小儿肝胆性黄疸的发病机制和常见疾病

Hepatobiliary jaundice in children:pathogenesis and relative diseases

小儿肝胆性黄疸是因小儿时期肝细胞和(或)胆管系统发生各种疾病,未能将非结合胆红素充分在肝细胞内转化成结合胆红素,并将结合胆红素随同胆汁流经胆管系统排至十二指肠,引起血非结合胆红素和结合胆红素同时增多而产生的黄疸。按其发生病变的组织部位可细分为亚细胞性黄疸、肝细胞性黄疸、胆管性黄疸和混合性黄疸。除Crigler-Najjar综合征因有大量脂溶性非结合胆红素损伤脑组织外,其他各种疾病中出现的黄疸,均无明显的由非结合胆红素和(或)结合胆红素造成的组织损害。因此重点是找出引起黄疸的基础疾病,予以相应治疗。

Hepatobiliary jaundice in children was caused by diseases which damage liver cells and/or biliary system. Unconjugated bilirubin in serum can not be fully transformed into conjugated bilirubin inside the hepatocytes, and/or all conjugated bilirubin can not be drained with bile through biliary system and finally go into intestine. So both serum unconjugated and conjugated bilirubin were elevated and lead to jaundice. Hepatobiliary jaundice can be divided into subcellular jaundice, hepatocellular jaundice, cholangio-jaundice, and mixture. Except Crigler-Najjar syndrome, no significant injury would be induced either by unconjugated bilirubin or conjugated bilirun. Therefore, the most important thing is to find out the underlying diseases and treat them accordingly.