[Histiocytosis X and sclerosing cholangitis].

Annales de pediatrie Pub Date : 1993-05-01
H Soua, H Pousse, F Ladeb, S Korbi, A Ayadi, R Boussoffara, Y Belkhir, B Descos, M T Sfar, A Harbi
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Abstract

The case of a seven-year-old with multifocal (type II) Langheran's cell histiocytosis since the age of two years is reported. Despite therapy, biliary cirrhosis with portal hypertension developed gradually. Histologic studies of liver biopsy specimens, computed tomography, and transhepatic cholangiography disclosed dilatation of the bile ducts, suggesting sclerosing cholangitis, a specific complication of Langherans' cell histiocytosis which precipitates the development of biliary cirrhosis.

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[组织细胞增多症和硬化性胆管炎]。
本文报告1例7岁多灶性(II型)朗格兰细胞组织细胞增多症,发病时间为2年。尽管治疗,胆汁性肝硬化合并门脉高压症逐渐发展。肝活检标本的组织学研究、计算机断层扫描和经肝胆管造影显示胆管扩张,提示硬化性胆管炎,这是Langherans细胞组织细胞增多症的一种特异性并发症,可促进胆汁性肝硬化的发展。
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