Malone M, Mieli-Vergani G, Mowat A P, Portmann B
Department of Histopathology, Hospital for Sick Children, London, United Kingdom.
Pediatr Pathol. 1989;9(6):623-31. doi: 10.3109/15513818909022371.
The lack of information on the state of fetal liver in PiZZ alpha-1-antitrypsin (AAT) deficiency and a single case report claiming a hypoplasia of interlobular bile ducts in a 20-week PiZZ fetus, instigated this histologic study of the liver in five PiZZ fetuses, 17-20 weeks of gestation and five age-matched controls. We found no difference between the percentage of portal tracts with identifiable bile ducts in the PiZZ (median 22.2%, range 21%-23%) and in the control (median 21.4%, range 20%-24%) on hematoxylin- and eosin-stained sections. Immunostaining with AE1, a monoclonal antibody to cytokeratins restricted to normal bile ducts, doubled the number of recognizable ducts in both PiZZ and control livers. In four PiZZ livers, but in none of the controls, granular deposits of AAT could be detected by specific immunoperoxidase staining. We conclude that an apparent paucity of interlobular bile ducts is normal in the 20-week fetal liver, and our data may be taken as reference for future study dealing with similar material. Except for the cytoplasmic deposition of granules immunoreactive to AAT antiserum, there was no evidence of any developmental anomaly, in particular of the bile duct system in these five PiZZ fetal livers.
关于PiZZ型α1抗胰蛋白酶（AAT）缺乏症胎儿肝脏状态的信息匮乏，以及一份单一病例报告称一名20周龄的PiZZ型胎儿小叶间胆管发育不全，促使我们对5例妊娠17 - 20周的PiZZ型胎儿肝脏以及5例年龄匹配的对照胎儿肝脏进行了组织学研究。我们发现，在苏木精和伊红染色切片上，PiZZ型胎儿（中位数22.2%，范围21% - 23%）和对照胎儿（中位数21.4%，范围20% - 24%）中可识别胆管的门管区百分比没有差异。用AE1（一种仅针对正常胆管的细胞角蛋白单克隆抗体）进行免疫染色后，PiZZ型和对照胎儿肝脏中可识别的胆管数量都增加了一倍。在4例PiZZ型胎儿肝脏中，但在对照胎儿肝脏中均未检测到，通过特异性免疫过氧化物酶染色可检测到AAT的颗粒状沉积物。我们得出结论，20周龄胎儿肝脏中明显缺乏小叶间胆管是正常现象，我们的数据可为未来处理类似材料的研究提供参考。除了对AAT抗血清有免疫反应的颗粒在细胞质中的沉积外，在这5例PiZZ型胎儿肝脏中没有任何发育异常的证据，特别是胆管系统没有异常。
