Objective: To retrieve, evaluate and summarize the best evidence for disease management in children with primary nephrotic syndrome. Methods: The relevant literatures on the management of primary nephrotic syndrome in children were systematically searched in comprehensive databases, relevant guidelines websites and professional society websites at home and abroad. The search time was from the establishment of the database to January 2025. Two researchers independently completed the quality evaluation, evidence extraction and integration of the literature. Results: A total of 16 articles were included, including 4 evidence-based guidelines, 5 expert consensuses, 1 systematic review, and 6 clinical decisions. A total of 34 best evidences were summarized and integrated, including management objectives, medication management, urine monitoring, diet management, infection prevention, lifestyle and recurrence assessment. Conclusion: This study summarizes the best evidence of disease management in children with primary nephrotic syndrome, and provides evidence-based basis for clinical medical staff to implement disease management and health education for children. Clinical medical staff should fully combine the clinical situation and the actual situation of the child when selecting evidence, transform it and apply it to clinical practice.
Bajeer I, Khatri S, Hashmi S, et al., 2022, Factors Predicting Short Term Outcome in Children With Idiopathic Nephrotic Syndrome: A Prospective Cohort Study. Cureus, 14(1): e21538.
Noone D, Iijima K, Parekh R, 2018, Idiopathic Nephrotic Syndrome in Children. Lancet (London, England), 392(10141): 61–74.
Ye Q, Mao J, 2020, Immunopathogenesis of Idiopathic Nephrotic Syndrome in Children: Current Status and Future Directions. Chinese Journal of Pediatrics, 58(9): 705–707.
Zeng Y, Du Y, 2022, Analysis of Health-Related Quality of Life in Children with Primary Nephrotic Syndrome. Chinese General Practice, 25(27): 3379–3383.
Nephrology Group, Pediatric Branch, Chinese Medical Association, 2017, Evidence-Based Guidelines for the Diagnosis and Treatment of Steroid-Sensitive, Recurrent/Dependent Nephrotic Syndrome in Children (2016). Chinese Journal of Pediatrics, 55(10): 729–734.
Zhuang Y, Cheng J, Wu C, et al., 2024, Correlation between SOCS3 Expression and Glucocorticoid Resistance in Children with Primary Nephrotic Syndrome. Journal of Clinical Pediatrics, 42(5): 414–418.
Zhu Z, Hu Y, Zhou Y, et al., 2020, Promoting the Translation of Evidence into Clinical Practice (Part III): Research Topic Selection and Question Formulation. Journal of Continuing Nursing Education, 35(9): 796–799.
Xing W, Zhou Y, Zhu Z, et al., 2025, The Evolution of the Evidence Pyramid: From the “6S” Model to the “5S” Model. Journal of Continuing Nursing Education, 40(13): 1345–1348.
Zhou F, Hao Y, Cong X, et al., 2018, Supplementary Explanation and Reflection on the Appraisal of Guidelines for Research and Evaluation (AGREE II) and Scores in Each Domain. Journal of Nursing, 25(18): 56–58.
Wang Z, Han S, 2024, Key Points for Quality Assessment of Expert Consensus Papers in the Field of Nursing. Chinese Journal of Nursing, 59(3): 267–270.
Zhou Y, Gu Y, Hu Y, et al., 2018, JBI Evidence-Based Healthcare Center Quality Assessment Tools for Different Types of Research: Quality Assessment of Interventional Studies (Part I). Journal of Continuing Nursing Education, 33(2): 112–113.
Gu Y, Zhang H, Zhou Y, et al., 2018, JBI Evidence-Based Healthcare Center Quality Assessment Tools for Different Types of Research: Methodological Quality Assessment of Systematic Reviews. Journal of Continuing Nursing Education, 33(8): 701–703.
Wang C, Hu Y, 2015, JBI Evidence Pre-grading and Evidence Recommendation Level System (2014 Edition). Journal of Continuing Nursing Education, 30(11): 964–967.
Trautmann A, Boyer O, Hodson E, et al., 2023, IPNA Clinical Practice Recommendations for the Diagnosis and Management of Children with Steroid-Sensitive Nephrotic Syndrome. Pediatric Nephrology, 38(3): 877–919.
