Progression of Fabry cardiomyopathy despite enzyme replacement therapy.
نویسندگان
چکیده
Maurizio Pieroni, Antonia Camporeale, Roberta Della Bona, Alessandra Sabini, Deborah Progression of Fabry Cardiomyopathy Despite Enzyme Replacement Therapy Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 2013 American Heart Association, Inc. All rights reserved. is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Circulation doi: 10.1161/CIRCULATIONAHA.113.002799 2013;128:1687-1688 Circulation. http://circ.ahajournals.org/content/128/15/1687 World Wide Web at: The online version of this article, along with updated information and services, is located on the
منابع مشابه
Long-term effects of enzyme replacement therapy on fabry cardiomyopathy: evidence for a better outcome with early treatment.
BACKGROUND Enzyme replacement therapy with recombinant alpha-galactosidase A reduces left ventricular hypertrophy and improves regional myocardial function in patients with Fabry disease during short-term treatment. Whether enzyme replacement therapy is effective in all stages of Fabry cardiomyopathy during long-term follow-up is unknown. METHODS AND RESULTS We studied 32 Fabry patients over ...
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Fabry disease is a rare, inherited disease with lack of the enzyme alpha-galactosidase A (α-Gal) in the cells of the body that participates in the breakdown of fat. The disease begins in early childhood, progresses slowly throughout life and results in severe damage of the kidneys, heart and central nervous system. The disease is life-threatening and if left untreated, death ...
متن کاملDialysis and transplantation in Fabry disease: indications for enzyme replacement therapy.
ESRD is a major cause of morbidity and premature mortality in Fabry disease, particularly in classically affected males. The decline of renal function in Fabry nephropathy is adversely affected by male gender, advanced chronic kidney disease (CKD), and severe proteinuria. The diagnosis of Fabry nephropathy may be missed if not specifically addressed in progressive CKD and patients have been fir...
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cement therapy in Fabry’s disease. N Engl J Med 2001;345:9–16. 17. Weidemann F, Breunig F, Beer M, Sandstede J, Turschner O, Voelker W, Ertl G, Knoll A, Wanner C, Strotmann JM. Improvement in cardiac function during enzyme replacement therapy in patients with Fabry disease: a prospective strain rate imaging study. Circulation 2003;108:1299–1301. 18. Hughes DA, Eliott PM, Shah J, Zuckerman J, Co...
متن کاملLeft Ventricular Geometry and Blood Pressure as Predictors of Adverse Progression of Fabry Cardiomyopathy.
BACKGROUND In spite of several research studies help to describe the heart in Fabry disease (FD), the cardiomyopathy is not entirely understood. In addition, the impact of blood pressure and alterations in geometry have not been systematically evaluated. METHODS In 74 FD patients (mean age 36±12 years; 45 females) the extent of myocardial fibrosis and its progression were quantified using car...
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عنوان ژورنال:
- Circulation
دوره 128 15 شماره
صفحات -
تاریخ انتشار 2013