Idiopathic nephrotic syndrome (INS) is a common chronic illness characterized by massive proteinuria and hypo-albuminemia in children. Baseline treatment is 6 month-corticotherapy. In cases of steroid resistant/dependent INS several types of treatment are used, including course of methyloprednisolone "pulses", alkylating agents, cyclosporin A, levamisole and mycophenolate mofetil. It has been suggested that children with frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome had a significantly longer relapse-free period if rituximab (RTX) treatment was additionally applied. We present a case of a 4.5 boy who due to steroid-sensitive, steroid-dependent nephrotic syndrome has been successfully treated with RTX. Administration of the one dose of Rituximab in the patient caused immediate decrease of CD19/CD20 positive B lymphocyte population. The depletion of B cells has been observed for the next six months. With regard to the fact that RTX treatment may affect patient's immune response, comprehensive immunodiagnostic has been conducted in a course of the Therapy.
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http://dx.doi.org/10.5114/ceji.2017.70976 | DOI Listing |
BMC Pediatr
January 2025
Geriatric Mental Health Research Center, Department of Psychiatry, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Background: This case report describes a unique presentation of sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS) caused by a rare SGPL1 variant, highlighting the diagnostic and management challenges associated with this condition.
Case Presentation: A 2-year-old Iranian female presented with steroid-resistant nephrotic syndrome (NS), primary adrenal insufficiency (AI), growth delay, seizures, and hyperpigmentation. Laboratory evaluation revealed hypoalbuminemia, significant proteinuria, hyperkalemia, and elevated adrenocorticotropic hormone (ACTH) levels.
Pediatr Nephrol
January 2025
Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, Henan, China.
Background: The effectiveness of rituximab (RTX) for steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) in children is well documented. However, there are insufficient data on relapse risk factors. Additionally, the retreat regimen for relapsed children requires further investigation.
View Article and Find Full Text PDFPediatr Allergy Immunol Pulmonol
January 2025
Clinical Immunology Unit, Faculty of Medicine and Health Sciences, Department of Paediatrics, Universiti Putra Malaysia, Selangor, Malaysia.
: RAS guanyl-releasing protein 1 (RASGRP1) deficiency is characterized by immune dysregulation and Epstein-Barr virus (EBV)-related lymphoproliferation. Diffuse mesangial sclerosis is one of the infrequent causes of infantile nephrotic syndrome. : Here, we described a 7-year-old girl who was diagnosed with diffuse mesangial sclerosis at 5 months old and subsequently developed chronic bilateral neck swelling at the age of 3 years.
View Article and Find Full Text PDFJ Nephrol
December 2024
Nephrology Unit, Department of Internal Medicine, Faculty of Medicine, Minia University, Taha Hussein Street, Minia, Egypt.
Background: Studies on renal vein thrombosis have been conducted as case reports or case series. The renal outcomes and mortality risk of renal vein thrombosis have not been fully established. We aimed to investigate the clinical characteristics, treatment modalities, and predictors of renal outcomes and mortality in patients with renal vein thrombosis in a large multicenter cohort.
View Article and Find Full Text PDFCEN Case Rep
December 2024
Department of Pediatrics, Songjiang Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, No.748, Zhongshan Middle Road, Songjiang District, Shanghai, 201600, China.
Nephrotic syndrome (NS) and atopic diseases are linked through shared immunological pathways, with allergic triggers often contributing to NS relapses, particularly in immunoglobulin E (IgE)-mediated pathways. Omalizumab, a humanized monoclonal antibody targeting free and cell-bound IgE, is commonly used in treating atopic diseases. We presented a pediatric case with a history of eczema, asthma, and recurrent atopic rhinitis, who first developed NS at age three, responding well to steroid therapy.
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