234 Electrophysiological characteristics in cardiac amyloidosis due to familial amyloid polyneuropathy
نویسندگان
چکیده
منابع مشابه
Cardiac involvement in familial amyloidosis with polyneuropathy.
Cardiac involvement in familial amyloidosis with polyneuropathy. Peter Eriksson, Department of Internal Medicine, University of Umeå, S-901 85 Umeå, Sweden Familial amyloidosis with polyneuropathy (FAP) is a neuropathic form of heredofamilial systemic amyloidosis. Clusters of patients have been re ported predominantly from Portugal, Japan and Sweden. The present study examines the involvement ...
متن کاملFamilial amyloid polyneuropathy.
PURPOSE OF REVIEW Transthyretin familial amyloid polyneuropathy is the most disabling hereditary polyneuropathy of adult onset because of a point mutation of transthyretin gene. This review updates our knowledge about natural history of the disease, phenotypes, diagnosis tools for small and large fibers involvement, expert's consensus for both symptomatic and asymptomatic follow-up, and treatme...
متن کاملFamilial amyloid polyneuropathy.
Familial amyloid polyneuropathies (FAPs) are a group of life-threatening multisystem disorders transmitted as an autosomal dominant trait. Nerve lesions are induced by deposits of amyloid fibrils, most commonly due to mutated transthyretin (TTR). Less often the precursor of amyloidosis is mutant apolipoprotein A-1 or gelsolin. The first identified cause of FAP-the TTR Val30Met mutation-is still...
متن کاملTreatment by pacemaker in familial amyloid polyneuropathy.
Amyloid deposits often involve the heart and cause disturbances in conduction and impulse formation in patients with familial amyloid polyneuropathy (FAP). Seven patients with FAP required pacemaker treatment during eight years. The most frequent bradyarrhythmias requiring pacing were sinus node dysfunction with junctional failure. Our seven patients had attacks and symptoms of bradyarrhythmias...
متن کاملEpidemiology of Familial Amyloid Polyneuropathy in Bulgaria
Methods Four mutations have been found in the country so far: Glu89Gln, Ser77Phe, Val30Met, Ser52Pro. Glu89Gln is the most frequent mutation affecting 48 different families. Selective genetic screening program is performed in the affected families. A total of 261 individuals belonging to affected families were examined. All individuals who took part in the screening program signed an informed c...
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ژورنال
عنوان ژورنال: Archives of Cardiovascular Diseases Supplements
سال: 2012
ISSN: 1878-6480
DOI: 10.1016/s1878-6480(12)70630-4