Paediatric Focal Segmental Glomerulosclerosis (FSGS): From Bench to Bedside and Beyond

Paediatric Focal Segmental Glomerulosclerosis (FSGS) is a leading cause of steroid-resistant nephrotic syndrome and progressive kidney failure in children. Early subclassification into primary, secondary, genetic, or undetermined forms is crucial for guiding appropriate management. Primary FSGS typically necessitates immunosuppressive therapy, whereas secondary FSGS benefits from supportive measures and treatment of the underlying cause. Emerging treatments—including SGLT2 inhibitors, endothelin receptor antagonists, and APOL1-targeted agents—show promise in reducing proteinuria and preserving kidney function. Insights into podocyte biology, including TRPC channel dysregulation and fibrotic signalling pathways, are opening new therapeutic avenues. As research continues to evolve, the future of paediatric FSGS management lies in individualised, pathophysiology-driven therapies that may significantly improve clinical outcomes.