有许多病理过程涉及肝胆树的破坏而致肝内胆管局灶性或弥漫性消失,仔细检查未能发现胆管的正常组成,临床上出现郁胆现象,称之为胆管消失综合征(VBDS),可由发生学、免疫学、血管、感染或化学机制等引起。表现为新生儿郁胆综合征的肝内胆管闭锁等先天性异常已被认为是在胚胎期的胆管被某些因子进行性破坏的结果,多量胆管累及时就出现肝内胆管缺乏(家族性 PIBD),常伴其他先天性畸形,慢性郁胆不伴发肝硬变或继发性门脉高压症。非家族性 PIBD 有时见于下列不同情况:巨细胞病毒、风疹、呼吸和肠道病 There are many pathological processes involving the destruction of liver and gallbladder tree caused by focal or diffuse disappearance of intrahepatic bile duct, a careful examination failed to find the normal composition of the bile duct, clinical manifestations of depression, called bile duct disappearance syndrome (VBDS) May be caused by learning, immunology, blood vessels, infections or chemical mechanisms. Congenital anomalies such as intrahepatic biliary atresia manifested as neonatal cholestatic syndrome have been considered as the result of progressive destruction of biliary ducts in embryonic stages by certain factors. Lack of intrahepatic bile ducts (familial PIBD ), Often accompanied by other congenital malformations, chronic cough without cirrhosis or secondary portal hypertension. Non-familial PIBD is sometimes found in the following different situations: cytomegalovirus, rubella, respiratory and intestinal diseases