Management of childhood steroid resistant nephrotic syndrome by paediatric nephrologists in Nigeria.

Abstract

Background: Steroid resistant nephrotic syndrome (SRNS) is a significant cause of morbidity and mortality in children. Management entails a prolonged course with varying investigations and immuno suppressants depending on practice of the Paediatric Nephrologists and affordability by the patients despite existing International guidelines.

Aim: To describe the management practices of Childhood SRNS among paediatric nephrologists / paediatricians in centres offering paediatric renal services in Nigeria.

Method:A cross-sectional online survey was conducted from 5^th December 2022 to 5^th January 2023 among paediatricians/paediatric nephrologists in 35 facilities that manage children with nephrotic syndrome in Nigeria using Google online questionnaire. The management including pattern of steroid and steroid-sparing medications usage in the management of SRNS was assessed.  

Result: Out of respondents surveyed, 32 (91.4%) completed the online survey and they were from the six geopolitical zones in Nigeria. Two-fifths (40.6%) practised paediatrics/paediatric nephrology for ≥ 10 years and a half (50%) practised in Federal teaching hospitals. The working definition of SRNS among respondents differed, with half (50%) defining SRNS as lack of remission after 8 weeks of prednisolone / prednisone at 2mg/kg/day or 60mg/m²/day and about one-third (28.1.%) and the remaining, one-fifth (21.9%) defining SRNS as lack of remission after 6 weeks of prednisolone/prednisone at 2mg/kg/day or 60mg/m²/day and lack of remission after 4 weeks of prednisolone/prednisone at 2mg/kg/day or 60mg/m²/day respectively. Majority (40.6%) rarely performed kidney biopsy following diagnosis of SRNS, 34.4% did most times while 9.4% did always. The top three choices of steroid sparing medications for SRNS were cyclophosphamide (37.5%), Cyclosporine A (28.1%) and Mycophenolate mofetil(28.1%) respectively. There were marked variations in the choice and duration of administration of steroid sparing medications, combination with low dose prednisolone, as well as the use of adjunct medications in SRNS. 

Conclusion: We identified that some aspects of management of SRNS were not in keeping with international guidelines.  Wide variations exist for the choice and duration of steroid-sparing medications, its' combination with low dose prednisolone and use of other adjunct medications, strengthening the need for a national guideline and continuous training among paediatricians and nephrologists.