Беременность и роды у больных гепатолентикулярной дегенерацией

Data of pregnancy course and delivery in 3 patients with hepatolenticular degeneration (GLD) a rare disease of autosomal recessive inheritance pattern are presented. In one case GLD was diagnosed prior pregnancy and treatment with cuprenil was continued during pregnancy. No progressive GLD clinical symptoms were observed. However pregnancy and delivery appeared to provoke GLD typical manifestations in other two patients. They have not got specific treatment with cuprenil during pregnancy as disease was diagnosed only after delivery. Deliveries in patients with GLD were successful and the newborns were healthy. No evidence of cuprenil teratogenic effect on fetus was revealed in the first case. This GLD is not contraindication for successful pregnancy but it is necessary to use cuprenil for prevention of disease exacerbation.