Supporting data for "Isogenic bile duct organoid-macrophage coculture for characterizing the roles of human cytomegalovirus infection in Biliary Atresia"

Biliary Atresia (BA) is a devastating rare childhood disease. In healthy individuals, liver bile is collected by the liver biliary system and deposited into the gall bladder, where it is stored. Then, when food is eaten, the gallbladder contracts pushing bile through the cystic duct and into the common bile duct and then into the duodenum to help break down the fats. In BA, when bile ducts become damaged, there is a blockage in the bile flow from the liver to the intestine, bile can back up into the liver, causing damage to liver cells, which can lead to liver failure. Though BA is a rare disease, the incidence is much higher in the Asian ethnic population than in other parts of the world. It is observed that for every 100,000 live births, 100-500 infants are born with BA in Japan and Taiwan. However, in the European population, BA is diagnosed in 5-25 babies in every 100,000 live births.Many models and concepts of disease progression and etiologies have been proposed, including autoimmune diseases, toxins, immune dysfunction, embryonic development abnormalities, and genetic abnormalities. However, none of them can solve the complete puzzle of BA.

胆道闭锁（BA）是一种毁灭性的罕见儿童疾病。在健康个体中，肝脏胆汁由胆管系统收集并储存在胆囊中。随后，当进食时，胆囊收缩，推动胆汁通过胆囊管进入胆总管，进而进入十二指肠，以助脂肪分解。在胆道闭锁的情况下，当胆管受损时，肝脏至肠道的胆汁流动出现阻塞，胆汁可逆流进入肝脏，导致肝细胞受损，进而可能引发肝衰竭。尽管胆道闭锁是一种罕见疾病，但其发病率在亚洲人群中远高于世界其他地区。观察发现，在日本和台湾，每10万活产婴儿中，有100-500名婴儿患有胆道闭锁。然而，在欧洲人群中，每10万活产婴儿中，有5-25名婴儿被诊断为胆道闭锁。关于疾病进展和病因的多种模型和概念已被提出，包括自身免疫性疾病、毒素、免疫功能紊乱、胚胎发育异常和遗传异常等。然而，这些理论均无法完全解开胆道闭锁的谜团。