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Long-term efficacy and safety of pegunigalsidase alfa administered every 4 weeks in adults with Fabry disease: results from up to 5 years of the BRIGHT F51 phase III, open-label extension study.

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Related Experiment Video

Updated: Jun 17, 2026

In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease
10:16

In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease

Published on: December 20, 2017

Enzyme replacement therapy and Fabry nephropathy.

David G Warnock1, Erica Daina, Giuseppe Remuzzi

  • 1Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama 35294-0007, USA. dwarnock@uab.edu

Clinical Journal of the American Society of Nephrology : CJASN
|December 17, 2009
PubMed
Summary
This summary is machine-generated.

Fabry nephropathy, a kidney disease, is treatable. Combining enzyme replacement therapy with angiotensin receptor blockers or ACE inhibitors is crucial for managing proteinuria and stabilizing kidney function.

Related Experiment Videos

Last Updated: Jun 17, 2026

In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease
10:16

In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease

Published on: December 20, 2017

Area of Science:

  • Nephrology
  • Genetic Disorders
  • Pharmacology

Background:

  • Kidney involvement (nephropathy) is common in Fabry disease for both males and females.
  • Progressive nephropathy often leads to significant proteinuria, declining kidney function, and end-stage renal disease (ESRD).
  • Physicians may overlook "normal" creatinine or "minimal" proteinuria, delaying proper assessment and management of Fabry nephropathy.

Purpose of the Study:

  • To highlight the treatability of Fabry nephropathy, even in advanced stages.
  • To emphasize the need for comprehensive management strategies beyond enzyme replacement therapy.
  • To present a systematic approach for stabilizing kidney function in Fabry disease patients.

Main Methods:

  • Enzyme replacement therapy (ERT) with agalsidase as a cornerstone treatment.
  • Addition of angiotensin receptor blockers (ARBs) or angiotensin-converting enzyme inhibitors (ACEIs) to ERT.
  • Regular monitoring of kidney function (eGFR) and urinary protein excretion at clinic visits.

Main Results:

  • ERT alone does not effectively reduce urine protein excretion.
  • Combined therapy (ERT + ARBs/ACEIs) is necessary to decrease proteinuria and stabilize kidney function.
  • Antiproteinuric therapy can be guided by urine protein targets, often leading to blood pressure reduction.

Conclusions:

  • Fabry nephropathy requires a multi-faceted treatment approach.
  • Aggressive management of proteinuria is key to slowing GFR decline.
  • The goal is to reduce GFR loss to -1 ml/min/1.73 m(2)/yr, mirroring normal aging.