Liver transplantation for hereditary transthyretin amyloidosis
نویسندگان
چکیده
منابع مشابه
Liver transplantation and transthyretin amyloidosis.
Liver transplantation as a specific treatment of transthyretin amyloidosis was first performed in 1990. The rationale for this treatment was that removal of the source (liver) of the amyloid precursor protein (mutated transthyretin) would stop progression of the disease. Indeed, after orthotopic liver transplantation (OLT), mutant transthyretin (TTR) is rapidly cleared from circulation. In the ...
متن کاملOutcome of gastric emptying and gastrointestinal symptoms after liver transplantation for hereditary transthyretin amyloidosis
BACKGROUND Hereditary transthyretin amyloid (ATTR) amyloidosis is a rare but fatal autosomal dominant condition that is present all over the world. A liver transplantation has been shown to halt the progress of the disease in selected patients and is currently considered to be the standard treatment. Gastrointestinal manifestations are common in hereditary ATTR amyloidosis and are important for...
متن کامل[Familial approach in hereditary transthyretin cardiac amyloidosis].
Cardiac amyloidosis is a disease of complex diagnosis and treatment. Some subtypes of cardiac amyloidosis are inherited. Among these, the most common variant is caused by mutations in the transthyretin gene. Correct identification of amyloidosis produced by a genetic defect is of great importance because it modifies the diagnostic and therapeutic approach in patients and their families. We desc...
متن کاملHereditary Transthyretin Amyloidosis in Eight Chinese Families
BACKGROUND Mutations of transthyretin (TTR) cause the most common type of autosomal-dominant hereditary systemic amyloidosis, which occurs worldwide. To date, more and more mutations in the TTR gene have been reported. Some variations in the clinical presentation are often observed in patients with the same mutation or the patients in the same family. The purpose of this study was to find out t...
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ژورنال
عنوان ژورنال: Liver Transplantation
سال: 2000
ISSN: 1527-6465,1527-6473
DOI: 10.1053/lv.2000.6145