Antiproteinuric therapy and Fabry nephropathy: factors associated with preserved kidney function during agalsidase-beta therapy

BackgroundNephropathy is an important feature of classical Fabry disease, which results in alpha-galactosidase A deficiency and cellular globotriaosylceramide accumulation. We report the safety and efficacy of antiproteinuric therapy with ACE inhibitors or angiotensin II receptor blockers (ARBs) in...

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Veröffentlicht in:	Journal of medical genetics 2015-12, Vol.52 (12), p.860-866
Hauptverfasser:	Warnock, David G, Thomas, Christie P, Vujkovac, Bojan, Campbell, Ruth C, Charrow, Joel, Laney, Dawn A, Jackson, Leslie L, Wilcox, William R, Wanner, Christoph
Format:	Artikel
Sprache:	eng
Schlagworte:	ACE inhibitors Adult Age alpha-Galactosidase - adverse effects alpha-Galactosidase - therapeutic use Angiotensin Receptor Antagonists - adverse effects Angiotensin Receptor Antagonists - therapeutic use Angiotensin-Converting Enzyme Inhibitors - adverse effects Angiotensin-Converting Enzyme Inhibitors - therapeutic use Biopsy Drug Therapy, Combination Fabry Disease - complications Fabry Disease - drug therapy Female Glomerular Filtration Rate Humans Isoenzymes - adverse effects Isoenzymes - therapeutic use Kidney - drug effects Kidney - physiopathology Kidney diseases Kidney Diseases - drug therapy Kidney Diseases - etiology Male Males Middle Aged Phenotypes Prospective Studies Proteins Rodents Treatment Outcome Urine
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Zusammenfassung:	BackgroundNephropathy is an important feature of classical Fabry disease, which results in alpha-galactosidase A deficiency and cellular globotriaosylceramide accumulation. We report the safety and efficacy of antiproteinuric therapy with ACE inhibitors or angiotensin II receptor blockers (ARBs) in a study of classical Fabry patients receiving recombinant agalsidase-beta therapy.Methods and designThe goal was maintenance of urine protein to creatinine ratio (UPCR)
ISSN:	0022-2593 1468-6244
DOI:	10.1136/jmedgenet-2015-103471