Saudi Journal of Kidney Diseases and Transplantation

ARTICLES
Year
: 2003  |  Volume : 14  |  Issue : 3  |  Page : 328--335

Congenital Nephrotic Syndrome


Radi MA Hamed 
 Department of Pediatrics, Jordan University Hospital, Jordan

Correspondence Address:
Radi MA Hamed
Department of Pediatrics, Jordan University Hospital, P.O.Box 13046, Amman
Jordan

The congenital nephrotic syndrome (CNS) is an uncommon disorder with onset of the nephrotic syndrome usually in the first three months of life. Several different diseases may cause the syndrome. These may be inherited, sporadic, acquired or part of a general malformation syndrome. The clinical course is marked by failure to thrive, recurrent life threatening bacterial infections, and early death from sepsis and/or uremia. A characteristic phenotype may be seen in children with CNS. The majority of reported cases of CNS are of the Finnish type (CNF). Although the role of the glomerular basement membrane has been emphasized as the barrier for retaining plasma proteins, recent studies have clearly shown that the slit diaphragm is the structure most likely to be the barrier in the glomerular capillary wall. The gene (NPHS1) was shown to encode a novel protein that was termed nephrin, due to its specific location in the kidney filter barrier, where it seems to form a highly organized filter structure. Nephrin is a transmembrane protein that probably forms the main building block of an isoporous zipper-like slit diaphragm filter structure. Defects in nephrin lead to the abnormal or absent slit diaphragm resulting in massive proteinuria and renal failure


How to cite this article:
Hamed RM. Congenital Nephrotic Syndrome.Saudi J Kidney Dis Transpl 2003;14:328-335


How to cite this URL:
Hamed RM. Congenital Nephrotic Syndrome. Saudi J Kidney Dis Transpl [serial online] 2003 [cited 2019 Dec 10 ];14:328-335
Available from: http://www.sjkdt.org/article.asp?issn=1319-2442;year=2003;volume=14;issue=3;spage=328;epage=335;aulast=Hamed;type=0