[I型戈谢病：门静脉高压症的罕见病因]
[Gaucher's disease type I: an infrequent cause of portal hypertension].
作者信息
Cano Ruiz A, Martín Scapa A, Monescillo Francia A, Moreno Caparrós A
机构信息
Servicio de Digestivo, Hospital Ramón y Cajal., Universidad de Alcalá de Henares, Madrid.
出版信息
An Med Interna. 1998 Sep;15(9):483-4.
PMID:10079540
Abstract
Portal hypertension associated to Gaucher's disease type I with non cirrhotic liver involvement is a very in-equent finding. We report a case of Gaucher's disease type I without cirrhosis in which portal hypertension is the main manifestation. The mechanism of the portal hypertension is discussed in our report and can be related to an elevation in hepatic fluid resistance due to the Gaucher's cells compression on sinusoids.
摘要
与非肝硬化性肝脏受累的I型戈谢病相关的门静脉高压是一种非常罕见的发现。我们报告一例无肝硬化的I型戈谢病病例，其中门静脉高压是主要表现。我们的报告讨论了门静脉高压的机制，其可能与戈谢细胞对肝血窦的压迫导致肝内液体阻力升高有关。
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