ArticleSurgery today2011
Manifestations of transthyretin-related familial amyloidotic polyneuropathy: long-term follow-up of Japanese patients after liver transplantation.
Article in Surgery today, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 44 citations in OpenAlex.
- Ophthalmological Manifestations of Hereditary Amyloidosis due to Transthyretin: A Systematic Review.Journal of ophthalmology · 2026Review
- Impact of Non-Cardiac Clinicopathologic Characteristics on Survival in Transthyretin Amyloid Polyneuropathy.Neurology and therapy · 2020Article
- Amyloid deposition in a mouse model humanized at the transthyretin and retinol-binding protein 4 loci.Laboratory investigation; a journal of technical methods and pathology · 2018Article
- Diagnosis and management of transthyretin familial amyloid polyneuropathy in Japan: red-flag symptom clusters and treatment algorithm.Orphanet journal of rare diseases · 2018Review
- Semi-quantitative models for identifying potent and selective transthyretin amyloidogenesis inhibitors.Bioorganic & medicinal chemistry letters · 2017Article
- Outcome of gastric emptying and gastrointestinal symptoms after liver transplantation for hereditary transthyretin amyloidosis.BMC gastroenterology · 2015Article
- Recent advances in transthyretin amyloidosis therapy.Translational neurodegeneration · 2014Review
- Tafamidis.Nature reviews. Drug discovery · 2012Article
- Familial amyloidotic polyneuropathy: current and emerging treatment options for transthyretin-mediated amyloidosis.The application of clinical genetics · 2012Article
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Authors and funding
13 authors at 3 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
purposeTo observe which symptoms of transthyretin-related familial amyloidotic polyneuropathy (FAP) progressed in the long term after liver transplantation (LT), focusing on cardiac, kidney, and ocular symptoms.
methodsWe reviewed the medical records of 34 Japanese patients with FAP, who underwent LT between 1994 and 2006. The mean follow-up period (± SD) after LT was 9.6 ± 3.4 years. Of the 34 patients, 30 had FAP amyloidogenic transthyretin (ATTR) Val30Met, 1 had FAP ATTR Ser50Ile, and 3 had FAP ATTR Tyr114Cys.
resultsThe 10-year survival rates from the onset of FAP and from the time of LT were 100% and 91.4%, respectively. Progression of ocular amyloidosis was seen in 17 (50%) patients, 13 of whom had de novo amyloid deposits in the vitreous body; progression of cardiac amyloidosis was seen in 10 (29%) patients, 4 of whom had newly granular sparkling echo on echocardiography, and 9 of whom had newly implanted pacemakers or implantable cardioverter-defibrillators. Although the mean serum creatinine levels did not increase significantly after LT in any of the patients, the estimated glomerular filtration rate had decreased significantly by 7 years after LT.
conclusionAlthough LT is life-saving for patients with FAP, we observed progression of the ocular and cardiac symptoms of FAP in a significant number of these patients over the long term after LT.
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