RecruitingNCT02378805

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

European Alport Therapy Registry - European Initiative Towards Delaying Renal Failure in Alport Syndrome: Current and Novel Therapies

Sponsor

University Hospital Goettingen

Enrollment

800 participants

Start Date

Jul 1, 1995

Study Type

OBSERVATIONAL

Conditions

Alport Syndrome Pediatric Kidney Disease Thin Basement Membrane Disease Hereditary Kidney Disease Familial Benign Hematuria

Summary

The hereditary type IV collagen disease Alport syndrome leads to kidney failure early in life. Currently there are no specific medications approved for treatment, however, several therapies have been evaluated preclinically and could improve outcome. For that reason, this non-interventional, observational study investigates, if medications (1) delay disease progression; (2) delay time to kidney failure; (3) improve life-expectancy compared to untreated patients (relatives). This observational study started in 2006 as an European registry. Since 2019, this registry has been expanded to "Alport XXL" via the International Alport Alliance as a global effort across all continents. From 2020 on to present, "Alport XXL" has a special focus on the outcomes of early therapy in young patients on ACE-inhibitors vs. Angiotensin-receptor blockers vs. their combination.

Eligibility

Inclusion Criteria2

Diagnosis of Alport syndrome (AS) by kidney biopsy or mutation analysis (or both).
Any type of genetic variant is accepted for X-linked, autosomal or digenic Alport syndrome (COL4A3, 4 or 5 genes).