title: Outcome of gastric emptying and gastrointestinal symptoms after liver transplantation for hereditary transthyretin amyloidosis.
abstract: BACKGROUND
  Hereditary 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.
  
  
  METHODS
  Swedish patients with hereditary ATTR amyloidosis transplanted between 1990 and 2012 were included. A standardized method for measuring gastric emptying with a Tc(99m)-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.
  
  
  RESULTS
  Gastric 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).
  
  
  CONCLUSIONS
  Gastric emptying and nutritional status were maintained after liver transplantation for hereditary ATTR amyloidosis, although gastrointestinal symptom scores had increased over time.
authors.affiliation: Departments of Public Health and Clinical Medicine, Umeå University, S-90187, Umeå, Sweden. jonas.wixner@medicin.umu.se.
authors.forename: Jonas
authors.initials: J
authors.lastname: Wixner
authors.affiliation: Radiation Sciences, Umeå University, S-90187, Umeå, Sweden. torbjorn.sundstrom@diagrad.umu.se.
authors.forename: Torbjörn
authors.initials: T
authors.lastname: Sundström
authors.affiliation: Departments of Public Health and Clinical Medicine, Umeå University, S-90187, Umeå, Sweden. pontus.karling@vll.se.
authors.forename: Pontus
authors.initials: P
authors.lastname: Karling
authors.affiliation: Departments of Public Health and Clinical Medicine, Umeå University, S-90187, Umeå, Sweden. intissar.anan@medicin.umu.se.
authors.forename: Intissar
authors.initials: I
authors.lastname: Anan
authors.affiliation: Departments of Public Health and Clinical Medicine, Umeå University, S-90187, Umeå, Sweden. ole.suhr@medicin.umu.se.
authors.forename: Ole B
authors.initials: OB
authors.lastname: Suhr
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000150:complications / Q000503:physiopathology* / Q000601:surgery*; D015992:Body Mass Index; D003248:Constipation / Q000209:etiology; D003967:Diarrhea / Q000209:etiology; D005260:Female; D005746:Gastric Emptying; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D044342:Malnutrition / Q000209:etiology; D008875:Middle Aged; D009325:Nausea / Q000209:etiology; D009752:Nutritional Status; D011184:Postoperative Period; D012709:Serum Albumin / Q000032:analysis; D011795:Surveys and Questionnaires; D013997:Time Factors; D014839:Vomiting / Q000209:etiology; D055815:Young Adult
chemical_list: D012709:Serum Albumin