11.6.2013(pediatrics) 34

Question 0

Most common type of JIA

Answer 0

Oligoarticular arthritis(40%)

Question 1

Types of juvenile idiopathic arthritis

Answer 1

Systemic onset
Poly articular 
Oligoarticular
Juvenile psoriatic
Enthisitis

Question 2

Oligoarticular arthritis associated with iridocyclitis

Answer 2

Type 1

Only self limiting iritis occurs type 2

Question 3

ANA negative juvenile idiopathic arthritis

Answer 3

Type 2 oligoarticular arthritis

Question 4

HLA B27 associated JIA

Answer 4

Enthesitis related

Type 2 oligoarticular

Question 5

HLA associated with type 1 pauciarticular JIA

Answer 5

HLADR5

HLADRW8

Question 6

Ocular complications of pauciarticular type1 JIA

Answer 6

Glaucoma

Cataract

Question 7

No of Joints involved in polyarticular arthritis

Answer 7

> =5 joints

Question 8

Features of RF+ polyarticular JIA

Answer 8

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

Question 9

Features of systemic onset JIA

Answer 9

Fever 
Evanescent non pruritic rash
Lymphadenopathy
HepatoSplenomegaly
Serositis

Question 10

Lab values in systemic onset JIA

Answer 10

Anemia
Leukocytosis
Thrombocytosis
Elevated CRP,ESR
ferritin
Abnormal LFT

Question 11

HPE in neonatal PAP

Answer 11

PAS +

Diastase resistant

Question 12

Mutations causing neonatal PAP

Answer 12

SP-B
SP-C
GM-CSF
ABCA3

Question 13

Hyperammonemia with neonatal PAP

Answer 13

Lysinuric protein intolerance

Question 14

Occupational disease resembling PAP

Answer 14

Silicosis

Question 15

Gold standard for diagnosis of PAP in children

Answer 15

HPE examination of lung biopsy

Question 16

Rx of PAP in adults

Answer 16

Total lung lavage

Question 17

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

Answer 17

HIV positive women

Question 18

Risk of transmission of congenital toxoplasmosis

Answer 18

Directly proportional to gestational age

While damage caused is inversely proportional

Question 19

____________ % of infants with congenital toxoplasmosis are symptomatic

Answer 19

30

Question 20

Ocular manifestations of congenital toxoplasmosis

Answer 20

Chorioretinitis
Microphthalmia
Optic atrophy
Blindness
Icterus
Ocular palsies

Question 21

Salt and pepper retinopathy

Answer 21

Congenital rubella syndrome

Question 22

Common cardiac anomalies in CRS

Answer 22

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

Question 23

Measurement of IgG antibody against toxoplasma

Answer 23

Sabin Feldman dye test

Question 24

Prevention of transmission of toxoplasma

Answer 24

Rx of mother with spiramycin

Question 25

Most common perinatal infection in developed world

Answer 25

CMV

Question 26

Congenital varicella

Answer 26

``` Varicella embryopathy(in utero infection) Varicella infection of newborn(perinatal infection) ```

Question 27

Severe form of VON occurs if maternal infection occurs between

Answer 27

5 days prior and 2 days after delivery

Question 28

__________ infants are at high risk of postnatal varicella infection

Answer 28

Preterm | Maternal transfer of IgG occurs in last trimester

Question 29

Ocular manifestations of varicella embryopathy

Answer 29

``` Cataract Microphthalmia Horner syndrome Nystagmus Chorioretinitis ```

Question 30

Musculoskeletal manifestations of rubella embryopathy

Answer 30

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

Question 31

Complications of VON

Answer 31

``` Meningoencephalitis Pneumonia Reyes syndrome Thrombocytopenia Pericarditis,myocarditis,endocarditis Hepatitis Pancreatitis Glomerulonephritis Purpura fulminans Arthritis ```

Question 32

GIT manifestations of CF in newborn

Answer 32

Meconium illeus- small intestinal obstruction | Meconium plug syndrome- large intestinal obstruction

Question 33

Pulmonary complications of CF

Answer 33

``` Atelectasis Hemoptysis(bronchiectasis) Pneumothorax Clubbing Hypertrophic pulmonary osteoarthropathy Allergic aspergillosis Bacterial superinfection ```

Question 34

In CF intestinal obstruction occurs at

Answer 34

Distal illeum

Question 35

Liver manifestations of CF

Answer 35

Cholesterol stones | Billiary cirrhosis

Question 36

International prognostic index for lymphoma

Answer 36

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

Question 37

Children with VSD become symptomatic by

Answer 37

6-10wks of age

Question 38

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

Answer 38

Hypoplastic left heart syndrome

Question 39

Hypoplastic left heart syndrome

Answer 39

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

Question 80

Enzyme deficient in classic galactosemia

Answer 80

galactose -1- phosphate uridyl transferase

Question 81

Chr involved in classic galactosemia

Answer 81

Chr 9p

Question 82

Galactosemia doesn't affect testis/ovaries?

Answer 82

Testis | Ovaries- hypergonadotrophic hypogonadism

Question 83

Effect of galactosemia on kidneys

Answer 83

Fanconi syndrome

Question 86

Age below which JIA can be diagnosed

Answer 86

16 yrs

Question 87

Galactosemia causes __________ cirrhosis

Answer 87

Macronodular

Question 88

Type III galactosemia

Answer 88

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

Question 89

CT brain in congenital varicella infection

Answer 89

Cortical and cerebellar atrophy