Sclerosing cholangitis in the paediatric patient
Sclerosing cholangitis in the paediatric patient
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DOI：
10.1053/bega.2001.0213
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发表时间：
2001-08-01
影响因子：
3.2
通讯作者：
Vergani, D
中科院分区：
文献类型：
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作者：
Mieli-Vergani, G;Vergani, D
硬化性胆管炎在儿童是一个异质性的条件，有不同的病因。硬化性胆管炎可在新生儿期遗传和诊断（新生儿硬化性胆管炎）;它可能在以后出现自身免疫（自身免疫性硬化性胆管炎）的特征;或者它可能与各种疾病有关，包括朗格汉斯细胞组织细胞增多症，免疫缺陷，银屑病，囊性纤维化，网状细胞肉瘤和镰状细胞贫血。与成人患者的经验相反，硬化性胆管炎作为个体疾病（原发性硬化性胆管炎）发生是罕见的。各种形式的硬化性胆管炎的起始事件和可能的致病机制各不相同，目前仍不清楚。治疗和预后取决于硬化性胆管炎的类型。
Sclerosing cholangitis in childhood is a heterogeneous condition, which has different aetiologies. Sclerosing cholangitis may be inherited and diagnosed in the neonatal period (neonatal sclerosing cholangitis); it may present later with features of autoimmunity (autoimmune sclerosing cholangitis); or it may be associated with a variety of disorders, including Langerhans cell histiocytosis, immunodeficiency, psoriasis, cystic fibrosis, reticulum cell sarcoma and sickle cell anaemia. In contrast to the experience in adult patients, sclerosing cholangitis occurring as an individual disease (primary sclerosing cholangitis) is rare. The initiating events and possible pathogenic mechanisms differ in the various forms of sclerosing cholangitis and are still obscure. Treatment and prognosis depend on the type of sclerosing cholangitis present.