End-stage kidney disease due to alport syndrome: Outcomes in 296 consecutive Australia and New Zealand dialysis and transplant registry cases

Abstract

Background. Alport syndrome is a rare inheritable renal disease. Clinical outcomes for patients progressing to endstage kidney disease (ESKD) are not well described. Methods. This study aimed to investigate the characteristics and clinical outcomes of patients from Australia and New Zealand commencing renal replacement therapy (RRT) for ESKD due to Alport syndrome between 1965 and 1995 (early cohort) and between 1996 and 2010 (contemporary cohort) compared with propensity score-matched, RRT-treated, non-Alport ESKD controls. Results. A total of 58 422 patients started RRT during this period of which 296 (0.5%) patients had Alport ESKD. In the Conclusion. Alport syndrome patients experienced comparable dialysis and renal transplant outcomes to matched non-Alport ESKD controls in the contemporary cohort due to relatively greater improvements in outcomes for non-Alport ESKD patients over time. Post-transplant anti-GBM disease was rare.