Trends in kidney transplant outcomes in children and young adults with cystinosis.

ABSTRACT

Temporal changes in kidney transplant outcomes for cystinosis are unknown. We used the SRTR to identify all kidney transplants performed for cystinosis in patients younger than 31 years between 1987 and 2017. We divided time into three equal eras (1987-1997, 1998-2007, and 2008-2017) to assess changes in outcomes using Cox proportional and linear regression models. We examined 441 transplants in 362 patients. Age at ESRD progressively increased (12.1 vs 13.3 vs 13.4; P = .046). Eras 2 and 3 had lower risk of acute rejection (aHR 2 vs 10.45; P < .001) (aHR 3 vs 10.26; P < .001) and higher 5-year mean GFR (difference 2 vs 19.2 mL/min/1.73 m2 ; P = .005) (difference 3 vs 112.9 mL/min/1.73 m2 ; P = .002) compared with era 1. Five-year graft survival was similar across eras, but 5-year patient survival was higher for era 2 (aHR 0.25; P = .01). Seventy-nine patients underwent retransplantation. Five-year patient (94.2% vs 92.5%; P = .57) and graft survival (79.1% vs 74.1%; P = .52) were similar between primary and subsequent transplants. Age at ESRD, acute rejection, GFR at 5 years, and patient survival improved over time. Kidney retransplantation is associated with excellent outcomes in children and young adults with cystinosis.