2022+IPNA临床实践建议：儿童激素敏感型肾病综合征的诊断和管理.pdf

Pediatric Nephrology /10.1007/s00467-022-05739-3 GUIDELINES IPNA clinical practice recommendations for the diagnosis and management of children with steroid‑sensitive nephrotic syndrome 1 2 3 4 5 6 Agnes Trautmann  · Olivia Boyer  · Elisabeth Hodson  · Arvind Bagga  · Debbie S. Gipson  · Susan Samuel  · 7 8 9 10 11 Jack Wetzels  · Khalid Alhasan  · Sushmita Banerjee  · Rajendra Bhimma  · Melvin Bonilla‑Felix  · Francisco Cano12 · Martin Christian13 · Deirdre Hahn14 · Hee Gyung Kang15 · Koichi Nakanishi16 · Hesham Safouh17 · Howard Trachtman18 · Hong Xu19 · Wendy Cook20 · Marina Vivarelli21 · Dieter Hafner22  · on behalf of the International Pediatric Nephrology Association Received: 15 June 2022 / Revised: 3 August 2022 / Accepted: 22 August 2022 © The Author(s) 2022 Abstract Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, afecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85–90% of patients attain complete remission of proteinuria within 4–6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70–80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive dru