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- W2759564741 abstract "Fabry’s disease (FD) is an inherited lysosomal storage diseaselinked to chromosome X, resulting from mutations in the gene for theenzyme α-galactosidase A, its deficiency causes the accumulationof globotriacilceramide. Objective: To evaluate the effect on bloodpressure in patients with FD with enzymatic replacement therapy withagalsidase B. Method: In this prospective study, for 20 months, twomales patients with FD, one of them in hemodialysis, during that periodin each patient was assessed blood pressure during infusion of theenzyme, with each patient was his own control, and compared the resultsbetween them. Results: There was a significant decrease in bloodpressure, being more evident in patient on hemodialysis and a negativecorrelation between the number of infusions and the average arterialpressure; during the period of study, in ASL patient the values ofdiastolic and mean arterial pressures showed a greater variation thanin patient CSL. Conclusion: ERT seems to generate a systemicvasodilatation that could control hypertension induced by chronic renalfailure secondary to FD, and is well tolerated in view that there wereno adverse reactions, despite signs of hypotension." @default.
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- W2759564741 date "2013-04-30" @default.
- W2759564741 modified "2023-09-23" @default.
- W2759564741 title "Behaviour of Blood Pressure during Agalsidase B infusion inFabry’s disease" @default.
- W2759564741 hasPublicationYear "2013" @default.
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