新生儿引起肝内胆汁淤积是由感染、遗传代谢性疾病、胃肠外营养、基因突变等各种不同病因导致胆汁形成、分泌和排泄障碍。胆汁在体内淤积会影响新生儿的生长发育,严重者出现肝纤维化、肝功能衰竭等不可逆的改变,其发病的机制涉及参与肝脏胆汁酸分泌的各种转运体、肝细胞膜转运蛋白转录和和转录后的调控等。本文归纳总结近年来国内外关于胆汁酸转运体及其转录和转录后的调控,从而为此疾病的防治提供理论依据,为将来降低其发生率和改善预后。

Neonatal intrahepatic cholestasis is caused by infection,genetic metabolic diseases,parenteral nutrition,gene mutation and other different causes leading to bile formation,secretion and excretion disorders．Bile stasis in the body will affect the growth and development of the newborn,and in severe cases,irreversible changes such as liver fibrosis and liver failure will occur．The pathogenesis of bile stasis involves various transporters involved in the secretion of bile acids in the liver,transcription and post transcription regulation of liver cell membrane transporters．In this paper,we summarized the recent studies on bile acid transporters and their transcriptional and post transcriptional regulation at home and abroad,so as to provide a theoretical basis for the prevention and treatment of this disease,and to reduce its incidence and improve its prognosis in the future.

目的 研究不同孕周妊娠期肝内胆汁淤积症（ICP）产妇胆汁酸、肝酶、α-羟丁酸脱氢酶（α-HBDH）水平与围产儿结局关系。方法 采用病例对照研究方法,选取孕中期和晚期ICP组、对照组为研究对象。生化仪检测总胆汁酸（TBA）、血清肝酶、α-HBDH水平。高效液相色谱串联质法检测血清内胆酸（CA）、鹅脱氧胆酸、脱氧胆酸（DCA）、石胆酸（LCA）等游离胆汁酸含量。结果 孕中期ICP组血清内CA、DCA、LCA、TBA丙氨酸氨基转移酶（ALT）、天门冬氨酸氨基转移酶（AST）均大于孕晚期组,差异有统计学意义（P<0.05）。多因素Logistic 回归分析结果显示：ALT、AST、a-HBDH、CA、DCA、LCA和TBA升高是围产儿不良结局的危险因素。结论 各类胆汁酸、肝酶、α-羟丁酸脱氢酶对围产儿结局预测价值不同。ALT、AST、a-HBDH、CA、DCA、LCA和TBA升高是围产儿不良结局的危险因素。