Published in:
01-02-2015 | Guideline
Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy
Authors:
Kenji Ishikura, Shinsuke Matsumoto, Mayumi Sako, Kazushi Tsuruga, Koichi Nakanishi, Koichi Kamei, Hiroshi Saito, Shuichiro Fujinaga, Yuko Hamasaki, Hiroko Chikamoto, Yasufumi Ohtsuka, Yasuhiro Komatsu, Toshiyuki Ohta, Takuhito Nagai, Hiroshi Kaito, Shuji Kondo, Yohei Ikezumi, Seiji Tanaka, Yoshitsugu Kaku, Kazumoto Iijima
Published in:
Clinical and Experimental Nephrology
|
Issue 1/2015
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Excerpt
Nephrotic 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 [
1]. 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) [
2]. However, 80 % of children with steroid-sensitive nephrotic syndrome experience one or more relapses, [
3] and 50 % of these children have frequent relapses [
4]. Those with frequently relapsing nephrotic syndrome are prone to suffer steroid-induced side effects such as obesity, growth impairment, hypertension, diabetes mellitus, osteoporosis, and adrenal insufficiency. Many cases of steroid-resistant nephrotic syndrome, where steroids are ineffective, progress to renal failure. …
