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09 June 2017 : Case report  Slovenia

Paricalcitol as an Antiproteinuric Agent Can Result in the Deterioration of Renal and Heart Function in a Patient with Fabry Disease

Unusual or unexpected effect of treatment, Diagnostic / therapeutic accidents, Rare disease, Adverse events of drug therapy , Congenital defects / diseases, Clinical situation which can not be reproduced for ethical reasons

Tajda Keber1ABCDEF*, Martin Tretjak1BCDEF, Andreja Cokan Vujkovac1BDEF, Marija Mravljak1DE, Katja Ravber1DE, Bojan Vujkovac1ABCDEFG

DOI: 10.12659/AJCR.903886

Am J Case Rep 2017; 18:644-648

Abstract

BACKGROUND: Fabry disease is a rare and progressive X-linked inherited disorder of glycosphingolipid metabolism that is due to deficient or absent lysosomal a-galactosidase A activity. Among its other associated signs and symptoms, patients present with renal failure and proteinuria, which are markers of disease progression. Renin-angiotensin-aldosterone system (RAAS) blockers can slow the progression of chronic renal failure and proteinuria. In fact, some studies have shown the beneficial effects of paricalcitol on proteinuria.

CASE REPORT: We present a case of a female patient with the classic variant of Fabry disease. She was treated with a high dose of paricalcitol as an antiproteinuric agent due to unsatisfactory double-RAAS blockage, which resulted in transient worsening of cardiac and renal function.

CONCLUSIONS: Despite the positive effects of paricalcitol as an antiproteinuric agent, as previously shown by some authors, our case highlights the possible serious adverse effects associated with the use of high doses of this drug.

Keywords: 25-Hydroxyvitamin D 2, Fabry Disease, Heart Failure, Diastolic, Proteinuria, renal insufficiency

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923