Rituximab for refractory cases of childhood  nephrotic syndrome

Rituximab for refractory cases of childhood nephrotic syndrome

  • نوع فایل : کتاب
  • زبان : انگلیسی
  • مؤلف : Jameela A. Kari & Salah M. El-Morshedy & Sherif El-Desoky & Hammad O. Alshaya & Khawla A. Rahim & Burhan M. Edrees
  • چاپ و سال / کشور: 2011

Description

Rituximab has been used over the last decade as a rescue therapy for refractory cases of nephrotic syndrome (NS). Here we report the use of rituximab in four children with idiopathic steroid-resistant nephrotic syndrome (SRNS) with various histological backgrounds (two cases with focal segmental glomerulosclerosis, one case with IgM nephropathy, and one case with minimal change disease), who failed to respond to other immunsuppressions. Their median age (range) was 10 (8–11) years. NPHS2 genetic mutation was negative in all of them. All patients received a single dose of rituximab (375 mg/m2) and achieved complete B cell depletion as CD19 was <1% for 3 months following rituximab infusion. Only one patient achieved non-sustained remission as he relapsed after 4 months despite zero CD19 level. Patients received no further doses of rituximab as B cell was depleted in the peripheral circulation. We conclude that a single dose of rituximab was not effective in inducing sustained remission in children with idiopathic SRNS, despite complete B cell depletion in the peripheral circulation. Further doses might be indicated to deplete non-circulating B cells.
Pediatr Nephrol (2011) 26:733–737 DOI 10.1007/s00467-011-1778-0 Received: 28 September 2010 / Revised: 5 January 2011 / Accepted: 10 January 2011 / Published online: 31 January 2011
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