Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease
No specific or efficient treatment exists for Alport syndrome, an X-linked hereditary disease caused by mutations in collagen type IV, a crucial component of the glomerular basement membrane. Kidney failure is usually a major complication of the disease, and patients require renal replacement therap...
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Hindawi Publishing Corporation
2016
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| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4749773/ |
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pubmed-47497732016-03-03 Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease Trimarchi, H. Canzonieri, R. Muryan, A. Schiel, A. Araoz, A. Paulero, M. Andrews, J. Rengel, T. Forrester, M. Lombi, F. Pomeranz, V. Iriarte, R. Zotta, E. Case Report No specific or efficient treatment exists for Alport syndrome, an X-linked hereditary disease caused by mutations in collagen type IV, a crucial component of the glomerular basement membrane. Kidney failure is usually a major complication of the disease, and patients require renal replacement therapy early in life. Microhematuria and subsequently proteinuria are hallmarks of kidney involvement, which are due to primary basement membrane alterations that mainly cause endothelial thrombosis and podocyte contraction and ulterior irreversible detachment. Commonly drug-based approaches include angiotensin-converting enzyme inhibitors and angiotensin receptor blockers, which are employed to reduce proteinuria and thus retard kidney disease progression and cardiovascular morbidity and mortality. However, as any hereditary disease, it is expressed as early as in the intrauterine life, and usually an index case is helpful to detect family-related cases. As no specific treatment exists, pathophysiologically based approaches are useful. The present case illustrates the reduction rate of urinary podocyte loss and proteinuria after amiloride administration and suggests the molecular pathways involved in Alport renal disease. Finally, podocyturia rather than proteinuria should be considered as an earlier biomarker of kidney involvement and disease progression in Alport disease. Hindawi Publishing Corporation 2016 2016-01-28 /pmc/articles/PMC4749773/ /pubmed/26942026 http://dx.doi.org/10.1155/2016/1492743 Text en Copyright © 2016 H. Trimarchi et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
| repository_type |
Open Access Journal |
| institution_category |
Foreign Institution |
| institution |
US National Center for Biotechnology Information |
| building |
NCBI PubMed |
| collection |
Online Access |
| language |
English |
| format |
Online |
| author |
Trimarchi, H. Canzonieri, R. Muryan, A. Schiel, A. Araoz, A. Paulero, M. Andrews, J. Rengel, T. Forrester, M. Lombi, F. Pomeranz, V. Iriarte, R. Zotta, E. |
| spellingShingle |
Trimarchi, H. Canzonieri, R. Muryan, A. Schiel, A. Araoz, A. Paulero, M. Andrews, J. Rengel, T. Forrester, M. Lombi, F. Pomeranz, V. Iriarte, R. Zotta, E. Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease |
| author_facet |
Trimarchi, H. Canzonieri, R. Muryan, A. Schiel, A. Araoz, A. Paulero, M. Andrews, J. Rengel, T. Forrester, M. Lombi, F. Pomeranz, V. Iriarte, R. Zotta, E. |
| author_sort |
Trimarchi, H. |
| title |
Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease |
| title_short |
Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease |
| title_full |
Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease |
| title_fullStr |
Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease |
| title_full_unstemmed |
Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease |
| title_sort |
podocyturia: a clue for the rational use of amiloride in alport renal disease |
| description |
No specific or efficient treatment exists for Alport syndrome, an X-linked hereditary disease caused by mutations in collagen type IV, a crucial component of the glomerular basement membrane. Kidney failure is usually a major complication of the disease, and patients require renal replacement therapy early in life. Microhematuria and subsequently proteinuria are hallmarks of kidney involvement, which are due to primary basement membrane alterations that mainly cause endothelial thrombosis and podocyte contraction and ulterior irreversible detachment. Commonly drug-based approaches include angiotensin-converting enzyme inhibitors and angiotensin receptor blockers, which are employed to reduce proteinuria and thus retard kidney disease progression and cardiovascular morbidity and mortality. However, as any hereditary disease, it is expressed as early as in the intrauterine life, and usually an index case is helpful to detect family-related cases. As no specific treatment exists, pathophysiologically based approaches are useful. The present case illustrates the reduction rate of urinary podocyte loss and proteinuria after amiloride administration and suggests the molecular pathways involved in Alport renal disease. Finally, podocyturia rather than proteinuria should be considered as an earlier biomarker of kidney involvement and disease progression in Alport disease. |
| publisher |
Hindawi Publishing Corporation |
| publishDate |
2016 |
| url |
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4749773/ |
| _version_ |
1613537091301408768 |