Fracchia M, Soubrane O, Houssin D, Galatola G
Divisione di Gastroenterologia, Ospedale Mauriziano Umberto I, Torino, Italy.
Ital J Gastroenterol. 1995 Oct-Nov;27(8):430-5.
Obstructive dominant biliary strictures are considered irreversible lesions in patients with primary sclerosing cholangitis; therapy with ursodeoxycholic acid has been shown to determine clinical, biochemical and histological improvement in such patients. To date, two reports of anatomical improvement of biliary tract lesions during ursodeoxycholic acid therapy have been published. We observed a 19-year-old male patient with primary sclerosing cholangitis who presented with severe cholestasis due to an obstructive dominant stricture of the common hepatic duct and absence of biliary excretion at cholescintigraphy. Oral administration of 900 mg/day ursodeoxycholic acid was followed by rapid decrease of jaundice and a normalization of life activity. Twelve months later a repeat cholescintigraphy showed normal biliary excretion. Despite continuing therapy, two years later the patient needed liver transplantation because of recurring severe obstructive jaundice. This report suggests that ursodeoxycholic acid may reverse a severe cholestatic syndrome in the course of primary sclerosing cholangitis despite a dominant stricture in the biliary tree. The mechanisms responsible for this effect are still obscure, but may involve restoration of bile flow through the biliary stricture. The late relapse during continuing therapy suggests that--at least in some patients--these effects may be temporary and ursodeoxycholic acid may lose its efficacy in the long term.
在原发性硬化性胆管炎患者中，梗阻性主导型胆管狭窄被认为是不可逆性病变；已证实熊去氧胆酸治疗可使这类患者出现临床、生化及组织学改善。迄今为止，已发表了两篇关于熊去氧胆酸治疗期间胆管病变出现解剖学改善的报告。我们观察了一名19岁原发性硬化性胆管炎男性患者，该患者因肝总管梗阻性主导型狭窄导致严重胆汁淤积，且在胆管闪烁造影时无胆汁排泄。口服900毫克/天的熊去氧胆酸后，黄疸迅速减轻，生活活动恢复正常。12个月后复查胆管闪烁造影显示胆汁排泄正常。尽管持续治疗，但两年后该患者因复发性严重梗阻性黄疸而需要进行肝移植。本报告提示，尽管胆管树存在主导型狭窄，但熊去氧胆酸可能会在原发性硬化性胆管炎病程中逆转严重胆汁淤积综合征。造成这种效应的机制仍不清楚，但可能涉及胆汁通过胆管狭窄处的流动恢复。持续治疗期间的晚期复发提示，至少在某些患者中，这些效应可能是暂时的，从长远来看熊去氧胆酸可能会失去其疗效。
