Witzleben C L
Perspect Pediatr Pathol. 1982;7:185-201.
Reduction of the number of interlobular bile ducts (BDP) has potentially significant physiologic consequences. Most of the disorders currently recognized as BDP involve an easily discernible reduction in duct numbers, but actual duct quantitation needs to be done routinely in the examination of liver biopsies in order to recognize and assay the importance of less marked reduction. Ducts are seldom if ever completely absent, and may not be uniformly reduced throughout the liver, so a wedge biopsy is the only completely satisfactory sample for the morphologic evaluation of duct numbers. In some conditions the number of ducts varies greatly with time. BDP may be only one aspect or manifestation of a disease primarily characterized by other features, or it may be the principal cause of distress and a major feature in defining a disorder. In a few conditions, the mechanism of the paucity has been quite well established as the destruction of previously existing ducts, but at present the basis for duct paucity in most conditions remain obscure. The treatment of BDP is similar in all conditions. Because of the wide range of circumstances in which BDP occurs, the prognosis is highly variable and depends on the underlying condition. In some patients BDP is associated with a long life of relatively good quality, in others it is associated with rapidly progressive liver disease, and in others with an intermediate course. In children particularly, conditions with BDP may be familial. In recent years there has been some advance in recognizing individual conditions associated with BDP, but many cases are still unclassifiable. Evaluation of a patient with BDP should be directed not only to diagnosing known conditions, but also to defining new ones, and should include family history, alpha 1-antitrypsin determinations, bile acid analyses, chromosome analysis, and consideration of virus infection. Additional efforts should be made to determine the pathogenesis of the duct paucity in individual conditions in order to prevent, arrest, or reverse the processes involved.
小叶间胆管数量减少具有潜在的重大生理影响。目前大多数被认为是小叶间胆管数量减少的疾病，其胆管数量减少易于辨别，但在肝活检检查中仍需常规进行实际的胆管定量分析，以便识别和评估不太明显的减少情况的重要性。胆管极少完全缺失，且在整个肝脏中减少情况可能并不均匀，因此楔形活检是评估胆管数量形态学的唯一完全令人满意的样本。在某些情况下，胆管数量随时间变化很大。小叶间胆管数量减少可能只是以其他特征为主的疾病的一个方面或表现，也可能是痛苦的主要原因以及定义一种疾病的主要特征。在少数情况下，胆管数量稀少的机制已明确为先前存在的胆管遭到破坏，但目前大多数情况下胆管数量稀少的原因仍不清楚。小叶间胆管数量减少在所有情况下的治疗方法相似。由于小叶间胆管数量减少发生的情况范围广泛，预后差异很大，取决于潜在病情。在一些患者中，小叶间胆管数量减少与较长的相对高质量生活相关，在另一些患者中与快速进展的肝病相关，并在其他患者中与中间病程相关。特别是在儿童中，伴有小叶间胆管数量减少的疾病可能具有家族性。近年来，在识别与小叶间胆管数量减少相关的个别疾病方面取得了一些进展，但许多病例仍无法分类。对小叶间胆管数量减少患者的评估不仅应针对诊断已知疾病，还应针对确定新疾病，应包括家族史、α1抗胰蛋白酶测定、胆汁酸分析、染色体分析以及考虑病毒感染。应做出进一步努力，以确定个别情况下胆管数量稀少的发病机制，以便预防、阻止或逆转所涉及的过程。
