilloma
Absent nasal bone
Atrioventricular canal defect
Duodenal atresia
Hyperechoic bowel
Short femur
Pyelectasia(dilation of renal pelvis)
```
84
USG marker of down in first trimester
Nuchal translucency
Abnormal ductal flow velocity
Absent nasal bone
