Hepatol Int. 2010 Aug 4;4(3):653-8. doi: 10.1007/s12072-010-9205-3.
BACKGROUND/AIMS: Sclerosing cholangitis is a rare complication of Langerhans cell histiocytosis in children which can result in liver failure. This combination is even rarer in adults.
We report a 65-year-old female who developed sclerosing cholangitis 4 years after the diagnosis of Langerhans cell histiocytosis.
Sclerosing cholangitis caused by Langerhans cell histiocytosis is a rare condition in the adult population, but it has a high mortality. There is no definitive therapy other than liver transplantation. The long-term efficacy of liver transplantation remains unknown.
背景/目的:硬化性胆管炎是儿童朗格汉斯细胞组织细胞增生症的罕见并发症,可导致肝衰竭。这种组合在成人中更为罕见。
我们报告了一例 65 岁女性,在诊断为朗格汉斯细胞组织细胞增生症 4 年后发展为硬化性胆管炎。
成人朗格汉斯细胞组织细胞增生症引起的硬化性胆管炎是一种罕见疾病,但死亡率很高。除肝移植外,尚无明确的治疗方法。肝移植的长期疗效仍不清楚。