IMPACT OF GASTROINTESTINAL DYSFUNCTION ON SURVIVAL AFTER LIVER-TRANSPLANTATION FOR FAMILIAL AMYLOIDOTIC POLYNEUROPATHY

Liver transplantation is the only effective treatment of familial amyl oidotic polyneuropathy type I (FAP). The aim of the present investigat ion was to identify factors at the time of submission for transplantat ion that had impact on survival, with special reference to gastrointes tinal disturbances. All 28 liver-transplanted FAP patients evaluated a t Umea University Hospital were included in the study. A modified body mass index was used to assess nutritional status. Intestinal examinat ions were performed to diagnose bile acid malabsorption, gastric reten tion, and bacterial contamination of the small bowel. A significantly improved survival rate was found for patients in a good nutritional st ate (P = 0.002). Peripheral neurological symptoms were unrelated to su rvival, whereas increased mortality was found for patients with bile a cid malabsorption (P < 0.05). Bacterial contamination and gastric rete ntion were common complications of the disease. In conclusion, malabso rption and malnutrition have a profound impact on the outcome of liver transplantation for familial amyloidotic polyneuropathy.