Abstract
Congenital nephrotic syndrome (CNS) is defined as the presence of proteinuria > 50 mg/kg/24h associated with a protein concentration g/L or albuminemia 30 g/L in an infant less than 3 months old. The CNS is rare, of various clinical forms dominated by the Finnish type caused by a mutation of the NPHS1 gene located on chromosome 19. The edematous syndrome is the most common mode of discovery. We report a case discovered in an infant of 50 days admitted for ascites of great abundance. The aim of this study was to describe the main epidemiological, diagnostic, therapeutic and evolutionary aspects of this syndrome. Improving the prognosis of this condition requires advocacy with the political authorities of Cote d’Ivoire to provide Teaching Hospital for the resources needed to perform kidney transplantation.
Highlights
The Congenital nephrotic syndrome (CNS) is rare, of various clinical forms dominated by the Finnish type caused by a mutation of the NPHS1 gene located on chromosome 19
We report a case discovered in an infant of 50 days admitted for ascites of great abundance
The CNS is of various clinical forms dominated by the Finnish type caused by a mutation of the NPHS1 gene located on chromosome 19
Summary
When NS occurs between birth and the age of 3 months, it is called congenital nephrotic syndrome (CNS). It is a rare condition, accounting for 3% - 13% of the child’s NS, with a poor prognosis [4] [5]. The management of the Finnish-type CNS is based on renal transplantation preceded by a conservative treatment made of drips daily or every two days of albumin, the administration of gamma globulins, the hyperprotein and low-salt diet, the vitamin supplementation, thyroxine supplementation, prevention of infections and thrombotic complications [8]. In severe forms where protein leakage is massive, Niaudet [7] recommended that, pending renal transplantation, bilateral nephrectomy followed by dialysis sessions until the patient reaches a weight of 8 - 9 kg
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