Document Detail

Manifestations of transthyretin-related familial amyloidotic polyneuropathy: Long-term follow-up of Japanese patients after liver transplantation.
MedLine Citation:
PMID:  21874417     Owner:  NLM     Status:  In-Data-Review    
PURPOSE: To observe which symptoms of transthyretinrelated familial amyloidotic polyneuropathy (FAP) progressed in the long term after liver transplantation (LT), focusing on cardiac, kidney, and ocular symptoms.
METHODS: We 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.
RESULTS: The 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.
CONCLUSION: Although 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.
Yuki Ohya; Sadahisa Okamoto; Masayoshi Tasaki; Mitsuharu Ueda; Hirofumi Jono; Konen Obayashi; Kazuhisa Takeda; Hideaki Okajima; Katsuhiro Asonuma; Ryuhei Hara; Hidenobu Tanihara; Yukio Ando; Yukihiro Inomata
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Publication Detail:
Type:  Journal Article     Date:  2011-08-26
Journal Detail:
Title:  Surgery today     Volume:  41     ISSN:  1436-2813     ISO Abbreviation:  Surg. Today     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-08-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9204360     Medline TA:  Surg Today     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  1211-8     Citation Subset:  IM    
Department of Transplantation and Pediatric Surgery, Kumamoto University, 1-1-1 Honjo, Kumamoto, 860-8556, Japan.
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