Pape Lars, Olsson Karen, Petersen Claus, von Wasilewski Reinhard, Melter Michael
Department of Pediatric Nephrology, Hepatology, and Metabolic Disorders, Hannover Medical School, Hannover, Germany.
Liver Transpl. 2009 Aug;15(8):876-82. doi: 10.1002/lt.21711.
Biliary atresia (BA) is the leading indication for liver transplantation (LTx) during childhood. Predictive markers for progression are lacking. We investigated the correlation between quantified fibrosis of the liver at the time of Kasai hepatic portoenterostomy (KPE) and the clinical course. Liver tissue was obtained at the time of KPE in 53 children and stained with Picrosirius red. The mean volume of fibrosis per number of periportal fields (Vfib) and the Ishak score were calculated. Vfib was 3.9%. Transplant-free survival was significantly higher in those patients with Vfib < 2.5% (P < 0.05, Kaplan-Meier analysis). Vfib < 2.5% predicted transplant-free survival with a sensitivity of 100% and a specificity of 48% (positive predictive value: 100%, negative predictive value: 17%). Vfib was correlated with bilirubin 6 months after KPE (Bili6m). The negative predictive value could be enhanced to 23% if Vfib < 2.5% was combined with Bili6m < 20 micromol/L. The Ishak score showed no correlation with transplant-free survival or Vfib. Detection of Vfib at the time of KPE is a valid marker in predicting transplant-free survival in children with BA. This method should be considered an integral part of scoring systems predicting the indication for LTx. Liver Transpl 15:876-882, 2009. (c) 2009 AASLD.
胆道闭锁（BA）是儿童期肝移植（LTx）的主要适应证。目前缺乏疾病进展的预测标志物。我们研究了在Kasai肝门肠吻合术（KPE）时肝脏定量纤维化与临床病程之间的相关性。在53例儿童进行KPE时获取肝组织，并用天狼星红染色。计算每个门周区域的纤维化平均体积（Vfib）和Ishak评分。Vfib为3.9%。Vfib<2.5%的患者无移植生存显著更高（P<0.05，Kaplan-Meier分析）。Vfib<2.5%预测无移植生存的敏感度为100%，特异度为48%（阳性预测值：100%，阴性预测值：17%）。Vfib与KPE后6个月的胆红素（Bili6m）相关。如果将Vfib<2.5%与Bili6m<20μmol/L相结合，阴性预测值可提高到23%。Ishak评分与无移植生存或Vfib无相关性。在KPE时检测Vfib是预测BA患儿无移植生存的有效标志物。该方法应被视为预测肝移植适应证评分系统的一个组成部分。《肝脏移植》15:876 - 882，2009年。（c）2009年美国肝病研究学会。
