Does hepatic hilum morphology influence long-term prognosis in type I/I cyst biliary atresia?
Does hepatic hilum morphology influence long-term prognosis in type I/I cyst biliary atresia?
复制标题
肝门形态是否影响 I/I 型胆道囊肿闭锁的长期预后？
DOI：
10.1007/s00383-015-3771-3
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发表时间：
2015
期刊：
影响因子：
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通讯作者：
Tanaka H.
中科院分区：
文献类型：
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作者：
Nio M;Wada M;Sasaki H;Tanaka H.
目的部分胆囊性胆道闭锁（BA）患者获得了非常好的术后病程。早期区分儿科患者的可切除型疾病可以导致有益的长期术后管理。我们研究了肝门形态是否影响I/I型囊肿BA（胆总管闭锁伴/不伴囊肿）的长期预后，方法自1972年以来确定的253例BA患者中，40例被归类为I/I型囊肿，并根据肝管大小分为两种亚型α亚型18例，导管直径≥1 mm;结果α和β亚型患者黄疸消失分别为16例（89%）和19例（86%），分别（p= 0.81），13例（72%）和12例（55%）使用自体肝脏存活（p= 0.18）平均年龄分别为23.3岁和25.5岁（p= 0.42）.结论α和β亚型之间的长期结果没有显著差异，尽管大约40%的患者在术后过程中出现肝功能衰竭。因此，密切的长期随访是必要的I/I型囊肿，无论肝门形态。
PurposeSome patients with cystic biliary atresia (BA) achieve exceptionally good postoperative courses. Early differentiation of pediatric patients with the favorable-type disease can lead to beneficial long-term postoperative management. We examined whether the hepatic hilum morphology affects long-term prognosis in type I/I cyst BA, atresia of the common bile duct with/without a cyst.MethodsOf 253 BA patients identified since 1972, 40 were classified as having type I/I cysts and were divided into two subtypes according to hepatic duct size (subtype α:n= 18; duct diameter, ≥1 mm and subtype β:n= 22; duct diameter, <1 mm) to compare postoperative clinical courses.ResultsIn subtypes α and β, jaundice disappeared in 16 (89 %) and 19 (86 %) patients, respectively (p= 0.81), and 13 (72 %) and 12 (55 %) survived with native livers (p= 0.18) at a mean age of 23.3 and 25.5 years, respectively (p= 0.42).ConclusionsThere were no significant differences in long-term outcomes between subtypes α and β, although approximately 40 % developed liver failure during the postoperative course. Thus, close long-term follow-up is essential in type I/I cysts, regardless of the hepatic hilum morphology.