Raweily E A, Gibson A A, Burt A D
University Department of Pathology, Western Infirmary Glasgow, Scotland, UK.
Histopathology. 1990 Dec;17(6):521-7. doi: 10.1111/j.1365-2559.1990.tb00791.x.
The infantile cholangiopathies are a group of conditions associated with neonatal jaundice, which include extrahepatic biliary atresia, paucity of intra-hepatic bile ducts and disorders associated with persistence of fetal biliary structures, the so-called ductal plate malformations. Although previously regarded as distinct entities, it has recently been suggested that they may represent parts of a disease spectrum in which the principal process is one of bile duct destruction, the morphological manifestations in individual cases being influenced by the stage of intra-uterine development at which such injury occurs and by the site within the biliary system at which there is maximum damage. To further examine this concept, we have studied liver biopsy specimens from 37 neonates with extrahepatic biliary atresia, with particular reference to abnormalities of the intrahepatic bile ducts. Paucity of intrahepatic ducts, defined as a bile duct: portal tract ratio of less than 0.9, was identified in six cases (16.2%). In eight cases (21.6%) we found concentric tubular ductal structures similar to those observed in ductal plate malformations. In one case, both abnormalities could be demonstrated. Our findings support the concept that there is overlap between the various types of infantile cholangiopathy.
婴儿期胆管病是一组与新生儿黄疸相关的病症，包括肝外胆道闭锁、肝内胆管稀少以及与胎儿胆管结构持续存在相关的疾病，即所谓的胆管板畸形。尽管以前被视为不同的实体，但最近有人提出，它们可能代表一种疾病谱的一部分，其中主要过程是胆管破坏，个别病例的形态学表现受这种损伤发生时的宫内发育阶段以及胆道系统中损伤最严重部位的影响。为了进一步研究这一概念，我们研究了37例肝外胆道闭锁新生儿的肝活检标本，特别关注肝内胆管的异常情况。肝内胆管稀少定义为胆管与门静脉分支比例小于0.9，在6例（16.2%）中被发现。在8例（21.6%）中，我们发现了与胆管板畸形中观察到的同心管状胆管结构相似的结构。在1例中，两种异常情况都能被证实。我们的研究结果支持了各种类型婴儿期胆管病之间存在重叠的概念。
