Question of the Week

Question :

Posted On : 24 Aug 2007

Can you please tell me the different modalities of treatment available for congenital nephrotic syndrome? Why is it steroid resistant?

Expert Answer :

No expert answer available.

Answer Discussion :

pediatriconcall

Depends on the cause. If due to congenital infections, the infection should be treated. Thyroid dysfunction should be corrected. If Finnish type then ACE inhibitors to control proteinuria is required. Indometahcin may be useful. Steroids do not work as it is not an immunological disorder.

17 years ago

ajay chiba

Most patients have primary congenital nephrotic syndrome. The Finnish type is the most common. The defect is in the NPHS1 gene{19q13.1}which encodes for nephrin. Nephrin is a transmembrane protein produced by podocytes that is involved in cell-cell or cell-matrix interactions. Abnormality in Nephrin leads to defects in structure and function of podocyte. Giving steroids will not help reverse the process as the defect in not immunologically based.

17 years ago

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