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The kidney in Fabry's disease.

A Pisani1, B Visciano, M Imbriaco

  • 1Department of Nephrology, Federico II University of Naples, Naples, Italy.

Clinical Genetics
|March 21, 2014
PubMed
Summary

Fabry disease (FD) causes kidney damage due to enzyme deficiency. Enzyme replacement therapy (ERT) is recommended for all patients with signs of renal involvement to slow disease progression.

Keywords:
Fabry diseaseFabry nephropathychronic kidney diseaseenzyme replacement therapy

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Area of Science:

  • Nephrology
  • Genetics
  • Metabolic Disorders

Background:

  • Fabry disease (FD) is an X-linked genetic disorder caused by mutations in the GLA gene.
  • This leads to a deficiency in α-galactosidase A enzyme activity.
  • This deficiency results in the accumulation of glycosphingolipids, primarily globotriaosylceramide, within lysosomes of multiple organs.

Purpose of the Study:

  • To review the progression of kidney disease in Fabry disease.
  • To discuss factors affecting renal function decline.
  • To emphasize the role of enzyme replacement therapy (ERT) in managing Fabry nephropathy.

Main Methods:

  • Review of existing literature on Fabry disease and nephropathy.
  • Analysis of factors influencing chronic kidney disease (CKD) progression in FD patients.
  • Evaluation of the efficacy of enzyme replacement therapy (ERT).

Main Results:

  • Fabry nephropathy progresses insidiously, similar to diabetic nephropathy, often leading to end-stage renal disease by the 50s in untreated males.
  • Renal function decline is exacerbated by male gender, advanced CKD, hypertension, and severe proteinuria.
  • Enzyme replacement therapy (ERT) has demonstrated effectiveness in slowing the progression of kidney disease in Fabry patients.

Conclusions:

  • Fabry nephropathy is a significant complication of FD requiring early intervention.
  • Factors like male gender, CKD stage, hypertension, and proteinuria worsen renal outcomes.
  • Current consensus supports initiating ERT in all individuals with FD exhibiting renal involvement to preserve kidney function.