Management of adult biliary atresia patients: should hard work and pregnancy be discouraged?

Purpose: The purpose of the paper was to study the potential risk and management of adult biliary atresia (BA) patients. Materials and Methods: Clinical courses and biochemical data were retrospectively analyzed in 52 BA patients who were operated in our department and are now older than 15 years, with special emphasis on 47 patients who survived beyond the age of 20 years. Results: Of these 47 patients, 45 (95.7%) are employed, receiving higher education, and/or married. Thirty-three patients showed favorable liver function at the age of 20 years with their native liver. Thereafter, 6 (18.2%) developed liver dysfunction after working hard or pregnancy. In these 6 patients, cholinesterase levels were significantly lower at the age of 20 years than those in the 27 stable patients (239.5 ± 42.6 vs 285.8 ± 47.5, P = .0216), whereas no significant difference was found in alanine aminotransferase, aspartate aminotransferase, or total protein levels at the age of 20 years or in any biochemical parameters at the age of 15 years. Of the 14 transplanted patients, 5 who underwent transplantation after the age of 20 years had relatively uneventful courses until the age of 20 years; however, their biochemical data already showed significant deterioration at puberty. Conclusion: Social activities and pregnancy should be managed individually according to the risk assessment for potential hepatic failure even in stable adult BA patients. A falling cholinesterase level after the age of 20 years may predict the risk for the late hepatic dysfunction.