Gauthier-Villars Marion, Franchi Stéphanie, Gauthier Frédéric, Fabre Monique, Pariente Danièle, Bernard Olivier
Division of Hepatology, Hôpital Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre cedex, France.
J Pediatr. 2005 Apr;146(4):568-73. doi: 10.1016/j.jpeds.2004.12.025.
We describe cholestasis as a result of bile duct abnormalities in 8 children with portal vein obstruction. In a clinical, biochemical and radiological investigation of 121 children with cavernous transformation of the portal vein seen between 1986 and 2000, 8 presented with jaundice, pruritus, and/or raised serum aminotransferases and/or gamma glutamyl transpeptidase (gamma GT) activities. Each displayed dilation and narrowing of intra- and/or extrahepatic bile ducts. Surgical decompression of the portal system (portal-systemic or Rex anastomosis) resulted in the regression of the signs of cholestasis in all children. We conclude that children with portal vein obstruction may exhibit clinically significant cholestasis as a result of external compression of the bile duct by the cavernoma.
我们描述了8例门静脉阻塞患儿因胆管异常导致的胆汁淤积。在1986年至2000年间对121例门静脉海绵样变性患儿进行的临床、生化及放射学调查中，8例出现黄疸、瘙痒、血清转氨酶和/或γ-谷氨酰转肽酶（γ-GT）活性升高。每例均表现为肝内和/或肝外胆管扩张和狭窄。门静脉系统手术减压（门体分流或雷克斯吻合术）使所有患儿的胆汁淤积体征消退。我们得出结论，门静脉阻塞患儿可能因海绵状血管瘤对胆管的外部压迫而出现具有临床意义的胆汁淤积。
