Proteinuria and its consequences in renal disease

A. Schieppati, G. Remuzzi

Research output: Contribution to journalArticlepeer-review


Chronic renal diseases that involve proteinuria are typically characterized by an inexorable progression to end-stage renal failure. Many studies suggest that this progression may be the result of factors, such as intraglomerular hypertension and glomerular hypertrophy, that are unrelated to the initial disease. This paper reviews the mechanisms of progression of chronic renal diseases and discusses therapeutic strategies that should prevent or minimize further renal damage and the applicability of these strategies to patients with the rare X-linked lysosomal storage disorder Fabry disease. Renal involvement is a major feature of Fabry disease, which is characterized by vacuolated epithelial cells in the glomerulus and distal tubules, resulting from lipid inclusions within these cells. Although enzyme replacement therapy is the key strategy to halt the progression of Fabry disease, additional therapeutic options include blood pressure control, reduction of proteinuria, lipid control and inhibition of the renin-angiotensin system. Conclusion: A range of therapeutic options, used in conjunction with enzyme replacement therapy, may have beneficial effects on the renal manifestations of Fabry disease.

Original languageEnglish
Pages (from-to)9-13
Number of pages5
JournalActa Paediatrica, International Journal of Paediatrics, Supplement
Issue number443
Publication statusPublished - Dec 2003


  • Chronic renal disease
  • Fabry disease
  • Proteinuria
  • Renal pathology

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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