ArticleCase reports in nephrology2014
Amiloride as an Alternate Adjuvant Antiproteinuric Agent in Fabry Disease: The Potential Roles of Plasmin and uPAR.
Article in Case reports in nephrology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 30 citations in OpenAlex.
- Nephrotic Syndrome: From Pathophysiology to Novel Therapeutic Approaches.Biomedicines · 2024Review
- Treatment of Fabry Nephropathy: A Literature Review.Medicina (Kaunas, Lithuania) · 2023Review
- The Perspective of Vitamin D on suPAR-Related AKI in COVID-19.International journal of molecular sciences · 2022Review
- Lyso-Gb3 Increases αvβ3 Integrin Gene Expression in Cultured Human Podocytes in Fabry Nephropathy.Journal of clinical medicine · 2020Article
- Mechanisms of Podocyte Detachment, Podocyturia, and Risk of Progression of Glomerulopathies.Kidney diseases (Basel, Switzerland) · 2020Review
- The effect of enzyme replacement therapy on clinical outcomes in male patients with Fabry disease: A systematic literature review by a European panel of experts.Molecular genetics and metabolism reports · 2019Review
- A Novel Mechanism of Renal Microcirculation Regulation: Connecting Tubule-Glomerular Feedback.Current hypertension reports · 2019Review
- Nephrotic Syndrome: Oedema Formation and Its Treatment With Diuretics.Frontiers in physiology · 2018Review
- Recent Treatment Advances and New Trials in Adult Nephrotic Syndrome.BioMed research international · 2017Review
- Expression of uPAR in Urinary Podocytes of Patients with Fabry Disease.International journal of nephrology · 2017Article
- In Acute IgA Nephropathy, Proteinuria and Creatinine Are in the Spot, but Podocyturia Operates in Silence: Any Place for Amiloride?Case reports in nephrology · 2017Article
- Podocyturia is significantly elevated in untreated vs treated Fabry adult patients.Journal of nephrology · 2016Observational
- Podocyte directed therapy of nephrotic syndrome-can we bring the inside out?Pediatric nephrology (Berlin, Germany) · 2016Review
- Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease.Case reports in nephrology · 2016Article
- Antiproteinuric therapy and Fabry nephropathy: factors associated with preserved kidney function during agalsidase-beta therapy.Journal of medical genetics · 2015Observational
- Copious Podocyturia without Proteinuria and with Normal Renal Function in a Young Adult with Fabry Disease.Case reports in nephrology · 2015Article
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Patients with Fabry disease present a higher risk of cardiovascular and kidney morbidity. We present a patient with a past history of biopsy-proven Fabry disease and stage 3 chronic kidney disease. Proteinuria partially dropped from 6.8 g/day to 2.1 g/day despite an aggressive regime which consisted of low-salt diet, agalsidase beta infusions, dual blockade of the renin-angiotensin system, and low-dose maintenance of steroids. As proteinuria is considered a risk marker of cardiovascular disease and of progression of kidney disease, we added amiloride 5 mg/day, a drug with proven effects in podocyte stabilization and proteinuria actions at the distal convoluted tubule. Proteinuria finally decreased to 0.8 g/day. This report highlights the relevance of intervening on proteinuria in a multitarget approach in order to reduce it as much as possible. Due to this pharmacological response, we suggest that although agalsidase beta specific treatment protects the endothelium, the podocyte, and the tubule in Fabry disease and secondary haemodynamic and immunologic pathways are treated with inhibition of the renin-angiotensin system and steroids, amiloride may act as a complementary tool in podocyte stabilization and in proteinuria effects at the distal tubule.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.