Journal
NEPHROLOGY DIALYSIS TRANSPLANTATION
Volume 29, Issue -, Pages 124-130Publisher
OXFORD UNIV PRESS
DOI: 10.1093/ndt/gfu028
Keywords
Alport syndrome; chaperon therapy; discoidin domain receptor 1; kidney fibrosis; microRNA-21
Categories
Funding
- European Regional Development Fund
- Republic of Cyprus through the Research Promotion Foundation [NEW INFRASTRUCTURE/STRATEGIC/0308/24]
- Alport Syndrome Foundation
- Baxter foundation
- Association pour l'Information et la Recherche sur les maladies renale Genetiques (AIRG) France
- KfH-Foundation Preventive Medicine
- AbbVie GmbH Co. KG Germany
- German Kidney Foundation
- Deutsche Forschungsgemeinschaft [GR 1852/4-1, GR 1852/4-2]
- European Renal Association ERA/EDTA
- Kidney Foundation of Canada
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The hereditary type IV collagen disease Alport syndrome (AS) always leads to end-stage renal failure. Yesterday, for the past 90 years, this course was described as 'inevitable'. Today, RAAS blockade has changed the 'inevitable' course to a treatable disease. Tomorrow, researchers hope to erase the 'always' from 'always leads to renal failure' in the textbooks. This review elucidates therapeutic targets that evolve from research: (i) kidney embryogenesis and pathogenesis; (ii) phenotypegenotype correlation and the role of collagen receptors and podocytes; (iii) the malfunctioning Alport-GBM; (iv) tubulointerstitial fibrosis; (v) the role of proteinuria in pathogenesis and prognosis; and (vi) secondary events such as infections, hyperparathyroidism and hypercholesterolaemia. Therefore, moderate lifestyle, therapy of bacterial infections, Paricalcitol in adult patients with hyperparathyroidism and HMG-CoA-reductase inhibitors in adult patients with dyslipoproteinemia might contribute to a slower progression of AS and less cardiovascular events. In the future, upcoming treatments including stem cells, chaperon therapy, collagen receptor blockade and anti-microRNA therapy will expand our perspective in protecting the kidneys of Alport patients from further damage. This perspective on current and future therapies is naturally limited by our personal focus in research, but aims to motivate young scientists and clinicians to find a multimodal cure for AS.
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