Antiproteinuric Agents and Fabry Disease

The University of Alabama at Birmingham

Status: Completed

Conditions: Fabry Disease; Proteinuria

Study type: Observational

Funder types: Other

Identifiers

NCT00343577

X050202007

Details and patient eligibility

About

Fabry disease is a rare disorder that often has kidney involvement with increased urine protein excretion. Proteinuria is recognized as an important risk factor for progression of chronic kidney disease. Our hypothesis is that using drugs that reduce urine protein excretion (ACE inhibitors and ARBs) will have a beneficial effect on patients with Fabry disease who already are receiving enzyme replacement therapy. A longitudinal, observational study is being undertaken to determine the utility of these agents in Fabry disease, realizing that these agents are primarily indicated for reducing systemic blood pressure, and most patients with Fabry disease have relatively low blood pressures at baseline.

Full description

Fabry disease is a rare disorder that often has kidney involvement with increased urine protein excretion. Proteinuria is recognized as an important risk factor for progression of chronic kidney disease. Our hypothesis is that using drugs that reduce urine protein excretion (ACE inhibitors and ARBs) will have a beneficial effect on patients with Fabry disease who already are receiving enzyme replacement therapy. A longitudinal, observational study is being undertaken to determine the utility of these agents in Fabry disease, realizing that these agents are primarily indicated for reducing systemic blood pressure, and most patients with Fabry disease have relatively low blood pressures at baseline.

Sex: All

Ages: 14 to 95 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• genetically confirmed Fabry disease
• institution of commercially available agalsidase-beta

Exclusion criteria

• s/p kidney transplant