Kasai术后生存12年以上患儿的随访

Long-term outcomes of Kasai＇s procedure-- twelve-years follow-up

目的对Kasai术后长期生存者进行随访，了解其生活质量、生理发育和肝功能情况。方法1989年3月至1997年3月对92例胆道闭锁患儿行Kasai术，对术后存活12年以上并得到随访的23例患儿，进行身高、体重、第二性征等生理发育的评价，以及肝功能、肝脏和门静脉的影像学检查。结果23例患儿中21例进行门诊复查，2例进行信访和电话复查。21例门诊复查患儿均测量身高和体重，有4例患儿身高低于同年龄同性别参照人群身高的均值，体重则均不低于同性别、同身高参照人群体重的均值-1SD。对21例的性征发育情况进行调查，0例14岁以上男性患儿，阴茎已发育9例，9例中出现遗精4例。女性9例中，5例在15岁以前月经初潮。21例肝功能检查，16例胆红素在正常范围内，白蛋白低于正常值1例，谷丙转氨酶升高7例。血常规检查正常范围的16例，红细胞、白细胞和血小板低于正常值2例。15例患儿超声检查13例有肝硬化改变。结论①Kasai手术后虽然仅有小部分（23／92）的患儿存活10年以上，存活者能与正常儿童一样上学读书；②与正常同龄儿相比，患儿远期生长发育还是受一定的影响，男孩的体格生长迟缓比例较高，青春发育延迟。女孩的生长发育和性征发育则无明显延迟；③术后应定期对血清肝功能及肝脏和门静脉包括影像学的检查；④Kasai手术后远期有部分患儿需行肝移植。

Objective Children undergoing the Kasai operation for biliary atresia were reviwed to assess the quality of life and physiological ,developments. Methods The physical developments （heights, weights, secondary sexual characteristics etc. ）, liver function test, hepatic portal vein ul trasound were reviewed in twenty-three biliary atresia patients 12 years after Kasai procedure. Results Of the 23 patients, we had a systematic assessment of 21 cases, and for the remaining 2 cases, patients condition were assessed via mail or telephone communication. Four out of the 21 cases had significant lower statues. The body weight of all 21 patients were within the normal range. Of the 10 boys more than 14 years of age, 9 cases had penile developments, 4 cases have had ejaculations;. Of the 10 girls, 5 began menstruations, before the age of 15 years. Sixteen patients had normal hilirubin values, one had a lower albumin level but normal A/G ratio, and 7 had elevated GTP（glutamate-pyruvale transaminase） levels. Blood cell counts were normal in 16 patients. The red blood cell, white blood cell or blood platelet counts were low in 2 patients. Thirteen out of 15 patients were found to have liver cirrhosis on ultrasound. Conclusions One quarter of the patients with biliary atresia survive more than 10 years after Kasai operation. They enjoy normal life. The patients＇ developments in our study were delayed , especially in boys. Periodical imaging of liver, portal vein and spleen is still necessary. Some of the patients underwent Kasai operation may require liver transplantation in the future.