Analysis of left ventricular mass in untreated men and in men treated with agalsidase-ß: data from the Fabry Registry.
Genet Med
; 15(12): 958-65, 2013 Dec.
Article
em En
| MEDLINE
| ID: mdl-23703683
ABSTRACT
PURPOSE:
The aim of this study was to evaluate the progression of left ventricular hypertrophy in untreated men with Fabry disease and to assess the effects of agalsidase-ß (recombinant human α-galactosidase A) on left ventricular hypertrophy.METHODS:
Longitudinal Fabry Registry data were analyzed from 115 men treated with agalsidase-ß (1 mg/kg/2 weeks) and 48 untreated men. Measurements included baseline left-ventricular mass and at least one additional left-ventricular mass assessment over ≥ 2 years. Patients were grouped into quartiles, based on left-ventricular mass slopes. Multivariate logistic regression analyses identified factors associated with left ventricular hypertrophy progression.RESULTS:
For men in whom treatment was initiated at the age of 18 to <30 years, mean left ventricular mass slope was -3.6 g/year (n = 31) compared with +9.5 g/year in untreated men of that age (n = 15) (P < 0.0001). Untreated men had a 3.4-fold higher risk of having faster increases in left-ventricular mass compared with treated men (odds ratio 3.43; 95% confidence interval 1.05-11.22; P = 0.0415). A baseline age of ≥ 40 years was also associated with left--ventricular hypertrophy progression (odds ratio 5.03; 95% confidence interval 1.03-24.49; P = 0.0457) compared with men younger than 30 years.CONCLUSION:
Agalsidase-ß treatment for ≥2 years may improve or stabilize left-ventricular mass in men with Fabry disease. Further investigations may determine whether early intervention and stabilization of LVM are correlated with clinical outcomes.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Doença de Fabry
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Hipertrofia Ventricular Esquerda
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Alfa-Galactosidase
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Isoenzimas
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
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Aged
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Humans
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Male
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Middle aged
Idioma:
En
Ano de publicação:
2013
Tipo de documento:
Article