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AIIMS > 11.6.2013(pediatrics) 34 > Flashcards

11.6.2013(pediatrics) 34 Flashcards

0
Q

Most common type of JIA

A

Oligoarticular arthritis(40%)

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1
Q

Types of juvenile idiopathic arthritis

A 
Systemic onset
Poly articular 
Oligoarticular
Juvenile psoriatic
Enthisitis
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2
Q

Oligoarticular arthritis associated with iridocyclitis

A

Type 1

Only self limiting iritis occurs type 2

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3
Q

ANA negative juvenile idiopathic arthritis

A

Type 2 oligoarticular arthritis

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4
Q

HLA B27 associated JIA

A

Enthesitis related

Type 2 oligoarticular

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5
Q

HLA associated with type 1 pauciarticular JIA

A

HLADR5

HLADRW8

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6
Q

Ocular complications of pauciarticular type1 JIA

A

Glaucoma

Cataract

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7
Q

No of Joints involved in polyarticular arthritis

A

> =5 joints

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8
Q

Features of RF+ polyarticular JIA

A 
Age of onset late
RF+
Rheumatoid nodules+
ANA+
Uveitis+
Symmetric arthritis
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9
Q

Features of systemic onset JIA

A 
Fever 
Evanescent non pruritic rash
Lymphadenopathy
HepatoSplenomegaly
Serositis
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10
Q

Lab values in systemic onset JIA

A 
Anemia
Leukocytosis
Thrombocytosis
Elevated CRP,ESR
ferritin
Abnormal LFT
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11
Q

HPE in neonatal PAP

A

PAS +

Diastase resistant

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12
Q

Mutations causing neonatal PAP

A

SP-B
SP-C
GM-CSF
ABCA3

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13
Q

Hyperammonemia with neonatal PAP

A

Lysinuric protein intolerance

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14
Q

Occupational disease resembling PAP

A

Silicosis

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15
Q

Gold standard for diagnosis of PAP in children

A

HPE examination of lung biopsy

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16
Q

Rx of PAP in adults

A

Total lung lavage

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17
Q

Recurrent maternal toxoplasma infection doesn’t cause infection in children except in

A

HIV positive women

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18
Q

Risk of transmission of congenital toxoplasmosis

A

Directly proportional to gestational age

While damage caused is inversely proportional

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19
Q

____________ % of infants with congenital toxoplasmosis are symptomatic

A

30

20
Q

Ocular manifestations of congenital toxoplasmosis

A 
Chorioretinitis
Microphthalmia
Optic atrophy
Blindness
Icterus
Ocular palsies
21
Q

Salt and pepper retinopathy

A

Congenital rubella syndrome

22
Q

Common cardiac anomalies in CRS

A
  1. PDA
  2. occlusion of right pulmonary artery
  3. occlusion of left pulmonary artery
  4. valvular pulmonic stenosis
23
Q

Measurement of IgG antibody against toxoplasma

A

Sabin Feldman dye test

24
Q

Prevention of transmission of toxoplasma

A

Rx of mother with spiramycin

25
Q

Most common perinatal infection in developed world

A

CMV

26
Q

Congenital varicella

A 
Varicella embryopathy(in utero infection)
Varicella infection of newborn(perinatal infection)
27
Q

Severe form of VON occurs if maternal infection occurs between

A

5 days prior and 2 days after delivery

28
Q

__________ infants are at high risk of postnatal varicella infection

A

Preterm

Maternal transfer of IgG occurs in last trimester

29
Q

Ocular manifestations of varicella embryopathy

A 
Cataract
Microphthalmia
Horner syndrome
Nystagmus
Chorioretinitis
30
Q

Musculoskeletal manifestations of rubella embryopathy

A

Cicatrical dermatomal skin lesions and scarring
Unilateral limb atrophy with scarring and paresis
Rudimentary digits

31
Q

Complications of VON

A 
Meningoencephalitis 
Pneumonia
Reyes syndrome
Thrombocytopenia
Pericarditis,myocarditis,endocarditis
Hepatitis
Pancreatitis
Glomerulonephritis 
Purpura fulminans
Arthritis
32
Q

GIT manifestations of CF in newborn

A

Meconium illeus- small intestinal obstruction

Meconium plug syndrome- large intestinal obstruction

33
Q

Pulmonary complications of CF

A 
Atelectasis 
Hemoptysis(bronchiectasis)
Pneumothorax
Clubbing
Hypertrophic pulmonary osteoarthropathy 
Allergic aspergillosis
Bacterial superinfection
34
Q

In CF intestinal obstruction occurs at

A

Distal illeum

35
Q

Liver manifestations of CF

A

Cholesterol stones

Billiary cirrhosis

36
Q

International prognostic index for lymphoma

A 
Age>60
Ann arbor stage III or IV
LDH level elevated
Extra nodal sites>1
Performance status

PELAS

37
Q

Children with VSD become symptomatic by

A

6-10wks of age

38
Q

A child is admitted on 7 days of life with shock and respiratory distress. He was discharged 2 days back healthy. Probable diagnosis?

A

Hypoplastic left heart syndrome

39
Q

Hypoplastic left heart syndrome

A

Atresia of mitral or aortic orifice
Hypoplasia of ascending aorta
Non functioning left ventricle

80
Q

Enzyme deficient in classic galactosemia

A

galactose -1- phosphate uridyl transferase

81
Q

Chr involved in classic galactosemia

A

Chr 9p

82
Q

Galactosemia doesn’t affect testis/ovaries?

A

Testis

Ovaries- hypergonadotrophic hypogonadism

83
Q

Effect of galactosemia on kidneys

A

Fanconi syndrome

86
Q

Age below which JIA can be diagnosed

A

16 yrs

87
Q

Galactosemia causes __________ cirrhosis

A

Macronodular

88
Q

Type III galactosemia

A

UDP hexose 4 epimerase deficiency
It can manifest in two forms depending on tissue distribution
Mild form: abnormality only in blood cells

89
Q

CT brain in congenital varicella infection

A

Cortical and cerebellar atrophy

AIIMS (13 decks)

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