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目的了解住院儿童门脉高压症的病因和预后。方法回顾性分析１９７８～１９９７年共２０年的住院病例５３例，随访４０例，随访时间为０５～１２年，中位数１３年。结果９０年代以后儿童门脉高压症的住院率有上升的趋势，诊断的病因亦增多；病因主要是门脉海绵样变、门脉性肝硬化和先天性胆道闭锁，遗传代谢病亦占有一定的比例；在主要病因中，以先天性胆道闭锁所致的门脉高压症预后最差，门脉性肝硬化的预后极不一致，部分预后良好，门脉海绵样变以分流术后的预后较好。结论儿童门脉高压症的病因构成与成人相比有明显不同的特点，其预后与病因和治疗方式有关，临床上应有所认识。
Objective To understand the etiology and prognosis of hospitalized children with portal hypertension. Methods A retrospective analysis of 53 hospitalized cases with a total of 20 years from 1978 to 1997 was conducted in 40 cases. The follow-up time ranged from 0 5 to 12 years and the median was 1 3 years. Results The hospitalization rate of children with portal hypertension increased after the 1990s and the etiology of the diagnosis also increased. The etiology mainly included portal sponge-like change, portal cirrhosis and congenital biliary atresia, and the inherited metabolic disease also had certain Ratio; the main cause of congenital biliary atresia due to the worst prognosis of portal hypertension, the prognosis of portal hypertension is very inconsistent, part of the prognosis is good, portal vein sponge-like shunt after the prognosis is better . Conclusions The etiology of children with portal hypertension is significantly different from that of adults. The prognosis is related to the etiology and treatment and should be recognized clinically.