Liver transplantation is the only definitive treatment for transthyretin amyloidosis, with an excellent
5-year survival in endemic countries where the Met30 mutation is predominant. We report our
experience of liver transplantation for transthyretin amyloidosis. We reviewed the clinical records
of 17 transplanted patients (11 males, 6 females; age at liver transplant: 45.7±11.7 years).
We had a wide spectrum of non-Met30 mutations (52.9%), with a predominance of Gln89 (23.5%).
Five-year survival after transplantation was 43.8%; at multivariate analysis, both non-Met30
mutations (HR 17.3, 95% CI 1.03-291.7) and modified BMI (HR 0.50, 95% CI 0.29-0.87) showed
significant and independent prognostic roles (P=0.048 and P=0.015, respectively). Five out of the 9
non-Met30 carriers received combined heart transplantation because of severe cardiomyopathy;
they showed a trend towards a better prognosis vs. the 4 patients who did not receive combined
heart transplantation (although not statistically significant; P=0.095). At follow-up, no significant
improvement of transthyretin amyloidosis manifestations was observed.
The results of liver transplantation for transthyretin amyloidosis in our population are poorer than
those reported in the literature probably because of the high prevalence of non-Met30 mutations.
| Identifer | oai:union.ndltd.org:unibo.it/oai:amsdottorato.cib.unibo.it:1128 |
| Date | 23 June 2008 |
| Creators | Berardi, Sonia <1974> |
| Contributors | Sama, Claudia |
| Publisher | Alma Mater Studiorum - Università di Bologna |
| Source Sets | Università di Bologna |
| Language | English |
| Detected Language | English |
| Type | Doctoral Thesis, PeerReviewed |
| Format | application/pdf |
| Rights | info:eu-repo/semantics/openAccess |
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