胆道闭锁的临床现状与思索

Clinical characteristics, surgical treatment and outcome of biliary atresia

目的 回顾性分析1999年至2008年十年间收治的胆道闭锁患儿临床资料，为今后临床诊断、防治提供有益资料。方法收集共184例胆道闭锁患儿，其中34例放弃治疗（18．47％），余150例作基本概况，临床表现、诊断、外科处理和转归等分析。男86例，女64例，求医时年龄（56．20±39．44）d，其中55例D90d（36．67％），诉大便转白时间为（22．33±20．3）d。均疑黄疸消退迟缓〉2周，有进行性血胆红素增高；临床诊断方法有B超检查（91．33%60），同位素闪烁检查（53．33％），血胆红素、酶测定（100％），影像学检查（58．66％）。结果手术探查时年龄（81．11±31．01）d，行Kasai手术率仅66％（99/150），随访率77．33%／60（116／150），随访9个月～8．2年，平均（23．77±16．4）个月，死亡56例（48．27％），肝移植6例（5．17％），正常生活30例（25．86％），带门脉高压等合并症生存24例（20．69％）。结论胆道闭锁是新生儿高胆红素血症中常见疾病。①其病因学说多种多样，大便转自在出生后（22．33±20．3）d，此现象以Schreiber提出的观点能较合理解释；②诊断无特异敏感的方法，还是以多种检查综合评估为好，腹部B超无创，值得进一步探索；③本组病死率较高48．27％，与文献报告有差距；④终末期肝移植是唯一有效方法，但本组仅6例，可能与经济、传统观念、供肝者条件不好等有关。

Objective To retrospectively analyze the clinical characteristics, surgical treatment and outcome of biliary atresia （BA） during the last decade Methods From 1999 to 2008, the clinical data of 184 patients with BA were collected. Among them, 34 patients gave up further treatment. The basic conditions, clinical manifestations, diagnosis, surgical treatment and outcome of the other 150 patients were retrospectively analyzed. Results The 150 patients with a mean age of 56. 02 ±39. 44 days included 86 males and 64 females. Fifty five patients were older than 90 days at the time of diagnose. Common complaints were presented with jaundice longer than 2 weeks, white stools and dark u rine. To diagnose their diseases, the patients underwent ultrasonography （ 137/150, 91. 33%）, hepatobiliary seintigraphy （80/150, 53. 33 % ）, biochemical liver function tests （ 150/150, 100 % ） and imaging examination （88/150, 58. 66 % ）. Kasai procedure was performed on 88 patients （58. 66 % ） with an average age of 81.11 ± 31.01 days. One hundred and sixteen patients （77. 33%） were followed up for 0. 75 to 8. 2 years after operation （mean, 23. 77 ± 16. 4 months）. Fifty six patients died, 6 （5. 17%） underwent liver transplantation and 30 patients（25.86%）had normal quality of life. Twenty four （20. 6%） patients were complicated with portal vein hypertension. Conclusions BA is a common cause of cholestatic jaundice in neonates. From the experience at this center, （1） the aetiology of BA remains unknown. The patients with BA start having Acholic （white） stools on an average age of 22. 33 ±20. 3 days, which may be explained by the so called two-hit theory. （2） Current diagnostic methods are not specific and sensitive enough, and should be performed combined examinationg. （3） In this study, mortality （48. 27%） is higher than European BA Registries report. （4） The overall prognosis of BA patients has improved since the era of pediatric liver transplantation Only 6 eases were performed in our group liver transplantation due to its high cost.