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

NCT02378805 RECRUITING

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.

Details

Lead sponsor	University Hospital Goettingen
Status	RECRUITING
Enrolment	800
Start date	1995-07
Completion	2036-03-01

Conditions

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

Interventions

• ACE-inhibitor
• Angiotensin-receptor blocker (ARB)
• HMG-Coenzyme inhibitor (statin)
• Spironolactone or Finerenone
• Paricalcitol
• SGLT2 inhibitor

Primary outcomes

• age at onset of end stage kidney failure (need for renal replacement therapy) — until end of observation in 2037
  kidney disease in Alport syndrome starts at birth, age at onset of end stage kidney failure in years
• life-expectancy in years (age at death) — until end of observation in 2037
  Alport syndrome starts at birth, age at death of patients in years
• yearly loss of estimated glomerular filtration rate (eGFR) — until end of observation in 2037
  kidney disease in Alport syndrome starts at birth, eGFR-loss per year in ml/min/1.73m2
• time in years until doubling or tripling of urinary albumin to creatinine ratio (UACR) — until end of observation in 2037
  kidney disease in Alport syndrome starts at birth; time since birth to doubling (AS stages I or II) or tripling (AS stage 0) of UACR in years.

Countries

Germany