Schijvens Anne M, van der Weerd Lucie, van Wijk Joanna A E, Bouts Antonia H M, Keijzer-Veen Mandy G, Dorresteijn Eiske M, Schreuder Michiel F
Department of Pediatric Nephrology, Radboud University Medical Center, Radboud Institute for Molecular Life Sciences, Amalia Children's Hospital, 804, P.O. Box 9101, 6500, HB, Nijmegen, The Netherlands.
Department of Pediatric Nephrology, Amsterdam University Medical Center, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
Eur J Pediatr. 2021 Jun;180(6):1885-1894. doi: 10.1007/s00431-021-03958-8. Epub 2021 Feb 3.
Nephrotic syndrome in childhood is a common entity in the field of pediatric nephrology. The optimal treatment of children with nephrotic syndrome is often debated. Previously conducted studies have shown significant variability in nephrotic syndrome management, especially in the choice of steroid-sparing drugs. In the Netherlands, a practice guideline on the management of childhood nephrotic syndrome has been available since 2010. The aim of this study was to identify practice variations and opportunities to improve clinical practice of childhood nephrotic syndrome in the Netherlands. A digital structured survey among Dutch pediatricians and pediatric nephrologists was performed, including questions regarding the initial treatment, relapse treatment, kidney biopsy, additional immunosuppressive treatment, and supportive care. Among the 51 responses, uniformity was seen in the management of a first presentation and first relapse. Wide variation was found in the tapering of steroids after alternate day dosing. Most pediatricians and pediatric nephrologists (83%) would perform a kidney biopsy in case of steroid-resistant nephrotic syndrome, whereas for frequent relapsing and steroid-dependent nephrotic syndrome this was 22% and 41%, respectively. Variation was reported in the steroid-sparing treatment. Finally, significant differences were present in the supportive treatment of nephrotic syndrome.Conclusion: Substantial variation was present in the management of nephrotic syndrome in the Netherlands. Differences were identified in steroid tapering, use of steroid coverage during stress, choice of steroid-sparing agents, and biopsy practice. To promote guideline adherence and reduce practice variation, factors driving this variation should be assessed and resolved. What is Known: • National and international guidelines are available to guide the management of childhood nephrotic syndrome. • Several aspects of the management of childhood nephrotic syndrome, including the choice of steroid-sparing drugs and biopsy practice, are controversial and often debated among physicians. What is New: • Significant practice variation is present in the management of childhood nephrotic syndrome in the Netherlands, especially in the treatment of FRNS, SDNS, and SRNS. • The recommendation on the steroid treatment of a first episode of nephrotic syndrome in the KDIGO guideline leaves room for interpretation and is likely the cause of substantial differences in steroid-tapering practices among Dutch pediatricians and pediatric nephrologists.
儿童肾病综合征是儿科肾脏病领域的常见病症。儿童肾病综合征的最佳治疗方法常常存在争议。此前开展的研究表明，肾病综合征的管理存在显著差异，尤其是在选择激素替代药物方面。自2010年以来，荷兰就有关于儿童肾病综合征管理的实践指南。本研究的目的是确定荷兰儿童肾病综合征临床实践中的差异以及改进机会。对荷兰儿科医生和儿科肾脏病医生进行了一项数字化结构化调查，包括有关初始治疗、复发治疗、肾活检、额外免疫抑制治疗和支持治疗的问题。在51份回复中，首次发病和首次复发的管理存在一致性。隔日给药后激素减量存在很大差异。大多数儿科医生和儿科肾脏病医生（83%）会在激素抵抗型肾病综合征的情况下进行肾活检，而对于频繁复发型和激素依赖型肾病综合征，这一比例分别为22%和41%。激素替代治疗也存在差异。最后，肾病综合征的支持治疗存在显著差异。结论：荷兰肾病综合征的管理存在很大差异。在激素减量、应激期间激素覆盖的使用、激素替代药物的选择和活检实践方面存在差异。为促进指南的遵循并减少实践差异，应评估并解决导致这种差异的因素。已知信息：• 有国家和国际指南可指导儿童肾病综合征的管理。•儿童肾病综合征管理的几个方面，包括激素替代药物的选择和活检实践，存在争议，医生之间经常进行讨论。新发现：• 荷兰儿童肾病综合征的管理存在显著的实践差异，尤其是在频繁复发型、激素依赖型和激素抵抗型肾病综合征的治疗方面。• KDIGO指南中关于肾病综合征首次发作的激素治疗建议留有解释空间，这可能是荷兰儿科医生和儿科肾脏病医生在激素减量实践中存在重大差异的原因。
