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的过程协同工作。胆汁酸 （BA） 的合成和排泄、胆汁的形成和流动以及 BA 的肠肝再摄取都有助于维持胆汁酸的循环，胆汁酸是脂质消化、代谢和细胞信号传导的关键分子，并且如综述中所述，胆汁淤积症发病机制中的关键介质。这些步骤中的一个或多个中断会导致毒性 BA 在胆管和肝细胞中积累，从而导致炎症、纤维化，并随着时间的推移导致胆汁和肝硬化。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同点和差异，可以更好地理解胆汁淤积的重要细胞机制基础。