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( Maria Adela Aguirre ),( Maria Lourdes Posadas Martinez ),( Melisa Blomberg ),( Dorotea Beatriz Fantl ),( Diego Hernan Giunta ),( Fernan Gonzalez Bernaldo De Quiroz ),( Maria Florencia Grande Ratti ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Background: Cardiac involvement is the leading cause of morbidity and mortality of amy-loidosis, especially due to light-chain (AL) and transthyretin, both wild and hereditary types. Objectives: To estimate the prevalence and mortality of patients with evidence of cardiac involvement in patients diagnosed with amyloidosis. Methods: Ambispective cohort study, with data obtained from all patients included between 01/2007 and 03/2013 with evidence of amyloidosis in the Institutional Regis-try Amyloidosis of the Hospital Italiano de Buenos Aires. The diagnostic criteria of cardiac amyloidosis were predefined. Mortality from cardiac amyloidosis was measured using a combination of active and passive assessment. Survival time was evaluated using the Kaplan-Meier estimator. SPSS 19.0 was used. Results: The registry included 126 patients and the prevalence of cardiac amyloidosis was 40% (95%, CI:31-49%). Among the 40% (50/126) of patients with confirmed amyloidosis and suspected of cardiac involvement, the median age was 72 years (IQR 79-81) and 26% were women (13/50). Suspected diagnosis of cardiac involvement was: clinical 82.4% (42/50), from complementary studies 67% (34/50), and diagnosed from another hospital 6% (3/50). From all patients with suspected cardiac involvement, 47% had con- firmed biopsy. Overall mortality of patients with cardiac involvement was 20% (11/50), the median survival was 1782 days of follow up (CI 95% 1365-2199). Conclusions: The prevalence of cardiac involvement and mortality in patients with amyloidosis was high. Advances in diagnosis of the possible causes of amyloidosis in the future will allow the detection of patients with high risk of death from cardiac causes in our country.