alexa Le Syndrome de Denys-Drash, une Cause Rare de Syndrome N ́ ephrotique : ` a Propos d’un Cas en Milieu P ́ ediatrique Tropical
ISSN: 2090-7214

Clinics in Mother and Child Health
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Research Article

Le Syndrome de Denys-Drash, une Cause Rare de Syndrome N ́ ephrotique : ` a Propos d’un Cas en Milieu P ́ ediatrique Tropical

 
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Abstract

Le syndrome de Denys-Drash (DD) est une affection g´en´etique rare due `a la mutation du g`ene WT1, impliqu´e dans la morphogen`ese des organes g´enitaux externes et du rein. Il associe un syndrome n´ephrotique cong´enital et une ambigu¨?t´e sexuelle. Les premiers signes surviennent d`es les 3 premiers mois de vie sous forme d’une scl´erose m´esangiale diffuse posant un v´eritable probl`eme th´erapeutique. L’´evolution spontan´ee se fait vers une insuffisance r´enale terminale et vers une tumeur de Wilms et/ou un gonadoblastome. Dans cet article, nous rapportons le cas d’un syndrome de DD chez un nourrisson de race noire ˆag´e de 6 mois dans l’unit´e de n´ephrologie p´ediatrique du CHU de Yopougon. Nous discutons `a partir d’une revue de la litt´erature les principaux aspects diagnostics, th´erapeutiques et ´evolutifs de ce syndrome. Nous insistons sur la n´ecessit´e d’une collaboration multidisciplinaire incluant les obst´etriciens, n´eonatalogistes, chirurgien p´ediatres et n´ephrop´ediatres pour un diagnostic pr´ecoce et une prise en charge adapt´ee.

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