Ascites of Great Abundance Revealing a Nephrotic Congenital Syndrome at the University Teaching Hospital of Bouaké: About a Case

• 作者: Christelle Avi John Patrick Yenan Koko Aude Aka-Tanoh Kossonou Roland Yeboua Kouadio Vincent Asse Kouassi Christian Yao Richard Azagoh-Kouadio Serge Gohi Bi Irie
• 来源期刊: 儿科学期刊(英文)
• 学科: 医学
• 关键词: ASCITES CONGENITAL Nephrotic SYNDROME FINNISH Type Renal Transplantation Cote d’Ivoire
• 年，卷（期）: 2018,（1）
• 页码范围: 19-24
• 页数: 6页
• 分类号: R73

摘要

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.