Trends in Kidney Transplant Outcomes in Children And Young Adults With Cystinosis

Abstract

Temporal changes in kidney transplant outcomes for children and young adults with cystinosis are unknown. We used the Scientific Registry of Transplant Recipients to identify all kidney transplants performed for cystinosis during 1987-2017 in patients < 31 years at transplant. We divided time into three equal eras (1987-1997, 1998-2007, and 2008-2017) to assess temporal changes in outcomes using Cox proportional and linear regression models. We examined 441 transplants in 362 patients. Age at ESRD increased over time (12.1 vs.13.3 vs.13.4; p=0.046). Eras 2 and 3 had lower risk of acute rejection (aHR 2 vs.1: 0.45; p=0.0002) (aHR 3 vs.1: 0.26; p <0.0001) compared with era 1. 5-year mean eGFR was higher for eras 2 (difference 2 vs.1: 9.2 ml/min/1.73m2; p=0.005) and 3 (difference 3 vs.1: 12.9 ml/min/1.73m2; p=0.002). The risk of death was lower for era 2 (adjusted hazard ratio (aHR):0.25; p=0.01). Seventy-three patients were retransplanted. The 5-year patient (94.2% vs. 92.5%; p=0.57) and graft survival (79.1% vs. 74.1%; p=0.52) were similar between primary vs. second transplants. Age at ESRD onset, delayed graft function, acute rejection, 5-year mean eGFR and patient survival have improved over time. Kidney retransplantation is associated with excellent outcomes in children and young adults with cystinosis.