European Alport Therapy Registry - European Initiative Towards Delaying Renal Failure in Alport Syndrome

• Status: Recruiting
• Trial end date: 2035-01-01
• Target enrollment: 0
• Participant gender: All

Summary

The hereditary type IV collagen disease Alport syndrome inevitably leads to end-stage renal disease. Currently there are no therapies known to improve outcome. Our non-interventional, observational study investigates, if medications such as ACE-inhibitors can (1) delay time to dialysis and (2) improve life-expectancy within three generations of Alport-families in Europe.

• Accepts Healthy Volunteers?: Accepts Healthy Volunteers

Details

• Lead Sponsor: University Hospital Goettingen
• Collaborators: Alport Selbsthilfe e.V.; Association pour l'Information et la Recherche sur les Maladies Rénales Génétiques (AIRG); Deutsche Gesellschaft für Nephrologie; Gesellschaft für Pädiatrische Nephrologie; KfH Foundation Preventive Medicine; Society for Pediatric Nephrology (Germany)
• Treatments: Angiotensin-Converting Enzyme Inhibitors; Ramipril; Spironolactone

Criteria

Inclusion Criteria/ Exclusion Criteria:

The diagnosis of Alport syndrome (AS) was proven by kidney biopsy or mutation analysis (or both). Patients were included if they were affected males with X-linked AS or patients with genetically proven homozygous autosomal AS. Patients were excluded if they did not give informed consent or the diagnosis was suspected but not confirmed.

The diagnosis of the heterozygous status was proven by (1) mutation analysis or (2) kidney biopsy plus genetic consultation for decision in between XLAS or ARAS inheritance (including a conclusive genealogic tree and/or linkage analysis). Patients were excluded if they were affected males with XLAS or patients with genetically proven homozygous ARAS. Patients were excluded if they did not give informed consent or the diagnosis was suspected but not confirmed or if they donated a kidney (living donor to affected family member).