A FRANCIS1, C PRESTIDGE2, J KAUSMAN3, A LE PAGE, N LARKINS5, H MCCARTHY6
1Department of Nephrology, Queensland Children’s Hospital, Brisbane, Australia, 2Department of Nephrology, Starship Children’s Hospital, Auckland, New Zealand, 3Department of Nephrology, Royal Children’s Hospital, Melbourne, Australia, 4Department of Nephrology, Monash Medical Centre, Melbourne, Australia, 5Department of Nephrology, Perth Children’s Hospital, Perth, Australia, 6Department of Nephrology, Sydney Children’s Hospital Network, Sydney, Australia
Aim: To describe the impact of initial steroid sensitivity on risk of disease recurrence and transplant failure for children following kidney transplantation for steroid resistant nephrotic syndrome (SRNS).
Background: Scarce prior data suggest that children transplanted for SRNS who were steroid responsive at their initial presentation have an increased risk of recurrence post transplantation. There are no data informing the impact of initial steroid sensitivity on transplant survival.
Methods: Children who received a kidney transplant between 2000 and 2019 for SRNS in 7 paediatric centres were included. Clinical data were gathered from chart reviews and transplant data were provided from ANZDATA following ethics approval. Transplant survival was estimated using the Kaplan-Meier estimator, and the association between variables and graft survival estimated by Cox modelling.
Results: There were 56 children, 32 (57%) male, average age at transplant 11 years (SD 4) and 36 (65%) Caucasian. At presentation, 19/56 (34%) were steroid sensitive before developing SRNS. Of those steroid sensitive initially, 18/19 (95%) had recurrence post-transplant, 9/18 (50%) achieved full remission, although 3 of these had further recurrences. In those not steroid sensitive initially, 14/37 (38%) had recurrence post-transplant, 7/14 (50%) achieved full remission although one had further recurrences. Overall, 5-year transplant survival was 72% (95%CI=60-86%) and initial steroid sensitivity was not significantly associated with transplant loss (p=0.07).
Conclusions: Initial steroid sensitivity is strongly associated with SRNS recurrence. Transplant survival for children with SRNS is poor. Further research in other cohorts is needed to assess if initial steroid sensitivity is associated with poorer graft outcomes.
Dr Anna Francis is a paediatric nephrologist and clinician researcher at Queensland Children’s Hospital. She completed her PhD at the University of Sydney on the topic of “Long-Term Outcomes of Chronic Kidney Disease in Childhood and Adolescence”. She has a Masters in Clinical Epidemiology. In 2017, Dr Francis was awarded a Churchill Fellowship, travelling to Germany, England and Harvard to explore transition programs to adult care for young kidney transplant recipients. In 2019 she was appointed as an Editorial Fellow to Kidney International.