Department of General Pediatrics, Division of Pediatric Nephrology, University Children's Hospital Bonn, Bonn, Germany.
Helios University Medical Center Wuppertal, Children's Hospital, Center for Clinical and Translational Research (CCTR), Center for Biomedical Education and Research (ZBAF), Witten/Herdecke University, Heusnerstr. 40, 42283, Wuppertal, Germany.
BMC Nephrol. 2019 Feb 7;20(1):45. doi: 10.1186/s12882-019-1233-1.
Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in childhood, despite its rarely occurrence in the general population. Detailed information about clinical data of NS (e.g. average length of stay, complications) as well as of secondary nephrotic syndrome (SNS) is not well known.
A nationwide ESPED follow-up study presenting the clinical course and management of children with NS in Germany.
In course of 2 years, 347 children developed the first onset of NS, hereof 326 patients (93.9%) had a primary NS, and 19 patients had a SNS (missing data in 2 cases), the majority due to a Henoch-Schönlein Purpura. Patients with steroid-resistant NS (SRNS) stayed significantly longer in hospital than children with steroid-sensitive NS (25.2 vs. 13.3 d, p < 0.001). Patients with bacterial/viral infections stayed longer in hospital (24.9 d/19.5d) than children without an infection (14.2 d/14.9 d; p < 0.001; p = 0.016). Additionally, children with urinary tract infections (UTI) (p < 0,001), arterial hypertension (AH) (p < 0.001) and acute renal failure (ARF) (p < 0,001) stayed significantly longer in hospital. Patients with SRNS had frequent complications (p = 0.004), such as bacterial infections (p = 0.013), AH (p < 0.001), UTI (p < 0.001) and ARF (p = 0.007). Children with a focal segmental glomerulosclerosis (FSGS) had significantly more complications (p = 0.04); specifically bacterial infections (p = 0.01), UTI (p = 0.003) and AH (p < 0,001). Steroid-resistance was more common in patients with UTI (p < 0.001) and in patients with ARF (p = 0.007). Furthermore, steroid-resistance (p < 0.001) and FSGS (p < 0.001) were more common in patients with AH.
This nationwide, largest German study presents results on the clinical course of children with NS considering a diverse range of complications that can occur with NS. The establishment of a region-wide and international pediatric NS register would be useful to conduct further diagnostic and therapy studies with the aim to reduce the complication rate and to improve the prognosis of NS, and to compare the data with international cohorts.
肾病综合征（NS）是儿童中最常见的慢性肾脏疾病之一，尽管其在普通人群中很少发生。关于 NS 的临床数据（例如平均住院时间、并发症）以及继发性肾病综合征（SNS）的详细信息并不清楚。
一项全国性的 ESPED 随访研究，介绍了德国儿童 NS 的临床过程和管理。
在 2 年的时间里，347 名儿童首次出现 NS，其中 326 名患者（93.9%）患有原发性 NS，19 名患者患有 SNS（2 例缺失数据），大多数是由于过敏性紫癜。类固醇耐药性 NS（SRNS）患儿的住院时间明显长于类固醇敏感性 NS 患儿（25.2 天 vs. 13.3 天，p<0.001）。患有细菌/病毒感染的患儿住院时间（24.9 天/19.5 天）长于无感染患儿（14.2 天/14.9 天；p<0.001；p=0.016）。此外，患有尿路感染（UTI）的患儿（p<0.001）、动脉高血压（AH）（p<0.001）和急性肾衰竭（ARF）（p<0.001）的患儿住院时间明显更长。患有 SRNS 的患儿经常出现并发症（p=0.004），如细菌感染（p=0.013）、AH（p<0.001）、UTI（p<0.001）和 ARF（p=0.007）。患有局灶节段性肾小球硬化症（FSGS）的患儿有更多的并发症（p=0.04），特别是细菌感染（p=0.01）、UTI（p=0.003）和 AH（p<0.001）。UTI（p<0.001）和 ARF（p=0.007）患儿中更常见类固醇耐药。此外，患有 UTI（p<0.001）和 ARF（p=0.007）的患儿更易发生类固醇耐药。
这项全国性的、德国最大规模的研究提供了关于儿童 NS 临床过程的结果，考虑了可能发生的多种并发症。建立一个区域性和国际性的儿科 NS 登记处将有助于进行进一步的诊断和治疗研究，以降低并发症发生率，改善 NS 的预后，并与国际队列进行数据比较。
