Alport syndrome: the effects of spironolactone on proteinuria and urinary TGF-β1

Background Alport syndrome (AS) is a progressive hereditary glomerular disease. Recent data indicate that aldosterone promotes fibrosis mediated by the transforming growth factor-β1 (TGF-β1) pathway, which may worsen proteinuria. Spironolactone (SP) antagonizes aldosterone and this study aimed to ev...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2013-09, Vol.28 (9), p.1837-1842
Main Authors: Giani, Marisa, Mastrangelo, Antonio, Villa, Roberta, Turolo, Stefano, Marra, Giuseppina, Tirelli, Amedea Silvia, Hopfer, Helmut, Edefonti, Alberto
Format: Article
Language:English
Subjects:
Adolescent
Aldosterone - blood
Alport's syndrome
Angiotensin-Converting Enzyme Inhibitors - therapeutic use
Blood Pressure - physiology
Case studies
Child
Children
Creatinine - urine
Diseases
Dosage and administration
Enalapril - therapeutic use
Female
Humans
Kidney Function Tests
Male
Medical research
Medicine
Medicine & Public Health
Medicine, Experimental
Mineralocorticoid Receptor Antagonists - therapeutic use
Mutation - physiology
Nephritis, Hereditary - drug therapy
Nephritis, Hereditary - genetics
Nephritis, Hereditary - urine
Nephrology
Original Article
Pediatrics
Proteinuria - drug therapy
Proteinuria - urine
Spironolactone
Spironolactone - therapeutic use
Transforming Growth Factor beta1 - urine
Treatment Outcome
Urology
Young Adult
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Summary:Background Alport syndrome (AS) is a progressive hereditary glomerular disease. Recent data indicate that aldosterone promotes fibrosis mediated by the transforming growth factor-β1 (TGF-β1) pathway, which may worsen proteinuria. Spironolactone (SP) antagonizes aldosterone and this study aimed to evaluate the efficacy of SP in reducing proteinuria and urinary TGF-β1 excretion in proteinuric AS patients. Methods The study involved ten children with AS, normal renal function, and persistent proteinuria (>6 months; uPr/uCr ratio >1). SP 25 mg once a day for 6 months was added to existing ACE inhibitor treatment with or without angiotensin-II receptor blockade. Urine and blood samples were examined monthly. Urinary TGF-β1 levels were measured twice before and three times during SP treatment. Plasma renin activity (PRA) and serum aldosterone levels were also measured. In eight patients, uProt/uCreat was also assessed after 9 months and 12 months of SP treatment. Results After beginning SP therapy, all patients showed significant decrease in mean uProt/uCreat ratio (1.77 ± 0.8 to 0.86 ± 0.6; p  
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-013-2490-z