End-stage renal disease in patients with Fabry disease: natural history data from the Fabry Registry
Nephrology Dialysis Transplantation, 11/05/2009
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Ortiz A et al. – While all Fabry patients are at risk of cardiovascular events and strokes, patients with Fabry nephropathy who develop kidney failure appear to have concurrent involvement of ... important that Fabry patients are diagnosed early and that their renal function is monitored carefully.
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Scoring system for renal pathology in Fabry disease: report of the International Study Group of Fabry Nephropathy (ISGFN)
Nephrology Dialysis Transplantation, 10/16/2009
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Fogo AB et al. – The development of a standardized scoring system of both disease–specific lesions, i.e. lipid deposition related, and general lesions of ... clinical stage of Fabry nephropathy. These findings support the role of kidney biopsy in the baseline evaluation of Fabry nephropathy, even with mild clinical disease. The scoring system will be useful for longitudinal assessment of prognosis and responses to therapy for Fabry nephropathy
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Evidence for a role of sphingosine-1 phosphate in cardiovascular remodelling in Fabry disease
European Heart Journal, 09/28/2009
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Brakch N et al. – Sphingosine–1 phosphate participates in cardiovascular remodelling in Fabry disease. These findings have implications for the treatment of cardiovascular involvement in Fabry disease.
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Effect of agalsidase alfa replacement therapy on fabry disease—related hypertrophic cardiomyopathy: A 12- to 36-month, retrospective, blinded echocardiographic pooled analysis
Clinical Therapeutics, 10/29/2009
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Kampmann C et al. – Treatment with agalsidase alfa for 12 or 36 months was associated with reduced LVM in these patients with Fabry disease with baseline ... appeared to stabilize LVM in these patients without baseline LVH.
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Enzyme replacement therapy with agalsidase alfa in patients with Fabry's disease: an analysis of registry data
The Lancet, 12/15/2009
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Mehta A et al. – By comparison with historical natural history data for patients with Fabry's disease who were not treated with enzyme replacement therapy, long–term treatment ... benefits.
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Enzyme replacement therapy with agalsidase alfa in patients with Fabry's disease: an analysis of registry data
The Lancet - Early Online Publication, 12/02/2009
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Mehta A et al. – By comparison with historical natural history data for patients with Fabry's disease who were not treated with enzyme replacement therapy, long–term treatment ... benefits.
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Aortic remodelling in Fabry disease
European Heart Journal, 10/26/2009
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Barbey F et al. – Fabry disease should be considered as a cardiovascular disease that affects the heart and arterial vasculature, including the thoracic aorta. Thus, patients with FD should be closely monitored for the presence, and possible progression ... aortic dilation.
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Shire Targets Fourth Quarter Filing of BLA for REPLAGAL? for Fabry Disease with U.S. FDA
Shire, 10/22/2009
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Shire plc, the global specialty biopharmaceutical company, announces plans to file a Biologics License Application with the U.S. Food and Drug Administration for REPLAGAL, its enzyme replacement therapy for Fabry disease, by the end of the year
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A case of minimal change disease in a Fabry patient
Pediatric Nephrology, 11/04/2009
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Zarate YA et al. – The authors recommend considering immunotherapy in addition to enzyme replacement therapy in those patients with confirmed Fabry disease and acute nephrotic syndrome with clinical and microscopic findings suggestive of
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Autonomic skin responses in females with Fabry disease
Journal of the Peripheral Nervous System, 11/24/2009
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Moller AT et al. – The findings suggest that female patients both have an impaired C–fiber function and local abnormalities in blood vessels and sweat glands.
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