以硬化性胆管炎为首发表现的儿童朗格罕细胞组织细胞增生症1例并文献复习  

A case report of sclerosing cholangitis as the first manifestation of pediatric Langerhans cell histiocytosis and literature review

作　　者：廖伟伟[1] 钟雪梅[1] 马昕[1] 宁慧娟[1] 张艳玲[1] 

机构地区：[1]首都儿科研究所附属儿童医院消化内科,100020

出　　处：《北京医学》2015年第6期551-554,共4页Beijing Medical Journal

摘　　要：目的探讨以硬化性胆管炎为首发表现的儿童朗格罕细胞组织细胞增生症(LCH)的临床特点,治疗及预后。方法对我院收治的1例以硬化性胆管炎为首发表现的LCH患儿的病历资料进行回顾性总结,并复习相关文献。结果患儿18个月,表现为多系统受累、高危组的LCH。临床以黄疸、皮疹为首发症状,经LCHⅢ方案化疗后未见好转,最终放弃治疗。结论以硬化性胆管炎为首发表现的LCH比较罕见,临床可以黄疸为首发症状,预后差,熊去氧胆酸可改善生化指标,合并严重肝损害时可选择肝移植。Objective To evaluate the clinical characteristics, treatment and prognosis of sclerosing cholangitis as the first manifestation of pediatric Langerhans cell histiocytosis （LCH）. Methods Clinical manifestations of the child were retrospectively analyzed. Results The patient with multisystem Langerhans cell histiocytosis was eighteen months old. The patient had no response to therapy of LCH III protocol. Conclusion Sclerosing cholangitis as the first manifestation of pediatric LCH is seldom seen. The first clinical symptom is jaundice. The prognosis is not good. The patients could be treated with ursodeoxycholic acid （UDCA） to improve serum liver tests.Liver transplantation should be considered early in cases with severe hepatic involvement.

关 键 词：朗格罕细胞组织细胞增生症 硬化性胆管炎 儿童 

分 类 号：R725.9[医药卫生—儿科]