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Systematic Review of Management Strategies for Alport Syndrome: Implications for Male Patients
Zouina Sarfraz1, Ayesha Khan2, Maryyam Liaqat3
1Fatima Jinnah Medical University Lahore Pakistan.
Insights
Alport Syndrome (AS) management shows promise with bardoxolone methyl, ramipril, and losartan in slowing kidney disease. Further research is needed to confirm these Alport Syndrome treatments and enhance patient quality of life.
Area of Science:
- Nephrology
- Genetics
- Pharmacology
Background:
- Alport Syndrome (AS) is a rare genetic disorder causing progressive kidney disease, hearing loss, and ocular abnormalities.
- Affects approximately 1 in 50,000 newborns, with severe implications for males with X-linked inheritance.
Purpose of the Study:
- To systematically review current Alport Syndrome management strategies.
- To identify advancements and gaps in treatment options for AS patients.
Main Methods:
- Systematic review of clinical trials and observational studies on AS management.
- Searched multiple databases (PubMed, Web of Science, etc.) up to December 2023.
- Assessed risk of bias using Cochrane ROB 2 and Newcastle-Ottawa Scale.
Main Results:
- Bardoxolone methyl, ramipril, and losartan show potential in slowing renal disease progression in Alport Syndrome.
- Early intervention may delay dialysis and improve life expectancy.
- Significant heterogeneity limited quantitative synthesis; 25 ongoing trials involve over 52,000 participants.
Conclusions:
- Bardoxolone methyl, ramipril, and losartan show promise for delaying renal failure in Alport Syndrome.
- Highlights the need for larger, diverse trials to validate therapies and explore new strategies.
- Future research should address evidence gaps to improve treatment efficacy and quality of life for AS patients.
Background And Aims:
Alport Syndrome (AS) is a rare genetic disorder characterized by progressive kidney disease, hearing loss, and ocular abnormalities, with an incidence of approximately 1 in 50,000 newborns. Due to the severity of the disease, particularly in males with X-linked inheritance, this systematic review consolidates current management strategies, highlighting advancements and existing gaps in treatment options.
Methods:
This systematic review followed a protocol registered on the OSF platform (osf. io/k86ms). A comprehensive search of PubMed, Web of Science, Scopus, Cochrane Library, Embase, ClinicalTrials.gov, and the WHO ICTRP was completed by December 24, 2023. Studies eligible for inclusion were clinical trials or observational studies evaluating AS management. Four clinical trials from six publications and two observational studies met the inclusion criteria. The risk of bias was assessed using the Cochrane ROB 2 tool for clinical trials and the Newcastle-Ottawa Scale (NOS) for observational studies. Key interventions examined included bardoxolone methyl, ramipril, and losartan.
Results:
Bardoxolone methyl, ramipril, and losartan demonstrated potential benefits in slowing renal disease progression in AS. Observational studies indicated that early intervention might delay the need for dialysis and improve life expectancy. However, significant heterogeneity among studies precluded quantitative synthesis. Ongoing studies on AS management encompass 25 trials involving 52,135 participants, reflecting an active area of research.
Conclusion:
Bardoxolone methyl, ramipril, and losartan show promise in delaying renal failure in AS. Nonetheless, the findings highlight the critical need for larger, more diverse trials to validate these therapies and explore additional treatment strategies. Future research must aim to address these evidence gaps, improving treatment efficacy and patient quality of life, particularly for males disproportionately affected by the disease.
Protocol Registration:
The protocol for this systematic review is registered in the Open Science Framework (OSF): osf. io/k86ms.
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