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Proteinuria and its consequences in renal disease.
1Clinical Research Centre for Rare Diseases, Aldo e Cele Daccò, Ranica, Italy.
Acta Paediatrica (Oslo, Norway : 1992). Supplement
|March 3, 2004
Summary
Enzyme replacement therapy is key for Fabry disease renal issues. Additional treatments like blood pressure control and reducing proteinuria can further benefit patients with this rare genetic disorder.
Area of Science:
- Nephrology
- Genetics
- Biochemistry
Background:
- Chronic kidney diseases with proteinuria often progress to end-stage renal failure.
- Progression may involve factors like intraglomerular hypertension and glomerular hypertrophy, independent of the initial cause.
- Fabry disease, an X-linked lysosomal storage disorder, significantly impacts renal function due to lipid accumulation in kidney cells.
Purpose of the Study:
- To review the mechanisms driving chronic kidney disease progression.
- To discuss therapeutic strategies for preventing or minimizing renal damage.
- To evaluate the applicability of these strategies to Fabry disease patients.
Main Methods:
- Literature review of chronic renal disease progression mechanisms.
- Analysis of therapeutic options for renal protection.
- Assessment of treatment applicability for Fabry disease's renal manifestations.
Main Results:
- Enzyme replacement therapy is the primary strategy for Fabry disease.
- Other beneficial strategies include blood pressure control, proteinuria reduction, lipid management, and renin-angiotensin system inhibition.
- These approaches aim to halt or slow the progression of kidney damage.
Conclusions:
- Enzyme replacement therapy is crucial for managing renal aspects of Fabry disease.
- A combination of therapies, including supportive measures, can improve renal outcomes.
- Targeting specific mechanisms can mitigate the progression of kidney disease in Fabry patients.
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