Pediatrics international : official journal of the Japan Pediatric Society
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Letter Case Reports
Paraneoplastic pemphigus with Castleman's disease and bronchiolitis obliterans.
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Several therapies including immunosuppressive agents have been shown to be effective and safe for frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome (FRNS/SDNS) and steroid-resistant nephrotic syndrome in children. It is evident, however, that a substantial number of children are still refractory to treatment despite these therapies. Rituximab is a chimeric monoclonal antibody, which inhibits CD20-mediated B-cell proliferation and differentiation. ⋯ The Research Group of Childhood-onset Refractory Nephrotic Syndrome (RCRNS) conducted a randomized, double-blind, placebo-controlled, multi-center clinical trial (RCRNS-01) and an open-label, multi-center, pharmacokinetic clinical trial (RCRNS-02). These two trials were investigator-initiated, registration-directed clinical trials designed to apply Ministry of Health, Labour and Welfare approval for the use of rituximab for childhood-onset refractory FRNS/SDNS in Japan. RCRNS-01 could be the first study to clarify whether rituximab is effective and safe for childhood-onset refractory FRNS/SDNS.
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Pandemic influenza A (H1N1) causes severe pneumonia in children. The mechanism of development of respiratory failure in pneumonia patients remains unknown. This report describes clinical features of childhood influenza A pneumonia. ⋯ Tachycardia, tachypnea, cyanosis and leukocytosis with neutrophilia, could be useful predictors at admission to identify high-risk influenza A (H1N1) pneumonia in children.