Skip to main content
SpringerLink
Log in

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

  • Transplantation
  • Published:
Digestive Diseases and Sciences Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Andrade C: A peculiar form of peripheral neuropathy. Familiar atypical generalized amyloidosis with special involvement of the peripheral nerves. Brain 75:408–427, 1952

    PubMed  Google Scholar 

  2. Andersson R: Familial amyloidosis with polyneuropathy: A clinical study based on patients living in northern Sweden. Acta Med Scand 198(suppl 590):1–64, 1976

    Google Scholar 

  3. Saraiva MJ, Costa PP, Birken S, Goodman DS: Presence of an abnormal transthyretin (prealbumin) in Portuguese patients with familial amyloidotic polyneuropathy. Trans Assoc Am Physicians 96:261–270, 1983

    PubMed  Google Scholar 

  4. Coutinho P, Martins da Silva A, Kopes Lima J, Resende Barbosa A: Forty years of experience with type I amyloid neuropathy. Review of 483 cases.In Amyloid and Amyloidosis. G Glenner, P Pinho e Costa, A Folcao de Freitas (eds). Amsterdam. Excerpta Medica, 1980, pp 88–98

    Google Scholar 

  5. Olofsson B-O: Cardiac involvement in familial amyloidosis with polyneuropathy. Int J Cardiol 4:71–73, 1983

    PubMed  Google Scholar 

  6. Olofsson B-O, Suhr O, Niklasson U, Wiklund U, Bjerle P, Beckman A: Assessment of autonomic nerve function in familial amyloidotic polyneuropathy: A clinical study based on spectral analysis of heart rate variability. Amyloid Int J Exp Clin Invest 1:138–142, 1994

    Google Scholar 

  7. Ikeda S, Yanagisawa N, Hongo M, Ito N: Vagus nerve and celiac ganglion lesions in generalized amyloidosis. A correlative study of familial amyloid polyneuropathy and AL-amyloidosis. J Neurol Sci 79:129–139, 1987

    PubMed  Google Scholar 

  8. Bergfeldt BL, Olofsson BO, Edhag KO: Electrophysiologic evaluation of the cardiac conduction system and its autonomic regulation in familial amyloid polyneuropathy. Am J Cardiol 56:647–652, 1985

    PubMed  Google Scholar 

  9. Steen L, Ek B: Familial amyloidosis with polyneuropathy. A long-term follow up of 21 patients with special reference to gastrointestinal symptoms. Acta Med Scand 214:387–397, 1984

    Google Scholar 

  10. Suhr O, Danielsson Å, Holmgren G, Steen L: Malnutrition and gastrointestinal dysfunction as prognostic factors for survival in familial amyloidotic polyneuropathy. J Intern Med 235:479–485, 1994

    PubMed  Google Scholar 

  11. Holmgren G, Ericzon B-G, Groth C-G, Steen L, Suhr O, Andersson O, Wallin B, Seymor A, Richardson S, Hawkins P, Pepys M: Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis. Lancet 341:1113–1116, 1993

    PubMed  Google Scholar 

  12. Lewis W, Skinner M, Simms R, Jones L, Cohen A, Jenkins R: Orthotopic liver transplantation for familial amyloidotic polyneuropathy. Clin Transplant 8:107–110, 1994

    PubMed  Google Scholar 

  13. Steen L, Holmgren G, Suhr O, Wikström L, Groth C-G, Ericzon BG: Worldwide survey of liver transplantation in patients with familial amyloidotic polyneuropathy. Amyloid Int J Exp Clin Invest 1:138–142, 1994

    Google Scholar 

  14. Suhr O, Holmgren G, Steen L, Wikström L, Nordén G, Friman S, Duraj F, Groth C-G, Ericzon B-G: Liver transplantation in familial amyloidotic polyneuropathy. Follow-up of the first 20 Swedish patients. Transplantation 60:933–938, 1995

    PubMed  Google Scholar 

  15. Suhr O, Holmgren G: Difficulties in clinical diagnosis and prediction of outcome in patients with transthyretin Met 30 mutation: Report of two cases. Amyloid Int J Exp Clin Invest 2:179–182, 1995

