Cholestasis is the predominate feature of many pediatric hepatobiliary diseases. The physiologic flow of bile requires multiple complex processes working in concert. Bile acid (BA) synthesis and excretion, the formation and flow of bile, and the enterohepatic reuptake of BAs all function to maintain the circulation of BAs, a key molecule in lipid digestion, metabolic and cellular signaling, and, as discussed in the review, a crucial mediator in the pathogenesis of cholestasis. Disruption of one or several of these steps can result in the accumulation of toxic BAs in bile ducts and hepatocytes leading to inflammation, fibrosis, and, over time, biliary and hepatic cirrhosis. Biliary atresia, progressive familial intrahepatic cholestasis, primary sclerosing cholangitis, and Alagille syndrome are four of the most common pediatric cholestatic conditions. Through understanding the commonalities and differences in these diseases, the important cellular mechanistic underpinnings of cholestasis can be greater appreciated.

中文翻译：

---

小儿胆汁淤积性疾病：常见和独特的致病机制

胆汁淤积是许多小儿肝胆疾病的主要特征。胆汁的生理流动需要多个复杂过程协同工作。胆汁酸 (BA) 的合成和排泄、胆汁的形成和流动以及 BA 的肠肝再摄取都具有维持 BA 循环的功能，BA 是脂质消化、代谢和细胞信号传导的关键分子，并且如评论中所述，胆汁淤积发病机制中的重要介质。这些步骤中的一个或几个步骤的破坏可能会导致有毒的胆汁酸在胆管和肝细胞中积聚，从而导致炎症、纤维化，并且随着时间的推移，会导致胆汁性肝硬化和肝硬化。胆道闭锁、进行性家族性肝内胆汁淤积、原发性硬化性胆管炎和 Alagille 综合征是四种最常见的儿科胆汁淤积性疾病。通过了解这些疾病的共性和差异，可以更好地理解胆汁淤积的重要细胞机制基础。