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儿童局灶节段性肾小球硬化:一项纵向评估

Focal and segmental glomerulosclerosis in children: a longitudinal assessment.

作者信息

Boyer Olivia, Moulder Janelle K, Somers Michael J G

机构信息

Division of Nephrology, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA.

出版信息

Pediatr Nephrol. 2007 Aug;22(8):1159-66. doi: 10.1007/s00467-007-0493-3. Epub 2007 Apr 17.

Abstract

Recent data suggest that the histologic finding of focal and segmental glomerulosclerosis (FSGS) is increasing among children. There are, however, limited longitudinal pediatric data on prevalence, demographics, and steroid responsiveness in FSGS. We identified 201 consecutive nephrotic children diagnosed between 1977 and 2002 with 2 years follow-up; 51% had undergone renal biopsy due to steroid sequelae or resistance; 48 children with FSGS were diagnosed. Compared with non-FSGS children, FSGS children were older at diagnosis (6.9 years vs 4.4 years, P<0.02), more likely girls (54% vs 28%, P<0.02), Black or Hispanic (42% vs 16%, P<0.001), and the FSGS was more likely to be steroid resistant (73% vs 10%, P<0.001). To assess for longitudinal differences, we grouped children by presentation: pre-1985, between 1985 and 1995, and post-1995. There was no difference in proportion of children biopsied or diagnosed with FSGS during each interval. Among FSGS children, there was no difference in racial or gender composition in each period, but there was a difference in age at diagnosis (2.6 vs 5.7 vs 8.5 years; P=0.01), also observed in the non-FSGS children (2.2 vs 3.9 vs 4.9 years; P=0.02). In contradistinction to non-FSGS children, there was a marked increase in steroid resistance with FSGS (43% vs 62% vs 86%; P=0.03).

摘要

近期数据表明,局灶节段性肾小球硬化(FSGS)的组织学表现在儿童中呈上升趋势。然而,关于FSGS的患病率、人口统计学特征及对类固醇反应性的儿科纵向数据有限。我们确定了1977年至2002年间连续诊断的201例肾病患儿,并进行了2年随访;51%的患儿因类固醇后遗症或耐药性接受了肾活检;确诊为FSGS的患儿有48例。与非FSGS患儿相比,FSGS患儿诊断时年龄更大(6.9岁对4.4岁,P<0.02),更可能为女孩(54%对28%,P<0.02),黑人或西班牙裔(42%对16%,P<0.001),且FSGS更可能对类固醇耐药(73%对10%,P<0.001)。为评估纵向差异,我们按就诊时间将患儿分组:1985年前、1985年至1995年间、1995年后。各时间段内接受活检或诊断为FSGS的患儿比例无差异。在FSGS患儿中,各时期的种族或性别构成无差异,但诊断时年龄有差异(2.6岁对5.7岁对8.5岁;P=0.01),非FSGS患儿中也观察到类似差异(2.2岁对3.9岁对4.9岁;P=0.02)。与非FSGS患儿不同,FSGS患儿的类固醇耐药性显著增加(43%对62%对86%;P=0.03)。

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