Management of Childhood Onset Nephrotic Syndrome
ABSTRACT The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967. The characteristics of children presenting with nephrotic syndrome have changed over recent decades with greater frequency of the challenging condition focal segmental glomerulosclerosis and a greater prevalence of obesity and diabetes mellitus, which may be resistant to glucocorticoids in the former and exacerbated by long-term glucocorticoid therapy in the latter 2 conditions. The Children's Nephrotic Syndrome Consensus Conference was formed to systematically review the published literature and generate a children's primary nephrotic syndrome guideline for use in educational, therapeutic, and research venues.
SourceAvailable from: Yohei Ikezumi[Show abstract] [Hide abstract]
ABSTRACT: IntroductionPediatric idiopathic nephrotic syndrome is a very important disease in the field of pediatric nephrology. The Japanese Society for Pediatric Nephrology published the “Clinical Practice Guideline for Medical Treatment of Pediatric Idiopathic Nephrotic Syndrome (version 1.0) (in Japanese)” in 2005. The guideline, aiming to support appropriate decision and treatment for pediatric idiopathic nephrotic syndrome, illustrated standard regimens of medical treatment of pediatric idiopathic nephrotic syndrome at that time and has been credited with standardization and optimization of the treatment. In 2011, 6 years after the publication, the need to revise the guideline became recognized against the background of changes in care setting including introduction of rituximab. Additionally, development of guideline covering general therapies was required.The Scientific Committee of the Japanese Society for Pediatric Nephrology established a new operation to revise the guideline and publi ...Clinical and Experimental Nephrology 02/2015; 19(1). DOI:10.1007/s10157-014-1031-9 · 1.71 Impact Factor
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ABSTRACT: Background To investigate the long-term outcome in children with frequently relapsing nephrotic syndrome (FRNS) we conducted a follow-up of a previous randomized controlled trial (RCT) 10 years after the initiation of the treatment protocol. Methods We previously conducted an RCT on the efficacy of cyclosporine for treating children with FRNS. After 2 years of treatment, a recommended a management protocol of steroids, and immunosuppressants was provided. Results Valid information was available for 46 of the 56 patients (82.1 %) enrolled in the original RCT. The median follow-up period was 10.3 years from the start of protocol treatment with cyclosporine. At last follow-up (mean age 18.7 years), only ten patients (21.7 %) showed disease-free remission (no relapse for at least 2 years). In contrast, 23 (50.0 %) continued to relapse frequently or were on immunosuppressants, eight patients (17.4 %) had infrequent relapses without immunosuppressants. Adverse effects attributable to treatment included short stature (6 patients), osteoporosis (six patients), obesity (4 patients), cataracts (3 patients) and hypertension (3 patients). No lethal event or renal dysfunction occurred during follow-up. Conclusions This 10-year follow-up study shows that most children with FRNS experience relapses after 2 years of cyclosporine treatment, in adolescence and into adulthood. Outcomes in terms of life expectancy and renal function are favorable.Pediatric Nephrology 10/2014; 30(3). DOI:10.1007/s00467-014-2955-8 · 2.88 Impact Factor
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ABSTRACT: IntroductionNephrotic syndrome is a disorder characterized by severe proteinuria, hypoproteinemia, and generalized edema resulting from damage to the glomerular basement membrane. In Western countries, nephrotic syndrome affects 2 of 100,000 children per year . In Japan, approximately 1,300 new cases per year of pediatric nephrotic syndrome are reported to the Medical Aid for Specific Chronic Disease of Children and the disease develops in 5 of 100,000 children per year. Approximately 90 % of the cases of pediatric nephrotic syndrome are idiopathic, or of unknown cause. The first-line treatment for an initial episode of pediatric idiopathic nephrotic syndrome is oral steroid therapy, which leads to remission in approximately 80 % of cases (steroid-sensitive nephrotic syndrome) . However, 80 % of children with steroid-sensitive nephrotic syndrome experience one or more relapses,  and 50 % of these children have frequent relapses . Those with frequently relapsing nephrotic sy ...Clinical and Experimental Nephrology 02/2015; 19(1). DOI:10.1007/s10157-014-1030-x · 1.71 Impact Factor