Efficacy and tolerance of plasmapheresis during pregnancy in a woman with Byler's syndrome.

We report the first known case of a successful pregnancy in a 22-year-old w hite woman suffering from hepatic Byler's syndrome, a Familial fibrogenic c holestasis observed in children and usually leading to death during adolesc ence or to liver transplantation, A first pregnancy was unsuccessful with i ntrauterine death, Medical surveillance of the second pregnancy was reinfor ced due to major cholestasis and itching sensation, Plasmapheresis was empl oyed weekly from the 19th week of gestation, Within 2 weeks of treatment on set, plasma alkaline phosphatase levels returned to normal; pruritus comple tely disappeared after the second session. Delivery was decided at 33 weeks gestation with uterine section, The female baby weighed 2 090 g and outcom e was excellent. This is the first reported case of Byler's syndrome with s uccessful pregnancy. We underline the efficacy and safety of apheresis in t his very particular disease.