Taba Ryusuke, Yamakawa Masaru, Miyakoshi Chisato, Imai Yukihiro
Department of Pediatrics, Kobe City Medical Center General Hospital, Kobe, Japan.
J Paediatr Child Health. 2012 Mar;48(3):E126-31. doi: 10.1111/j.1440-1754.2010.01874.x. Epub 2010 Oct 6.
Cholestasis in neonates is infrequently associated with Rh isoimmunization, and usually resolves within a month. The suggested pathophysiology is inspissated bile and hepatocellular damage. We report a rare case of refractory cholestasis presenting with cholangiolitis in a newborn with anti-E isoimmunisation. The cholangiolitis was disclosed by immunohistochemical investigation of conjugated hyperbilirubinaemia and by liver biopsy, which showed a number of CD8(+) lymphocytes within the portal tract damaging the interlobular bile duct. Bilirubin levels dramatically decreased after 14-day corticosteroid therapy (prednisolone, 2 mg/kg/day) implying that the cause of cholestasis could be immune-mediated cholangiolitis.
新生儿胆汁淤积症很少与Rh血型同种免疫相关，通常在一个月内消退。推测的病理生理学机制是胆汁浓缩和肝细胞损伤。我们报告了一例罕见的难治性胆汁淤积症病例，该新生儿因抗-E同种免疫而出现胆管炎。通过对结合胆红素血症的免疫组织化学研究和肝活检发现了胆管炎，肝活检显示门管区内有一些CD8(+)淋巴细胞破坏小叶间胆管。14天的皮质类固醇治疗（泼尼松龙，2毫克/千克/天）后胆红素水平显著下降，这意味着胆汁淤积的原因可能是免疫介导的胆管炎。
