CAJ | 학술논문

目的通过分析胆道闭锁患儿Kasai术后自体肝存活超过20年患者的病例特点,总结影响其远期预后的相关因素.方法对自1989年3月至1993年3月在本医疗中心诊断为胆道闭锁并行手术治疗的患儿37例进行回顾性研究,其中男22例,女15例,手术日龄在60 d以内、61～80 d、81～100 d、100 d以上的患儿分别为6例、14例、14例和3例.所有患儿均为Ⅲ型胆道闭锁.胆道闭锁患儿术后自体肝存活超过20年的患儿共有11例,其中男7例,女4例.分析并比较其远期自体肝存活率、性别、手术年龄、术后早期胆管炎的发作、Kasai术后肝门部胆管梗阻再手术等因素.结果手术日龄在60 d以内、61～80 d、81～100 d、100 d以上手术患儿长期生存分别为3例、4例、3例和1例,按手术年龄相比较,远期自体肝存活率的差异无统计学意义(x2值=1.669,P=0.644).26例在术后1个月内发生早期胆管炎,其中长期生存5例;11例患儿术后1个月内无早期胆管炎,长期生存6例,按术后早期胆管炎的发作相比较,远期自体肝存活率的差异有统计学意义(x2值=4.614,P=0.032).肝门部胆管梗阻再次肝门空肠吻合手术7例,长期生存2例.结论 Kasai手术是治疗胆道闭锁的一种有效手段,术后部分患儿可获得长期生存.手术年龄对远期预后有影响,但在一定年龄范围内(＜100 d),手术年龄并不是决定预后的最重要因素.术后早期胆管炎是影响胆道闭锁患儿Kasai手术,长期自体肝存活的主要因素之一,并可能引起肝门部胆管梗阻,可显著降低患儿远期生存率.胆道闭锁Kasai术后出现肝门部胆管梗阻的患儿,行再次手术仍有远期存活的可能.
목적 통과분석담도폐쇄환인Kasai술후자체간존활초과20년환자적병례특점,총결영향기원기예후적상관인소.방법 대자1989년3월지1993년3월재본의료중심진단위담도폐쇄병행수술치료적환인37례진행회고성연구,기중남22례,녀15례,수술일령재60 d이내、61～80 d、81～100 d、100 d이상적환인분별위6례、14례、14례화3례.소유환인균위Ⅲ형담도폐쇄.담도폐쇄환인술후자체간존활초과20년적환인공유11례,기중남7례,녀4례.분석병비교기원기자체간존활솔、성별、수술년령、술후조기담관염적발작、Kasai술후간문부담관경조재수술등인소.결과 수술일령재60 d이내、61～80 d、81～100 d、100 d이상수술환인장기생존분별위3례、4례、3례화1례,안수술년령상비교,원기자체간존활솔적차이무통계학의의(x2치=1.669,P=0.644).26례재술후1개월내발생조기담관염,기중장기생존5례;11례환인술후1개월내무조기담관염,장기생존6례,안술후조기담관염적발작상비교,원기자체간존활솔적차이유통계학의의(x2치=4.614,P=0.032).간문부담관경조재차간문공장문합수술7례,장기생존2례.결론 Kasai수술시치료담도폐쇄적일충유효수단,술후부분환인가획득장기생존.수술년령대원기예후유영향,단재일정년령범위내(＜100 d),수술년령병불시결정예후적최중요인소.술후조기담관염시영향담도폐쇄환인Kasai수술,장기자체간존활적주요인소지일,병가능인기간문부담관경조,가현저강저환인원기생존솔.담도폐쇄Kasai술후출현간문부담관경조적환인,행재차수술잉유원기존활적가능.
Objective To review the clinical features of BA (biliary atresia) patients surviving over 20 years after Kasai operation without liver transplantation and summarize their prognostic factors.Methods A total of 37 BA patients diagnosed and surgically treated at our center from March 1989 to March 1993 were studied retrospectively.There were 22 males and 15 females.Their operative ages were ＜60 (n =6),61-80 (n =14),81-100 (n =14) and ＞100 (n =3) days respectively.All of them were classified as type Ⅲ BA.Among them,7 males and 4 females survived over 20 years without transplantation.Their long-term transplant-free survival rate,gender,operative age,occurrence of early postoperative cholangitis and hilar bile duct obstruction after Kasai operation were analyzed.Results The operative age was ＜60 (n=3),61-80 (n =4),81-100 (n=3) and ≥100 (n=1) days.The operative age had no statistic significance toward long-term transplant-free survival(x2 =1.669,P＝ 0.644).In 26 patients,cholangitis occurred within 1 month,5 patients with early cholangitis achieved long-term survival.The other 11 patients did not experience early cholangitis,and 6 of these patients obtained long-term survival.There was statistical significance between the patients with or without early cholangitis (x2 =4.614,P =0.032).Seven patients underwent reoperation with portojejunal anastomosis for hilar bile duct obstruction,in which 2 patients obtained long-term survival.Conclusions The Kasai procedure is an effective method for treatment of BA,some patients treated with Kasai operation can obtain long-term survival.The operative age influenced prognosis within a certain age (＜100 days) and it was not the most significant prognostic factor.Early cholangitis after Kasai operation is one of major prognostic factors of determining long-term survival.Early cholangitis can also cause hilar bile duct obstruction so as to significantly reduce long-term survival rate.However,reoperation for hilar bile duct obstruction may still lead to long-term survival.