Srivastava T, Simon S D, Alon U S
Section of Pediatric Nephrology, Children's Mercy Hospital, University of Missouri at Kansas City 64108, USA.
Pediatr Nephrol. 1999 Jan;13(1):13-8. doi: 10.1007/s004670050555.
In recent adult literature, there have been reports of an increasing incidence of focal segmental glomerulosclerosis (FSGS) among patients with nephrotic syndrome. To examine whether this observation is also relevant to the pediatric population we utilized our hospital computerized database to analyze the data on children with primary nephrotic syndrome seen first between the years 1984 and 1995. A questionnaire was also sent to all metropolitan Kansas City pediatricians to identify possible patients outside the database. The inclusion criteria were clinical nephrotic syndrome or proteinuria with a kidney biopsy. A total of 148 patients (group A) were identified; 86 of them from metropolitan Kansas City (group B). In group A the incidence of minimal change disease (MCD) and FSGS was 52.7% [95% confidence interval (CI) 44%-60%] and 23.0% (95% CI 16-29%), respectively and in group B 54.7% (95% CI 44%-65%) and 24.5% (95% CI 15%-33%), respectively. Those numbers were significantly different from the International Study of Kidney Disease in Children (IS-KDC) reported incidence of 76.4% for MCD and 6.9% for FSGS. Similar to the ISKDC, in our population children over 6 years had a higher incidence of FSGS than younger children (32.8% vs. 16.7%, P = 0.028). The annual incidence rate for nephrotic syndrome in group B was 2.2 cases/10(5) children per year, of which MCD comprised 1.22 cases/10(5) children per year and FSGS 0.5 cases/10(5) children per year. The annual incidence rates of both primary nephrotic syndrome (3.6) and FSGS (1.6) were significantly higher in African-Americans, than Caucasians (1.8 and 0.3 cases/10(5) children per year, respectively). Our study indicates nearly no change in the annual incidence of pediatric primary nephrotic syndrome, but a higher incidence of FSGS with reciprocal decline in the incidence of MCD. The possibility of primary nephrotic syndrome being caused by a non-MCD entity is further raised among African-American and in children over 6 years. We conclude that our perception of primary nephrotic syndrome of childhood as a benign condition has to be carefully reexamined and a more-guarded prognostic approach adopted in our geographic area.
在近期的成人文献中，有报道称肾病综合征患者中局灶节段性肾小球硬化（FSGS）的发病率呈上升趋势。为了研究这一观察结果是否也适用于儿科人群，我们利用医院的计算机数据库分析了1984年至1995年间首次就诊的原发性肾病综合征患儿的数据。我们还向堪萨斯城大都会区的所有儿科医生发送了一份调查问卷，以确定数据库之外可能的患者。纳入标准为临床肾病综合征或伴有肾活检的蛋白尿。共确定了148名患者（A组）；其中86名来自堪萨斯城大都会区（B组）。在A组中，微小病变病（MCD）和FSGS的发病率分别为52.7%[95%置信区间（CI）44%-60%]和23.0%（95%CI 16%-29%），在B组中分别为54.7%（95%CI 44%-65%）和24.5%（95%CI 15%-33%）。这些数字与儿童肾脏病国际研究（IS-KDC）报告的MCD发病率76.4%和FSGS发病率6.9%有显著差异。与ISKDC相似，在我们的研究人群中，6岁以上儿童的FSGS发病率高于年幼儿童（32.8%对16.7%，P = 0.028）。B组肾病综合征的年发病率为2.2例/10⁵名儿童/年，其中MCD为1.22例/10⁵名儿童/年，FSGS为0.5例/10⁵名儿童/年。非裔美国人原发性肾病综合征（3.6）和FSGS（1.6）的年发病率均显著高于白种人（分别为1.8例和0.3例/10⁵名儿童/年）。我们的研究表明，儿科原发性肾病综合征的年发病率几乎没有变化，但FSGS发病率较高，而MCD发病率相应下降。在非裔美国人和6岁以上儿童中，原发性肾病综合征由非MCD实体引起的可能性进一步增加。我们得出结论，必须仔细重新审视我们对儿童原发性肾病综合征为良性疾病的认识，并在我们所在地区采用更谨慎的预后方法。
