Abstract:

To summarize and explore the etiopathogenisis, clinical characteristics, diagnosis and treatment and prognosis of idiopathic adulthood ductopenia. We described a 27-year-old Chinese female initially manifested with jaundice, pruritus, dark urine, and pallor of the stools in October, 2002. Symptoms occurred repetitively and severed progressively, and she received therapy at the General Hospital of Chinese People’s Armed Police Forces. B-ultrasound demonstrated that hepatic lesion and slightly swelling spleen. Pathological diagnosis of liver biopsy showed that she affected idiopathic adulthood ductopenia. The outcome of treatment of ursodeoxycholic acid and adrenal cortical hormone was not significant. She received orthotopic liver transplantation on June 24th, 2005. After liver transplantation, pruritus and jaundice were promptly disappeared. Each index of hepatic function recovered to a normal level. In March 2007, decreased dose of hormone induced an abnormal hepatic function, with mildly acute rejection. Drug dose was regulated, and hepatic function gradually returned to normal. During follow-up 51 months after the operation, she was doing well and was free of symptoms with normal liver function and no evidence of allograft dysfunction. The pathogenesis of idiopathic adulthood ductopenia is still unknown. There are still no precise and effective drugs for treatment of idiopathic adulthood ductopenia. Liver transplantation offers an effective therapy for end-stage patients with idiopathic adulthood ductopenia.