Orthotopic liver transplantation for hereditary fibrinogen amyloidosis

S. Zeldenrust, Morie Gertz, T. Uemichi, J. Björnsson, R. Wiesner, T. Schwab, M. Benson

Research output: Contribution to journalArticle

49 Citations (Scopus)

Abstract

Systemic amyloidosis results from the deposition of insoluble protein fibrils in various organs and tissues. To date, several different proteins have been associated with amyloid fibril formation, including immunoglobulin light chain, serum amyloid A protein, and transthyretin. Recent reports have shown that variant fibrinogen chains can form amyloid in certain kindreds. Hepatic transplantation has previously been reported in the treatment of hereditary amyloidosis associated with variant transthyretin proteins, which are mainly synthesized in the liver. This article reports the first use and long-term follow-up of combined hepatic and renal transplantation in the successful treatment of two patients with hereditary fibrinogen amyloidosis. Both patients experienced sustained improvement in renal function and nutritional status at 61/2 years and 28 months of follow-up, respectively. Orthotopic liver transplantation is effective and potentially curative treatment of hereditary fibrinogen amyloidosis.

Original languageEnglish (US)
Pages (from-to)560-561
Number of pages2
JournalTransplantation
Volume75
Issue number4
DOIs
StatePublished - Feb 27 2003

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Familial Amyloidosis
Liver Transplantation
Fibrinogen
Prealbumin
Amyloid
Immunoglobulin Light Chains
Serum Amyloid A Protein
Proteins
Amyloidosis
Nutritional Status
Kidney Transplantation
Therapeutics
Kidney
Liver

ASJC Scopus subject areas

  • Transplantation
  • Immunology

Cite this

Zeldenrust, S., Gertz, M., Uemichi, T., Björnsson, J., Wiesner, R., Schwab, T., & Benson, M. (2003). Orthotopic liver transplantation for hereditary fibrinogen amyloidosis. Transplantation, 75(4), 560-561. https://doi.org/10.1097/01.TP.0000046526.10003.EC

Orthotopic liver transplantation for hereditary fibrinogen amyloidosis. / Zeldenrust, S.; Gertz, Morie; Uemichi, T.; Björnsson, J.; Wiesner, R.; Schwab, T.; Benson, M.

In: Transplantation, Vol. 75, No. 4, 27.02.2003, p. 560-561.

Research output: Contribution to journalArticle

Zeldenrust, S, Gertz, M, Uemichi, T, Björnsson, J, Wiesner, R, Schwab, T & Benson, M 2003, 'Orthotopic liver transplantation for hereditary fibrinogen amyloidosis', Transplantation, vol. 75, no. 4, pp. 560-561. https://doi.org/10.1097/01.TP.0000046526.10003.EC
Zeldenrust S, Gertz M, Uemichi T, Björnsson J, Wiesner R, Schwab T et al. Orthotopic liver transplantation for hereditary fibrinogen amyloidosis. Transplantation. 2003 Feb 27;75(4):560-561. https://doi.org/10.1097/01.TP.0000046526.10003.EC
Zeldenrust, S. ; Gertz, Morie ; Uemichi, T. ; Björnsson, J. ; Wiesner, R. ; Schwab, T. ; Benson, M. / Orthotopic liver transplantation for hereditary fibrinogen amyloidosis. In: Transplantation. 2003 ; Vol. 75, No. 4. pp. 560-561.
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