Ndongo S, Mbengue M, Dia D, Seck S M, Ka M M, Moreira Diop T
Clinique médicale I, Hôpital A. Le Dantec, Dakar.
Dakar Med. 2008;53(2):127-30.
Auto-immune cholangitis appears by a table of cholestatic jaundice without anomalies of the hepatic bile ducts. It is a primitive biliary cirrhosis without antimitochondrial antibodies. This disease is reported at adult women in 90 % of cases. We report a 59 years old man case.
The patient was admitted for a diffuse melanodermy, a physical asthenia and a slimming which preceded 4 months by a pruritus with cholestatic jaundice. Biology showed a normal rate of transaminase. Alkaline phosphatases and direct bilirubine were high. Hepatitis (B and C) and HIV serologies were negative. Abdominal ultrasound showed a homogeneous liver. There were no dilations of the bile ducts. Antimitochondrial antibodies were negative and the histological examination confirms diagnosis.
Progress was good with ursodesoxycholic acid even if interval is short (5 months of evolution).
自身免疫性胆管炎表现为胆汁淤积性黄疸,而肝内胆管无异常。它是一种无抗线粒体抗体的原发性胆汁性肝硬化。90%的病例见于成年女性。我们报告一例59岁男性病例。
患者因弥漫性黑皮病、身体乏力和体重减轻入院,4个月前出现瘙痒伴胆汁淤积性黄疸。生化检查显示转氨酶水平正常。碱性磷酸酶和直接胆红素升高。乙肝、丙肝和艾滋病毒血清学检查均为阴性。腹部超声显示肝脏均匀。胆管无扩张。抗线粒体抗体阴性,组织学检查确诊。
即使病程较短(5个月),使用熊去氧胆酸治疗效果良好。