Outcome of gastric emptying and gastrointestinal symptoms after liver transplantation for hereditary transthyretin amyloidosis

BMC Gastroenterology

Research Article

Pontus Karling¹  Jonas Wixner¹  Intissar Anan¹  Ole B Suhr¹  Torbjörn Sundström² 

[1] Departments of Public Health and Clinical Medicine, Umeå University, S-90187, Umeå, Sweden;Radiation Sciences, Umeå University, S-90187, Umeå, Sweden;

关键词: Amyloidosis;    Hereditary;    Functional gastrointestinal disorders;    Gastric emptying;    Transplantation;    Liver;    Transthyretin;   

DOI  :  10.1186/s12876-015-0284-4

 received in 2014-09-26, accepted in 2015-04-17,  发布年份 2015

来源: Springer

【 摘 要 】

BackgroundHereditary transthyretin amyloid (ATTR) amyloidosis is a rare but fatal autosomal dominant condition that is present all over the world. A liver transplantation has been shown to halt the progress of the disease in selected patients and is currently considered to be the standard treatment. Gastrointestinal manifestations are common in hereditary ATTR amyloidosis and are important for the patients’ morbidity and mortality. The aim of this study was to evaluate the long-term outcome of gastric emptying, gastrointestinal symptoms and nutritional status after liver transplantation for the disease.MethodsSwedish patients with hereditary ATTR amyloidosis transplanted between 1990 and 2012 were included. A standardized method for measuring gastric emptying with a Tc99m-labelled meal followed by scintigraphy was utilized. Validated questionnaires were used to assess gastrointestinal symptoms and the modified body mass index (mBMI), in which BMI is multiplied by s-albumin, was used to evaluate nutritional status. Non-parametrical statistical tests were used.ResultsGastric emptying rates and nutritional statuses were evaluated approximately eight months before and two and five years after liver transplantation, whereas gastrointestinal symptoms were assessed in median nine months before and two and nine years after transplantation. No significant change was found in gastric emptying (median half-time 137 vs. 132 vs. 125 min, p = 0.52) or nutritional status (median mBMI 975 vs. 991 vs. 973, p = 0.75) after transplantation. Gastrointestinal symptom scores, however, had increased significantly over time (median score 7 vs. 10 vs. 13, p < 0.01).ConclusionsGastric emptying and nutritional status were maintained after liver transplantation for hereditary ATTR amyloidosis, although gastrointestinal symptom scores had increased over time.

【 授权许可】

CC BY   
© Wixner et al.; licensee BioMed Central. 2015