Korean J Transplant.  2022 Nov;36(Supple 1):S359. 10.4285/ATW2022.F-4997.

Pediatric kidney transplantation in cases of steroid-resistant nephrotic syndrome

• Kuttymuratov G

• KMID: 2535586
• DOI: http://doi.org/10.4285/ATW2022.F-4997

Abstract

Background
Kidney transplantation in children with end-stage renal failure associated with steroid-resistant nephrotic syndrome (SRNS) is a difficult task due to the lack of effect when using immunosuppressive therapy and the return of the disease after transplantation. SRNS can be return as early as 24 hours after kidney transplantation.
Methods
From 2018 to 2022 four kidney transplants were performed (three from a living donor and one from a cadaver) in children with morphologically diagnosed Focal segmental glomerulosclerosis, manifested by SRNS. Proteinuria, arterial hyperten-sion, edema were clinically observed in all patients. They received hemo- or peritoneal dialysis with an average duration of 3±1.5 years. The children were 7±2 years old. Before transplantation, all patients underwent a genetic study full exome sequencing, as a result of which the presence of specific genetic disorders that were the cause of the development of SRNS was revealed.
Results
After transplantation all children had proteinuria. In order to prevent recurrent NS, they underwent plasma exchange therapy two to three times a week on days 24 after surgery. The volume of plasma replacement ranged from 800 to 1,400 mL for one session. One child also developed transplant dysfunction. In two children after three and five plasma exchange sessions, respectively, proteinuria was arrested, the function of the graft remained satisfactory. The third child with proteinuria continues to receive plasma exchange without impairing the function of the graft. Immunosuppressive therapy included basiliximab, cyclosporine, mycophenolate mofetil and glucocorticoid.
Conclusions
In patients with SRNS, serological examination of donor-specific antibodies, histopathological examination, genetic research to predict the course of the disease and determine the tactics of treatment after transplantation should be performed. After kidney transplantation it is necessary to conduct plasma exchange sessions to prevent graft dysfunction. The diagnosis of focal segmental glomerulosclerosis graft return can be established on the basis of nephrobiopsy.