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Diagnostic and prognostic utility of cardiovascular magnetic resonance imaging in light-chain cardiac amyloidosis
The American Journal of Cardiology, 02/13/09
Ruberg FL et al. – Study concludes that in patients with systemic amyloidosis, late gadolinium enhancement (LGE) is highly sensitive and specific for the identification of cardiac involvement but does not predict survival. LGE is strongly correlated with heart failure severity as assessed by B-type natriuretic peptide (BNP).
Methods- Aim was to assess the diagnostic and prognostic significance of cardiovascular MRI in pts (n=28) with amyloid light-chain amyloidosis but unknown cardiac involvement
- The presence of cardiac amyloidosis was determined by separate clinical evaluation
- The performance of LGE for the prediction of cardiac amyloidosis and prognostic implications were determined
- LGE was observed in 68% pts; sensitivity, specificity, positive predictive value, and negative predictive value of LGE were 86%, 86%, 95%, and 67%, respectively
- During a median f/u period of 29 mo, there were 5 deaths (82% survival); LGE itself did not predict survival
- LGE volume was positively correlated with serum level of BNP
- Multivariate analysis: LGE volume was the strongest independent predictor of BNP
- BNP was correlated with New York Heart Association class
- Reduced right ventricular end-diastolic volume and stroke volume were associated with mortality
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Short and long-term outcome of treatment with high-dose melphalan and stem cell transplantation for multiple myeloma-associated AL amyloidosis
Annals of Hematology, 12/18/09
Clinical features and functional significance of the P369S/ R408Q variant in pyrin, the familial Mediterranean fever protein
Annals of Rheumatic Diseases, 12/11/09
MEFV mutations in Egyptian patients suffering from familial Mediterranean fever: analysis of 12 gene mutations
Rheumatology International, 10/16/09
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