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Abstract: TH-PO1200

Safety and Efficiency of Nephroprotective Therapy with Ramipril in Children

Session Information

Category: Pediatric Nephrology

  • No subcategory defined

Author

  • Gross, Oliver, University Medicine Goettingen, Goettingen, Germany

Group or Team Name

  • GPN-study group and EARLY PRO-TECT Alport investigators
Background

Children with Alport syndrome (AS) develop renal failure early in life. The safety and efficacy of preemptive nephroprotective therapy is uncertain.

Methods

In an investigator-initiated, double-blinded, randomized, placebo-controlled trial, we treated pediatric patients with Ramipril. Pretreated children and patients whose parents refused randomization versus placebo were treated open label. Prospective data from the US-Alport registry (NCT00622544) were added to substantiate our results in a Bayesian evidence synthesis approach. The primary endpoints were safety: adverse drug reactions before disease progression and efficacy: time to progression.

Results

Sixty-six oligosymptomatic children with (yet) normal renal function entered the up to 6-year treatment phase with a total of 216.4 patient-years on Ramipril. Most important, Ramipril was safe (hazard ratio 1.00, 95%CI 0.83-1.21). Efficacy analyses, though not significant, cumulated evidence in favor of Ramipril: in the randomized arm, Ramipril decreased the risk of disease progression by >40% (0.51; 95%CI 0.12–2.20), diminished the slope of albuminuria progression and the loss of glomerular filtration rate. Only 27.3% (3/11) of Ramipril-treated, but 55.6% (5/9) of placebo-treated children progressed. Efficacy was confirmed by comparison of untreated children from the US with participants treated open label, in whom Ramipril again reduced disease progression by >40% (0.53; 95%CI 0.23-1.24).

Conclusion

Early initiation of Ramipril therapy in children with AS is safe and can be expected to slow renal failure by many years, underlining the value of preemptive therapy in this CKD. Thus, screening programs for glomerular hematuria in children and young adults should include genetic testing for AS-gene variants. (Funded by the German Federal Ministry of Education and Research; EARLY PRO-TECT Alport ClinicalTrials.gov number, NCT01485978).

Analyses of the primary safety and efficacy endpoints.

Funding

  • Government Support - Non-U.S.