Enigmatic response of frequent use of rituximab in multidrug resistant nephrotic syndrome with focal segmental glomerulosclerosis with NPHS1 gene mutation children: genetic and pathophysiological consideration

Childhood nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) usually refractory to multiple immunosuppressive drugs including rituximab. Nephrotic syndrome secondary to NPHS1 gene mutation also showed multidrug resistance with bad prognosis. Here, we are reporting a case who has multidrug resistant nephrotic syndrome with FSGS with NPHS1 gene mutation but responded well with frequent periodic use of rituximab.