1q: Nephrotic syndrome is how many times more common in children than adults ?
a. 5 times
b. 10 times
c. 15 times
d. 20 times
2q: what is the incidence of nephrotic syndrome ?
a. 2-3/100000 children per year
b. 20-30/100000 children per year
c. 200-300/100000 children per year
d. 2000-3000/100000 children per year
3q: The characteristic features of nephrotic syndrome are ?
a. Heavy proteinuria ( greater than 3.5 g/24 hr in adults or 40 mg/sq.m/hr in children )
b. Hypoalbunemia ( less than 2.5 g/dL )
c. Edema
d. Hyperlipidemia
e. All the above
4q: Which of the following statements about nephrotic syndrome are true ?
a. Most children ( 90 % ) with nephrotic syndrome have a form of idiopathic nephrotic syndrome
b. Minimal change disease ( 85 % ) is the most common cause of idiopathic nephrotic syndrome
c. Focal segmental glomerulo sclerosis ( 10 % ) and mesangial proliferation ( 5 % ) are the next common causes of idiopathic nephrotic syndrome
d. The remaining 10 % who do not have idiopathic NS have secondary NS related to systemic or glomerular diseases such as membranous nephropathy or membranoproliferative glomerulonephritis
e. All the above statements are true
5q: Which of the following causes nephrotic syndrome which is steroid responsive ?
a. Minimal change nephrotic syndrome
b. FSGS
c. Denys-drash syndrome with diffuse mesangial sclerosis
d. Nail-patella syndrome
e. Finnish type of congenital NS
6q: All of the following are true about nephrin except ?
a. It is a product of the gene NPHS1
b. Location of the gene is on 1q25
c. The inheritance is of recessive type
d. A defect in the production of nephrin causes finnish type of congenital nephrotic syndrome
e. The NS produced by nephrin defect is steroid resistant
7q: Alpha actinin-4 is a product of which gene ?
a. NPHS1
b. NPHS2
c. FSGS1
d. FSGS2
e. WT1
8q: All of the following are true about podocin except ?
a. It is product of the gene LMX1B
b. Inheritance is of recessive type
c. A defect in podocin production is associated with FSGS
d. Gene location is on chromosome 1q25
9q: gene for alpha-actinin-4 ( FSGS1 ) is located on which chromosome ?
a. 19q13
b. 1q25
c. 2q35
d. 9q34
10q: What is the renal disease causing steroid resistant nephrotic syndrome associated with the defect in gene LMX1B which produces the protein LIM-homeodomain ?
a. Denys-Drash syndrome with diffuse mesangial sclerosis
b. Nail patella syndrome
c. FSGS
d. MCNS
11q: In the nephrotic state , serum lipid levels ( cholesterol, triglycerides ) are elevated for the following reason ?
a. Hypoalbuminemia stimulates generalized hepatic protein synthesis, including synthesis of lipoproteins
b. Lipid catabolism is diminished, as a result of reduced plasma levels of lipoprotein lipase, related to increased urinary losses of this enzyme
c. Both a and b
d. None
12q: How much percentage of children with Minimal Change Nephrotic Syndrome respond to corticosteroid therapy ?
a. 95 %
b. 50 %
c. 20 %
d. 5 %
13q: Electron microscopy of a patient with FSGS shows segmental scarring of the glomerular tuft with obliteration of the glomerular capillary lumen . A similar lesion may be seen with ?
a. HIV infection
b. Vesicoureteric reflux
c. Intravenous heroin abuse
d. All the above
14q: All of the following are true about FSGS associated nephrotic syndrome except ?
a. FSGS involves 85 % of all the cases presenting with idiopathic NS
b. 20 % of these cases respond to prednisone
c. Immunofluorence microscopy shows IgM and C3 staining in areas of segmental sclerosis
d. Electron microscopy shows segmental scarring of the glomerula tuft with obliteration of the glomerular capillary lumen
e. FSGS is frequently progressive, ultimately involving all glomeruli , and leads to end-stage renal disease in most patients
15q: Which of the following are uncommon findings in a patient with idiopathic nephrotic syndrome ?
a. Abdominal pain
b. Diarrhea
c. Anorexia and Irritability
d. Hypertension
e. Gross hematuria
16q: what is the differential diagnosis of a child with marked edema ?
a. Protein-losing enteropathy
b. Hepatic failure
c. Congestive heart failure
d. Acute or chronic glomerulonephritis
e. Protein malnutrition
f. All the above
17q: All of the following are true about idiopathic nephrotic syndrome except ?
a. More common in females than in males ( 2:1 )
b. Most commonly appears between the ages of 2 and 6 years
c. MCNS is present in 85-90 % of patients of less than 6 years of age
d. FSGS develops in older children
e. 20 -30 % of adolescents have MCNS
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