A Case of Familial Cardiac Amyloidosis
Korean Circulation Journal
;
: 520-526, 2004.
Artigo
em Coreano
| WPRIM
| ID: wpr-133529
ABSTRACT
Amyloidosis is defined by the extracellular deposition of fibrillar proteinacious material that binds Congo red dye. Amyloid fibrils can be deposited locally, but can involve virtually every organ system of the body. Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen Aalpha-chain, lysozyme or apolipoprotein A-I, is-extremely rare. A case of familial amyloidosis, involving the heart, was identified, where the patient complained exertional dyspnea. The echocardiographic findings were the markedly thickened the interventricular septum and right ventricular wall, as well as a granular sparkling appearance in the interventricular septum. On admission, the patient, and his younger brother, underwent endomyocardial biopsies, and the results of the Congo red staining and EM were consistent with amyloidosis. The patient was managed conservatively, and discharged without complication.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Biópsia
/
Fibrinogênio
/
Pré-Albumina
/
Ecocardiografia
/
Muramidase
/
Apolipoproteína A-I
/
Vermelho Congo
/
Amiloidose Familiar
/
Irmãos
/
Dispneia
Tipo de estudo:
Estudo prognóstico
Limite:
Humanos
Idioma:
Coreano
Revista:
Korean Circulation Journal
Ano de publicação:
2004
Tipo de documento:
Artigo
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