Enzyme replacement therapy administered during hemodialysis in patients with Fabry disease

Enzyme replacement therapy administered during hemodialysis in patients with Fabry disease.BackgroundEnzyme replacement treatment with recombinant human α-galactosidase A (r-hαGalA) is now available for patients with Fabry disease, many of whom are on maintenance hemodialysis. Because r-hαGalA must be infused over several hours, administering the enzyme during dialysis would save a day of treatment for patients receiving both therapies. However, these procedures have never been combined due to concerns about possible loss of enzyme in the dialysate.MethodsTen Fabry patients received r-hαGalA (1 mg/kg body weight continuously infused over 4 hours) during dialysis and separately in the interval between dialysis treatments. Plasma activity of r-hαGalA was measured at baseline and then every hour for both procedures. In two patients, a third r-hαGalA infusion during dialysis with a high-flux membrane was followed.ResultsThe rise in plasma concentrations of r-hαGalA during infusion and the steady-state levels reached were comparable for enzyme administrations with or without dialysis. The trend for the somewhat higher activities during hemodialysis was explained by volume contraction due to ultrafiltration. With the use of a high-flux dialyzer, the plasma r-hαGalA activities were identical to those that were observed during low-flux dialysis.ConclusionsAdministration of r-hαGalA during hemodialysis is not associated with a reduced activity of r-hαGalA therapy in patients with Fabry disease. Replacement therapy with r-hαGalA may therefore be performed during hemodialysis without apparent loss of enzyme into the dialysate

Evaluation der Lehre an der Ruhr-Universitaet Bochum Wintersemester 1991/92 durchgefuehrt im Auftrag des Rektorats der Ruhr-Universitaet in Zusammenarbeit mit der Universitaetskommission fuer Lehre Studium und Studienreform ; 1. Bericht zur Erhebung

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