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Antiproteinuric therapy and fabry nephropathy: sustained reduction of proteinuria in patients receiving enzyme replacement therapy with agalsidase-beta.
J Am Soc Nephrol. 2007 Sep; 18(9):2609-17.JA

Abstract

This report describes an open-label, nonrandomized, prospective evaluation of the effects of angiotensin-converting enzyme inhibitor and angiotensin receptor blocker therapy on patients who have Fabry disease and also received enzyme replacement therapy with agalsidase-beta, given at 1 mg/kg body wt every 2 wk. Previous placebo-controlled phase III and phase IV trials with agalsidase-beta demonstrated clearing of globotriaosylceramide from vascular endothelia but little effect on proteinuria or progressive loss of kidney function in patients with Fabry disease and severe chronic kidney disease marked by overt proteinuria and/or estimated GFR <60 ml/min per 1.73 m2. Angiotensin-converting enzyme inhibitor and/or angiotensin receptor blocker therapy is the standard of care for patients with proteinuric kidney diseases, but their use is challenging in patients with Fabry disease and low or low-normal baseline systemic BP. A group of patients with Fabry disease were treated with antiproteinuric therapy, in conjunction with agalsidase-beta; sustained reductions in proteinuria with stabilization of kidney function were achieved in a group of six patients who had severe Fabry nephropathy; the progression rate was -0.23 +/- 1.12 ml/min per 1.73 m2 per yr with 30 mo of follow-up.

Authors+Show Affiliations

Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, AL 35294-0006, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Journal Article

Language

eng

PubMed ID

17656478

Citation

Tahir, Hindia, et al. "Antiproteinuric Therapy and Fabry Nephropathy: Sustained Reduction of Proteinuria in Patients Receiving Enzyme Replacement Therapy With Agalsidase-beta." Journal of the American Society of Nephrology : JASN, vol. 18, no. 9, 2007, pp. 2609-17.
Tahir H, Jackson LL, Warnock DG. Antiproteinuric therapy and fabry nephropathy: sustained reduction of proteinuria in patients receiving enzyme replacement therapy with agalsidase-beta. J Am Soc Nephrol. 2007;18(9):2609-17.
Tahir, H., Jackson, L. L., & Warnock, D. G. (2007). Antiproteinuric therapy and fabry nephropathy: sustained reduction of proteinuria in patients receiving enzyme replacement therapy with agalsidase-beta. Journal of the American Society of Nephrology : JASN, 18(9), 2609-17.
Tahir H, Jackson LL, Warnock DG. Antiproteinuric Therapy and Fabry Nephropathy: Sustained Reduction of Proteinuria in Patients Receiving Enzyme Replacement Therapy With Agalsidase-beta. J Am Soc Nephrol. 2007;18(9):2609-17. PubMed PMID: 17656478.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Antiproteinuric therapy and fabry nephropathy: sustained reduction of proteinuria in patients receiving enzyme replacement therapy with agalsidase-beta. AU - Tahir,Hindia, AU - Jackson,Leslie L, AU - Warnock,David G, Y1 - 2007/07/26/ PY - 2007/7/28/pubmed PY - 2007/11/14/medline PY - 2007/7/28/entrez SP - 2609 EP - 17 JF - Journal of the American Society of Nephrology : JASN JO - J Am Soc Nephrol VL - 18 IS - 9 N2 - This report describes an open-label, nonrandomized, prospective evaluation of the effects of angiotensin-converting enzyme inhibitor and angiotensin receptor blocker therapy on patients who have Fabry disease and also received enzyme replacement therapy with agalsidase-beta, given at 1 mg/kg body wt every 2 wk. Previous placebo-controlled phase III and phase IV trials with agalsidase-beta demonstrated clearing of globotriaosylceramide from vascular endothelia but little effect on proteinuria or progressive loss of kidney function in patients with Fabry disease and severe chronic kidney disease marked by overt proteinuria and/or estimated GFR <60 ml/min per 1.73 m2. Angiotensin-converting enzyme inhibitor and/or angiotensin receptor blocker therapy is the standard of care for patients with proteinuric kidney diseases, but their use is challenging in patients with Fabry disease and low or low-normal baseline systemic BP. A group of patients with Fabry disease were treated with antiproteinuric therapy, in conjunction with agalsidase-beta; sustained reductions in proteinuria with stabilization of kidney function were achieved in a group of six patients who had severe Fabry nephropathy; the progression rate was -0.23 +/- 1.12 ml/min per 1.73 m2 per yr with 30 mo of follow-up. SN - 1046-6673 UR - https://www.unboundmedicine.com/medline/citation/17656478/Antiproteinuric_therapy_and_fabry_nephropathy:_sustained_reduction_of_proteinuria_in_patients_receiving_enzyme_replacement_therapy_with_agalsidase_beta_ L2 - https://jasn.asnjournals.org/cgi/pmidlookup?view=long&amp;pmid=17656478 DB - PRIME DP - Unbound Medicine ER -