Maladie de Von Hippel-Lindeau avec mutation de novo et multiples lésions pancréatiques symptomatiques. À propos d'une observation et revue de la littérature

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• 作者：A. N. Guingané ; R. A. Sombié…
• 关键词：Polycystic ; Pancreas disease Von Hippel ; Lindeau ; De novo mutation
• 刊名：Journal Africain d'H¨¦pato-Gastroent¨¦rologie
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：10
• 期：1
• 页码：25-30
• 全文大小：459 KB
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• 作者单位：A. N. Guingané (1)
  R. A. Sombié (1)
  T. R. C. Da-Ido (1)
  A. Bougouma (1)
  
  1. Service d’hépato-gastroentérologie, CHU Yalgado Ouédraogo, Ouédraogo, Burkina Faso
  
• 刊物主题：Gastroenterology; Infectious Diseases; Tropical Medicine;
• 出版者：Springer Paris
• ISSN：1954-3212

文摘

The Von Hippel-Lindau (VHL) is a rare, autosomal dominant multisystemic disease predisposing to the development of benign and malignant tumors richly vascularized. It is caused by germline mutations of the VHL tumor suppressor gene. During this disease, pancreatic disease is common but clinical manifestations are rare. Pancreatic damage usually consists of multiple cysts, more rarely serous cystadenomas or isolated cysts. We report the case of a patient who presented with acute pancreatitis during a Von Hippel-Lindeau de novo with pancreatic achieved by several types of lesions. The interest of our case lies in the achievement of pancreatic fashion and clinical expression, rarely described and the need to remember that this condition also exists in sub- Saharan Africa should be sought even in the absence of family history VHL.