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

M. Shanjida Sharmim; Afroza Begum; Delwar Hossain; Shamsun N. Shanta

doi:10.18203/2349-3291.ijcp20251106

Authors

M. Shanjida Sharmim Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
Afroza Begum Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh https://orcid.org/0000-0003-1894-9292
Delwar Hossain Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh https://orcid.org/0009-0000-2336-0015
Shamsun N. Shanta Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh https://orcid.org/0009-0002-8505-3285

DOI:

https://doi.org/10.18203/2349-3291.ijcp20251106

Keywords:

Multidrug resistant nephrotic syndrome, FSGS, NPHS1 gene mutation, Rituximab response

Abstract

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.

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