[Apheresis for Byler syndrome in pregnancy: tolerance and effectiveness]

We report the first known case of a successful pregnancy in a 22-year-old white woman suffering from hepatic Byler's syndrome, a familial fibrogenic cholestasis observed in children and usually leading to death during adolescence or to liver transplantation. A first pregnancy was unsuccessful with intrauterine death. Medical surveillance of the second pregnancy was reinforced due to major cholestasis and itching sensation. Plasmapheresis was employed weekly from the 19th week of gestation. Within 2 weeks of treatment onset, plasma alkaline phosphatase levels returned to normal; pruritus completely disappeared after the second session. Delivery was decided at 33 weeks gestation with uterine section. The female baby weighed 2,090 g and outcome was excellent. This is the first reported case of Byler's syndrome with successful pregnancy. We underline the efficacy and safety of apheresis in this very particular disease.