Orthotopic liver transplantation for autoimmune hepatitis and cryptogenic chronic hepatitis in children.

Background. Autoimmune hepatitis (AIH) and cryptogenic chronic hepatitis (C CH) are important causes of liver failure in children, frequently necessita ting orthotopic liver transplantation (OLT). The aim of this study is to re view disease progression and potential differences between subgroups of chi ldren with AIH and CCH. Methods. The medical records of 65 children diagnosed with AIH or CCH betwe en 1980 and 1998 were evaluated. Results. The median age at presentation was 9 years, 8 months (range 4 mont hs-19 years), and the median follow-up period was 8 years (range 3 months-1 8 years, 10 months). Forty-one patients (63%) were female. Twenty-eight pat ients were Hispanic, 28 were Caucasian, 8 were African-American, and I was Asian. Forty-three patients (66%) were diagnosed with type 1 AIH, 8 (12%) w ith type 2 AIH, and 14 (22%) with CCH. Forty patients (62%) underwent OLT ( 51% of those with type I AIH, 75% of those with type 2 AIH, and 86% of thos e with CCH). Thirteen (33%) of the transplanted patients experienced diseas e recurrence. African-American patients experienced a significantly higher rate of disease recurrence post-OLT than did Hispanic patients. Seven patie nts (11%) died, two without OLT, and five posttransplantation. Conclusions. AIH and CCH frequently necessitate OLT in children. CCH is a m ore aggressive disease than Type I AIH among children with these disorders. Ethnicity influences the rate of disease recurrence after liver transplant ation.