Possibly EMQ

Question 1

What is congenital nephrotic syndrome?

Answer 1

Autosomal recessive disorder characterized by:

Dilatation of proximal convoluted tubule (microcytic disease).
Mesangial proliferation.
Sclerosis.

Question 2

When does NS develop in congenital NS:

Answer 2

In the first 3 months of age but has proteinuria at birth

Question 3

What are the Cxs of CNS?

Answer 3

Prematurity.
Resp. distress syndrome.
Large placenta.
Separation of cranial suture.
Persistent edema and 
Infection.
Death by 5years due – to infection and renal failure

Question 4

What is the tx of CNS?

Answer 4

Corticosteroid and immune suppression drug is of no value.

Captopril and indomethacin or unilateral nephrectomy may decrease proteinuria and ameliorate Nephrotic syndrome state.