    Google Scholar 

  16. Drugge U, Andersson R, Chizari F, Danielsson M, Holmgren G, Sandgren O, Sousa A: Familial amyloidotic polyneuropathy in Sweden: A pedigree analysis. J Med Genet 30:388–392, 1993

    PubMed  Google Scholar 

  17. Holmgren G, Costa P, Andersson C, Asplund K, Steen L, Beckman L, Nylander P-O, Teixeira A, Saraiva M, Costa P: Geographical distribution of TTR met30 carriers in northern Sweden: Discrepancy between carrier frequency and prevalence rate. J Med Genet 31:351–354, 1994

    PubMed  Google Scholar 

  18. Holmgren G, Holmberg E, Lindström A, Lindström E, Nordenson I, Sandgren O, Steen L, Svensson B, Lundgren E, von Gabain A: Diagnosis of familial amyloidotic polyneuropathy in Sweden by RFLP analysis. Clin Genet 33:176–180, 1988

    PubMed  Google Scholar 

  19. Suhr O, Danielsson Å, Nyhlin H, Truedsson H: Bile acid malabsorption demonstrated by SeHCAT in chronic diarrhoea, with special reference to the impact of cholecystectomy. Scand J Gastroenterol 23:1187–1194, 1988

    PubMed  Google Scholar 

  20. Kerlin P, Wong L: Breath hydrogen testing in bacterial over-growth of the small intestine. Gastroenterology 95:982–988, 1988

    PubMed  Google Scholar 

  21. van de Kamer J, Huinink B, Ten B, Weyers H: Rapid method for the determination of fat in faeces. J Biol Chem 177:347–355, 1947

    Google Scholar 

  22. Suhr O, Danielsson Å, Steen L, Bile acid malabsorption caused by gastrointestinal motility dysfunction? An investigation of patients with familial amyloidosis with polyneuropathy. Scand J Gastroenterol 27:201–207, 1992

    PubMed  Google Scholar 

  23. El-Salhy M, Suhr O, Stenling R, Wilander E, Grimelius L: Impact of familial amyloid associated polyneuropathy on duodenal endocrine cells. Gut 35:1413–1418, 1994

    PubMed  Google Scholar 

  24. El-Salhy M, Suhr O: Endocrine cells in rectal biopsies from patients with familial amyloidotic polyneuropathy. Scand J Gastroenterol 31:68–73, 1996

    PubMed  Google Scholar 

  25. Suhr O, Danielsson Å, Hörstedt P, Stenling R: Bacterial contamination of the small bowel evaluated by breath tests,75Selabeled homocholic tauro acid and scanning electron microscopy. Scand J Gastroenterol 25:841–852, 1990

    PubMed  Google Scholar 

  26. King CE, Toskes PP: Comparison of the 1-gram [14C]xylose, 10-gram lactulose-H2, and 80-gram glucose-H2 breath tests in patients with small intestine bacterial overgrowth. Gastroenterology 91:1447–1451, 1986

    PubMed  Google Scholar 

  27. Gorazza G, Menozzi M, Strocchi A, Rasciti L, Vaira D, Lecchini R, Avanzini P, Chezzi C, Gasbarrini G: The diagnosis of small bowel bacterial overgrowth. Reliability of jejunal culture and inadequacy of breath hydrogen testing. Gastroenterology 98:302–309, 1990

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. From the Department of Medicine, Section for Gastroenterology and Department of Diagnostic Radiology, Umeå University Hospital, Umeå, Sweden

    Ole Suhr MD, PhD, Åke Danielsson MD, PhD, Anders Rydh MD, Nils Nyhlin MS, Sven Ola Hietala MD, PhD & Lars Steen MD, PhD

Authors
  1. Ole Suhr MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Åke Danielsson MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Anders Rydh MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Nils Nyhlin MS
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Sven Ola Hietala MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Lars Steen MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

The study was supported by grants from the patients association FAMY, Umeå Health District, the Swedish Medical Research Council, grant 19X-11240, Umeå University and the Joint Committee of the Northern Health Districts.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Suhr, O., Danielsson, Å., Rydh, A. et al. Impact of gastrointestinal dysfunction on survival after liver transplantation for familial amyloidotic polyneuropathy. Digest Dis Sci 41, 1909–1914 (1996). https://doi.org/10.1007/BF02093589

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02093589

Key words

Search

Navigation