Digestive Diseases and Sciences

, Volume 41, Issue 10, pp 1909–1914 | Cite as

Impact of gastrointestinal dysfunction on survival after liver transplantation for familial amyloidotic polyneuropathy

  • Ole Suhr
  • Åke Danielsson
  • Anders Rydh
  • Nils Nyhlin
  • Sven Ola Hietala
  • Lars Steen
Transplantation

Abstract

Liver transplantation is the only effective treatment of familial amyloidotic polyneuropathy type I (FAP). The aim of the present investigation was to identify factors at the time of submission for transplantation that had impact on survival, with special reference to gastrointestinal disturbances. All 28 liver-transplanted FAP patients evaluated at Umeå University Hospital were included in the study. A modified body mass index was used to assess nutritional status. Intestinal examinations were performed to diagnose bile acid malabsorption, gastric retention, and bacterial contamination of the small bowel. A significantly improved survival rate was found for patients in a good nutritional state (P=0.002). Peripheral neurological symptoms were unrelated to survival, whereas increased mortality was found for patients with bile acid malabsorption (P<0.05). Bacterial contamination and gastric retention were common complications of the disease. In conclusion, malabsorption and malnutrition have a profound impact on the outcome of liver transplantation for familial amyloidotic polyneuropathy.

Key words

hereditary amyloidosis inborn errors of metabolism malabsorption fat bile acids liver transplantation 

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Copyright information

© Plenum Publishing Corporation 1996

Authors and Affiliations

  • Ole Suhr
    • 1
  • Åke Danielsson
    • 1
  • Anders Rydh
    • 1
  • Nils Nyhlin
    • 1
  • Sven Ola Hietala
    • 1
  • Lars Steen
    • 1
  1. 1.From the Department of Medicine, Section for Gastroenterology and Department of Diagnostic RadiologyUmeå University HospitalUmeåSweden
  2. 2.Department of Medicine, Section for Gastroenterology and HepatologyUmeå University HospitalUmeåSweden

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