Department of Pediatric Surgery, Xinhua Hospital, School of Medicine, Shanghai Jiaotong University, No. 1665 Kongjiang Road, Yangpu District, Shanghai, 200092, China.
Department of Transplantation and Hepatic Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
World J Pediatr. 2017 Dec;13(6):604-610. doi: 10.1007/s12519-017-0018-5. Epub 2017 Mar 22.
An aberrant immune response is the predominant pathogenetic factor in biliary atresia (BA). Programmed death-1 (PD-1) and its two ligands, programmed death ligand-1 and programmed death ligand-2 (PD-L1 and PD-L2, respectively) play an important inhibitory role in immune reactions. We aimed to illustrate the expression of these molecules in BA.
Liver specimens were obtained from infants with BA during the Kasai procedure (early BA) and liver transplantation (late BA). Intrahepatic expression of PD- 1, PD-L1, and PD-L2 were examined by immunostaining and compared with that in patients with neonatal hepatitis syndrome and normal controls. The correlation between the expression levels of these molecules in the liver and clinicopathological parameters was analyzed for each group.
Enhanced expression of PD-1 and its ligands occurred in the livers with early BA. In the BA-affected livers, PD-1 was correlated with the degree of peri-biliary inflammation, while PD-L2 was linked more directly with portal fibrosis. None of the three molecules was correlated with the prognosis of the Kasai procedure in patients with early BA.
Only PD-1 and PD-L1 are involved in the immune reactions of early BA. Elucidation of the detailed role of PD-L2 in BA requires further research.
异常的免疫反应是先天性胆道闭锁（BA）的主要发病因素。程序性死亡受体-1（PD-1）及其两个配体，程序性死亡配体-1 和程序性死亡配体-2（PD-L1 和 PD-L2）在免疫反应中发挥重要的抑制作用。我们旨在阐明这些分子在 BA 中的表达。
在葛西手术（早期 BA）和肝移植（晚期 BA）期间，从患有 BA 的婴儿中获得肝组织标本。通过免疫组化检测 PD-1、PD-L1 和 PD-L2 在肝内的表达，并与新生儿肝炎综合征患者和正常对照进行比较。分析每组这些分子在肝脏中的表达水平与临床病理参数之间的相关性。
早期 BA 肝脏中 PD-1 及其配体的表达增强。在受 BA 影响的肝脏中，PD-1 与胆管周围炎症的程度相关，而 PD-L2 与门脉纤维化的关系更为直接。在早期 BA 患者中，这三种分子均与葛西手术的预后无关。
只有 PD-1 和 PD-L1 参与了早期 BA 的免疫反应。阐明 PD-L2 在 BA 中的详细作用需要进一步研究。
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