Kaku Y, Ohtsuka Y, Komatsu Y, et al., 2013, Clinical Practice Guideline for Pediatric Idiopathic Nephrotic Syndrome 2013: General Therapy. Clinical and Experimental Nephrology, 19(1): 34–53.
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group, 2012, KDIGO Clinical Practice Guideline for Glomerulonephritis. Kidney International, Supplement, 2(2012): 139–274.
Ehren R, Benz M, Brinkkötter P, et al., 2021, Pediatric Idiopathic Steroid-Sensitive Nephrotic Syndrome: Diagnosis and Therapy - Short Version of the Updated German Best Practice Guideline (S2e) - AWMF Registry No. 166-001, 6/2020. Pediatric Nephrology, 36(10): 2971–2985.
Li X, 2019, Expert Consensus on Vaccination for Children with Special Health Conditions, Part XVI: Kidney Diseases and Vaccination. Chinese Journal of Practical Pediatrics, 34(4): 265–266.
Pasini A, Benetti E, Conti G, et al., 2017, The Italian Society for Pediatric Nephrology (SINePe) Consensus Document on the Management of Nephrotic Syndrome in Children: Part I - Diagnosis and Treatment of the First Episode and the First Relapse. Italian Journal of Pediatrics, 43(1): 41.
Sinha A, Bagga A, Banerjee S, et al., 2021, Steroid Sensitive Nephrotic Syndrome: Revised Guidelines. Indian Pediatrics, 58(2021): 461–481.
Sun J, 2019, Expert Consensus on Vaccination for Children with Special Health Conditions (Part 19): Immunosuppressants and Vaccination. Chinese Journal of Practical Pediatrics, 34(5): 335–336.
Mi X, Yin Y, Gu J, 2019, Meta-Analysis of the Effectiveness of Calcium and Vitamin D in Preventing Bone Loss Caused by Glucocorticoids in Children with Nephrotic Syndrome. Journal of Clinical Pharmacy and Therapeutics, 17(2): 50–54.
Boyer O, 2023, Evaluation of Proteinuria in Children, https://www.uptodate.com/contents/evaluation-of-proteinuria-in-children
Niaudet P, 2023, Symptomatic Management of Nephrotic Syndrome in Children, https://www.uptodate.com/contents/symptomatic-management-of-nephrotic-syndrome-in-children
Valentini R, 2022, Evaluation and Management of Edema in Children, https://www.uptodate.com/contents/evaluation-and-management-of-edema-in-children
Niaudet P, 2024, Treatment of Idiopathic Nephrotic Syndrome in Children, https://www.uptodate.com/contents/treatment-of-idiopathic-nephrotic-syndrome-in-children
UPTODATE, 2024, Patient Education: Nephrotic Syndrome (The Basics), www-uptodate-cn-s.zju.sjuku.top/contents/nephrotic-syndrome-the-basics
UPTODATE, 2024, Patient Education: Low-Sodium Diet (The Basics), www-uptodate-cn-s.zju.sjuku.top/contents/low-sodium-diet-the-basics
Lu K, 2024, Analysis of Clinical Characteristics and Recurrence Factors in Children with Idiopathic Nephrotic Syndrome, thesis, Hebei Medical University.
Wang C, Yan J, Palmer R, et al., 2017, Childhood Nephrotic Syndrome Management and Outcome: A Single Center Retrospective Analysis. International Journal of Nephrology, 2017: 1–7.
Huo H, Huang X, Cao L, et al., 2023, Analysis of Risk Factors for Recurrence in Children with Primary Nephrotic Syndrome. Journal of Zhengzhou University (Medical Sciences), 58(5): 647–650.
Claudio P, Gabriella M, 2023, Nephrotic Syndrome: Pathophysiology and Consequences. Journal of Nephrology, 36(8): 2179–2190.
Yamamoto R, 2020, Incidence of Remission and Relapse of Proteinuria, End-Stage Kidney Disease, Mortality, and Major Outcomes in Primary Nephrotic Syndrome: The Japan Nephrotic Syndrome Cohort Study (JNSCS). Clinical and Experimental Nephrology, 24(2020): 526–546.