This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses. NS is one of the most common pediatric glomerular diseases, with an incidence of around 2–7 cases per 100000 children per year. Corticosteroids are the mainstay of treatment, but the optimal therapeutic regimen for managing childhood idiopathic NS is still under debate. In Italy, shared treatment guidelines were lacking and, consequently, the choice of steroid regimen was based on the clinical expertise of each individual unit. On the basis of the 2015 Cochrane systematic review, KDIGO Guidelines and more recent data from the literature, this working group, with the contribution of all the pediatric nephrology centres in Italy and on the behalf of the Italian Society of Pediatric Nephrology, has produced a shared steroid protocol that will be useful for National Health System hospitals and pediatricians. Investigations at initial presentation and the principal causes of NS to be screened are suggested. In the early phase of the disease, symptomatic treatment is also important as many severe complications can occur which are either directly related to the pathophysiology of the underlying NS or to the steroid treatment itself. To date, very few studies have been published on the prophylaxis and treatment of these early complications, while recommendations are either lacking or conflicting. This consensus provides indications for the prevention, early recognition and treatment of these complications (management of edema and hypovolemia, therapy and prophylaxis of infections and thromboembolic events). Finally, recommendations about the clinical definition of steroid resistance and its initial diagnostic management, as well as indications for renal biopsy are provided. 

本共识文件旨在提供关于诊断和治疗的最新、多学科概述 的小儿肾病综合征 (NS) 首次就诊时。这是首次产生的同类共识文件 由意大利的所有儿科肾病中心进行，与法国等其他国家已经存在的一致， 德国和美国。它是基于目前关于 NS 对症和类固醇治疗的知识， 以期为复发治疗的单独共识文件提供依据。NS是 最常见的儿科肾小球疾病，发病率约为每年2-7例/10万儿童。 皮质类固醇是主要的治疗方法，但是治疗儿童特发性 NS仍在争论中。在意大利，缺乏共同的治疗指南，因此，选择类固醇 方案基于每个单位的临床专业知识。基于2015年 Cochrane 系统 综述、KDIGO指南和来自文献的最新数据，该工作组贡献了所有 意大利的儿科肾病中心，并代表意大利儿科肾病学会， 已经产生了一个共享的类固醇方案，将对国家卫生系统医院和儿科医生有用。 提出了初次就诊时的调查和待筛查 NS 的主要原因。早期阶段 的疾病，对症治疗也很重要，因为可发生许多严重并发症，既可 与基础 NS 的病理生理学或类固醇治疗本身直接相关。迄今为止，很少有研究 已经发表了关于这些早期并发症的预防和治疗，而建议是 缺乏或相互矛盾。该共识为预防、早期识别和治疗提供了指征 这些并发症（水肿和血容量不足的管理、感染的治疗和预防以及 血栓栓塞事件）。最后，关于类固醇抵抗的临床定义及其 提供了初步诊断管理以及肾活检的适应症。