Background: We report a 7-year-old boy with high-degree steroid-dependent idiopathic nephrotic syndrome (SDNS) who went into remission with rituximab (RTX) maintenance therapy.
Case-diagnosis/treatment: Four months after this patient received his first RTX infusion, there was a progressive and sustained decrease of immunoglobulin (Ig)G and IgM levels. Thirteen months after the initiation of RTX therapy he was in sustained remission without any steroid or oral immunosuppressive therapy; however, B cell depletion was still present. At this time he developed a fulminant myocarditis due to enterovirus. Despite aggressive treatment and the administration of intravenous polyvalent immunoglobulins there was no clinical improvement. He successfully underwent heart transplant surgery.
Conclusions: We conclude that B cell depletion with RTX is efficacious in the treatment of paediatric SDNS but that it may be associated with severe infectious complications. Therefore, we recommend a close monitoring of Ig levels in children who have received RTX therapy and a supplementation with intravenous Ig as soon as the Ig levels fall below the lower limit of the normal range.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1007/s00467-013-2485-9 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Treatment of very high risk membranous nephropathy complicated by pulmonary embolism with glucocorticoids and rituximab: Case report.
Medicine (Baltimore)
January 2025
Division of Nephrology, Department of Medicine, National University Hospital, Singapore.
Rationale: We report the efficacy of combination prednisolone and intravenous (IV) rituximab as an immunosuppressive regimen for a young male presenting with extensive venous thromboembolism including a submassive pulmonary embolism secondary to life-threatening nephrotic syndrome from very high risk anti-phospholipase-A2 receptor (PLA2R) positive membranous nephropathy. Initial treatment was with mechanical thrombectomy and anticoagulation. Thereafter, oral prednisolone was initiated to induce remission, during a period of uninterrupted anticoagulation.
View Article and Find Full Text PDFOptimal functioning of hypothalamic-pituitary-adrenal axis after cessation of prednisolone therapy: a cross-sectional study in children with nephrotic syndrome.
Pediatr Nephrol
January 2025
Department of Pediatrics, Chacha Nehru Bal Chikitsalaya, Delhi, 110031, India.
Background: Hypothalamic-pituitary-adrenal (HPA) axis recovery after cessation of steroid therapy in children with nephrotic syndrome (NS) has hardly been studied in the literature.
Methods: This 22-month cross-sectional study recruited children (2-14 years) with NS, having received a minimum 3 months of prednisolone, now in remission, and off steroids for 1, 3, or 6 months. Serum cortisol-basal and stimulated (with long-acting intramuscular adrenocorticotropic hormone), and factors affecting them, were assessed.
Malakoplakia Associated with Diarrhoea and Colonic Lesions After Rituximab Treatment.
Eur J Case Rep Intern Med
December 2024
Gastroenterology Department, Adan Hospital Kuwait, Hadiya, Kuwait.
Unlabelled: Malakoplakia is a rare granulomatous condition that occurs due to defective lysosomal digestion during phagocytosis and can mimic inflammatory bowel disease (IBD) or malignancies, particularly in immunosuppressed patients. We report the case of a 62-year-old male with IgG4-related orbitopathy, who developed persistent diarrhoea and colonic lesions 6 weeks after receiving rituximab therapy for nephrotic syndrome secondary to membranoproliferative glomerulonephritis. Colonoscopy revealed pancolitis with mucosal granularity, loss of vascular pattern, and small nodules, raising initial suspicion for IBD.
View Article and Find Full Text PDFThe mechanisms of tripterygium glycosides-induced reproductive toxicity and detoxification strategies.
Reprod Toxicol
January 2025
Key Laboratory of Shaanxi Administration of Traditional Chinese Medicine for TCM Compatibility, Shaanxi University of Chinese Medicine, Xianyang 712046, Shaanxi Province, China. Electronic address:
Tripterygium glycosides (TG) is a widely used preparation in the treatment of rheumatoid arthritis (RA), nephrotic syndrome and diabetic nephropathy. Although the clinical efficacy is definite, the side-effects on reproductive system limit its wide application. It is of great significance to take measures to alleviate its reproductive toxicity and expand its clinical use.
View Article and Find Full Text PDFAdvancing the clinical assessment of glomerular podocyte pathology in kidney biopsies via super-resolution microscopy and angiopoietin-like 4 staining.
Theranostics
January 2025
Department of Nephrology, China-Japan Friendship Hospital (Institute of Clinical Medical Sciences), Beijing, 100029, China.
The tertiary structure of normal podocytes prevents protein from leaking into the urine. However, observing the complexity of podocytes is challenging because of the scale differences in their three-dimensional structure and the close proximity between neighboring cells in space. In this study, we explored podocyte-secreted angiopoietin-like 4 (ANGPTL4) as a potential morphological marker via super-resolution microscopy (SRM).
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!