Nephrotic syndrome in children: international evidence and Ukraine 2022

Abstract

This review aimed to summarize the current recommendations for conversion treatment of children with nephrotic syndrome and to identify the specifics of therapeutic management in Ukraine. The KDIGO guidelines of 2021 and the IPNA recommendations of 2020 and 2023, previous experience in Ukraine, and relevant clinical and legal aspects in 2022 were analyzed. Differences in the definitions of nephrotic children adopted in recent years were noted. International practice on disease conversion at the onset of nephrotic syndrome in children, steroid dependence, frequent recurrent course, and steroid resistance was summarized, and current measures of renal protection in children were presented. The spectrum of medicine used to treat nephrotic syndrome (glucocorticosteroids, calcineurin inhibitors, cyclophosphamide, mycophenolate mofetil, levamisole, chlorambucil, rituximab), their priority, dosage, monitoring, and duration of treatment in pediatrics depending on disease characteristics were presented. The consistency between the national treatment protocol and international recommendations for the main groups of medicines available in Ukraine was noted. In addition, the focus and limitations of treatment interventions depending on glucocorticosteroid sensitivity in Ukraine under martial law were summarized and practical points to facilitate clinical decision-making were highlighted. The urgency of establishing an adaptive guideline for the treatment of nephrotic children based on international experience and Ukrainian capabilities depending on the challenges of martial law was confirmed.

Keywords

• steroid-sensitive nephrotic syndrome, steroid-resistant nephrotic syndrome, treatment protocol, glucocorticosteroids, immunosuppression, renoprotection, evidence base, practice points, martial law.