Ueno Takehisa, Kodama Tasuku, Noguchi Yuki, Nomura Motonari, Saka Ryuta, Takama Yuichi, Tazuke Yuko, Bessho Kazuhiko, Okuyama Hiroomi
Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, 565-0871, Osaka, Japan.
Department of Pediatrics, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, 565-0871, Osaka, Japan.
Pediatr Surg Int. 2019 Oct;35(10):1065-1070. doi: 10.1007/s00383-019-04535-9. Epub 2019 Aug 7.
Biliary atresia (BA) is characterized by progressive liver fibrosis, but it is difficult to assess the progression after the patient develops cirrhosis. Mac-2-binding protein glycosylation isomer (M2BPGi) is a new marker for hepatic fibrosis. We examined the chronological changes in M2BPGi levels in BA patients with cirrhosis.
Patients with cirrhosis were selected from among pediatric BA patients who had their native livers. Serum M2BPGi levels and Child-Pugh classification were evaluated. A total of 11 pediatric BA patients with cirrhosis were recruited.
Initial M2BPGi level after diagnosis of liver cirrhosis based on liver biopsy was on average 3.4, and the most recent M2BPGi level under observation was on average 4.3. The follow-up period from the initial M2BPGi measurement averaged 22.6 months. The ratio of the initial and most recent values (M2BPGi ratio) was on average 1.3 (0.5-2.4). Three cases with improved fibrosis (M2BPGi ratio < 1.0) remained in Child A, as did six cases (1.0 ≤ M2BPGi ratio < 2.0), but two cases with marked fibrosis progression (2.0 ≤ M2BPGi ratio) advanced to decompensated cirrhosis Child B.
M2BPGi is useful as a prognostic factor for BA patients with liver cirrhosis. In addition, fibrosis improved even after the development of cirrhosis.
胆道闭锁（BA）的特征是进行性肝纤维化，但在患者发展为肝硬化后，很难评估其进展情况。Mac-2结合蛋白糖基化异构体（M2BPGi）是肝纤维化的一种新标志物。我们研究了肝硬化BA患者M2BPGi水平随时间的变化。
从保留自身肝脏的儿科BA患者中选取肝硬化患者。评估血清M2BPGi水平和Child-Pugh分级。共招募了11例儿科肝硬化BA患者。
基于肝活检诊断肝硬化后的初始M2BPGi水平平均为3.4，观察到的最近M2BPGi水平平均为4.3。从初始测量M2BPGi到随访的平均时间为22.6个月。初始值与最近值的比值（M2BPGi比值）平均为1.3（0.5 - 2.4）。3例纤维化改善（M2BPGi比值＜1.0）的患者仍处于Child A级，6例（1.0≤M2BPGi比值＜2.0）也是如此，但2例纤维化明显进展（2.0≤M2BPGi比值）的患者进展为失代偿期肝硬化Child B级。
M2BPGi可作为肝硬化BA患者的预后因素。此外，即使在肝硬化发生后，纤维化仍可改善。
