Ernould S, Godron A, Nelson J-R, Rigothier C, Llanas B, Harambat J
Service de pédiatrie, centre de référence maladies rénales rares du Sud-Ouest, hôpital Pellegrin-Enfants, CHU, place Amélie-Raba-Léon, 33076 Bordeaux, France; Service de pédiatrie, hôpital Gabriel-Martin, 97460 Saint-Paul, Réunion.
Service de pédiatrie, centre de référence maladies rénales rares du Sud-Ouest, hôpital Pellegrin-Enfants, CHU, place Amélie-Raba-Léon, 33076 Bordeaux, France.
Arch Pediatr. 2011 May;18(5):522-8. doi: 10.1016/j.arcped.2011.02.012. Epub 2011 Apr 1.
To estimate the incidence and describe the clinical presentation and outcome (steroid responsiveness, clinical course, complications) of idiopathic nephrotic syndrome in children in a population-based retrospective study.
Using local registries and the hospital discharge diagnosis system from two centers, all new cases of idiopathic nephrotic syndrome were identified in Gironde (France) between January 1992 and May 2008. To estimate incidence, population-based denominators were obtained from the National Institute for Statistics and Economic Studies (INSEE). Clinical data were collected from medical charts.
Ninety-nine cases of idiopathic nephrotic syndrome were reported (66 boys, 18 non-Caucasians) with an incidence of 2.3/100,000 (CI, 1.8-3.0) children less than 15 years. Ninety patients (91%) had steroid-sensitive nephrotic syndrome (SSNS) and nine (9%) were steroid-resistant (SRNS). The median time to remission in SSNS was 11 days. Relapses occurred in 75 (83%) children with SSNS with a median of four relapses (range, 1-32). The cumulative relapse-free incidence was 60% at 10 years after diagnosis in SSNS and 13% of patients aged 18 years old or over still had active disease. In SSNS, the only significant factor associated with steroid dependency or use of non steroid drugs was the time to initial response to steroids greater than 14 days. Nineteen children (19%) experienced severe complications of nephrotic syndrome including 11 bacterial infections and two thromboembolic complications. Two children with SRNS, of whom one was initially steroid-responsive, developed end-stage renal failure.
The incidence and outcome of idiopathic nephrotic syndrome in Gironde are comparable to the rates found in other studies. The disease may have a long course and the time for response to steroids at disease onset is the main predictor of steroid dependency and of use of non steroid agents.
在一项基于人群的回顾性研究中，评估儿童特发性肾病综合征的发病率，并描述其临床表现及转归（对类固醇的反应、临床病程、并发症）。
利用两个中心的本地登记处和医院出院诊断系统，确定了1992年1月至2008年5月期间法国吉伦特省所有新诊断的特发性肾病综合征病例。为了评估发病率，从国家统计与经济研究所（INSEE）获取基于人群的分母数据。临床数据从病历中收集。
共报告99例特发性肾病综合征病例（66例男孩，18例非白种人），15岁以下儿童的发病率为2.3/10万（95%可信区间，1.8 - 3.0）。90例（91%）患有类固醇敏感型肾病综合征（SSNS），9例（9%）为类固醇抵抗型（SRNS）。SSNS缓解的中位时间为11天。75例（83%）SSNS患儿出现复发，复发次数中位数为4次（范围1 - 32次）。SSNS诊断后10年累计无复发率为60%，18岁及以上患者中仍有13%患有活动性疾病。在SSNS中，与类固醇依赖或使用非类固醇药物相关的唯一显著因素是对类固醇初始反应时间大于14天。19例（19%）儿童出现肾病综合征的严重并发症，包括11例细菌感染和2例血栓栓塞并发症。2例SRNS患儿，其中1例最初对类固醇有反应，发展为终末期肾衰竭。
吉伦特省特发性肾病综合征的发病率和转归与其他研究结果相当。该病病程可能较长，疾病发作时对类固醇的反应时间是类固醇依赖和使用非类固醇药物的主要预测因素。
