Liver transplantation in familial amyloidotic polyneuropathy (FAP). A comparative study of transplanted and non-transplanted patient's survival
نویسندگان
چکیده
منابع مشابه
The impact of exercise training on liver transplanted familial amyloidotic polyneuropathy (FAP) patients.
BACKGROUND Liver transplantation is nowadays the only effective answer to adjourn the outcome of functional limitations associated with familial amyloidotic polyneuropathy (FAP), a neurodegenerative disease characterized by sensory and motor polyneuropathies. Nevertheless, there is a detrimental impact associated with the after-surgery period on the fragile physical condition of these patients....
متن کاملBody composition, muscle strength, functional capacity, and physical disability risk in liver transplanted familial amyloidotic polyneuropathy patients.
BACKGROUND Familial amyloidotic polyneuropathy (FAP) is a neurodegenerative disease leading to sensory and motor polyneuropathies, and functional limitations. Liver transplantation is the only treatment for FAP, requiring medication that negatively affects bone and muscle metabolism. The aim of this study was to compare body composition, levels of specific strength, level of physical disability...
متن کاملCombined heart and liver transplantation for familial amyloidotic polyneuropathy.
Familial amyloidotic polyneuropathy (FAP) is an inherited disease characterized by an abnormal systemic deposition of a mutant protein called transthyretin (TTR) with elective involvement of the peripheral nervous system, but often determining cardiac, gastrointestinal, and urinary tract dysfunction. FAP commonly affects the liver and the heart until end-organs failure. Transthyretin amyloidosi...
متن کاملA study of 159 Portuguese patients with familial amyloidotic polyneuropathy (FAP) whose parents were both unaffected.
We reviewed 1233 cases of familial amyloidotic polyneuropathy (FAP) from 489 Portuguese families registered at the Centro de Estudos de Paramiloidose, Porto, Portugal. It was found that in 159 cases, neither parent had shown symptoms of this hereditary dominant form of peripheral neuropathy. These cases appear to form a distinct group, with a later age at onset (mean 45.1 years, SD 12.0) than t...
متن کاملThe Coimbra University Hospital experience in liver transplantation in patients with familial amyloidotic polyneuropathy.
FAMILIAL amyloidotic polyneuropathy (FAP) is an inherited autosomal dominant systemic disease caused by an abnormal protein, the Transthyretin Methionine 30 (TTR Met 30, in the Portuguese variety methionine instead of valine). This abnormality is caused by a mutant gene in the 18th chromosome pair. The liver is the main site of production of the TTR Met 30 (more than 90%), and the largest affec...
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ژورنال
عنوان ژورنال: Transplant International
سال: 1998
ISSN: 0934-0874,1432-2277
DOI: 10.1111/j.1432-2277.1998.tb01104.x