Progressive cardiac amyloidosis following liver transplantation for familial amyloid polyneuropathy : Implications for amyloid fibrillogenesis

摘要: Background. Circulating transthyretin (TTR) is derived from the liver, and orthotopic liver transplantation (OLT) widely performed for variant TTR-associated familial amyloid polyneuropathy (FAP). The effect of OLT on FAP-related cardiac particular interest because wild-type TTR can itself be deposited as senile amyloid. Methods. Serial echocardiography was in 20 FAP patients, 14 whom underwent OLT, 10 other transplant patients. Follow-up included serum P component scintigraphy measurement plasma before after OLT. Results. Cardiac amyloidosis progressed rapidly three patients (TTR Pro52 Thr84 mutations) even though deposits elsewhere had stabilized or regressed. Results improved with Met30 remained normal seven Plasma levels were altered substantially but they did not reflect findings. Conclusions. Although deposition generally inhibited exacerbated, probably due to enhanced a template TTR. phenomenon may mutation-dependent. These findings suggest that formation de novo its subsequent accumulation promoted by different factors, which organ-specific.