• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

[10岁以上儿童原发性肾病综合征的临床表现及预后]

[Clinical presentation and prognosis in children over 10-year-old with primary nephrotic syndrome].

作者信息

Tu J, Chen C Y, Yang H X, Jia Y, Geng H Y, Li H R

机构信息

Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics,Beijing 100020, China.

出版信息

Zhonghua Er Ke Za Zhi. 2023 Aug 2;61(8):708-713. doi: 10.3760/cma.j.cn112140-20230104-00007.

DOI:10.3760/cma.j.cn112140-20230104-00007
PMID:37528011
Abstract

To summary the clinical presentation and prognosis of primary nephrotic syndrome (PNS) in teenagers. The clinical data, renal pathological types and prognosis of 118 children over 10-year-old with PNS treated in the Department of Nephrology of the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2020 were retrospectively analyzed, with 408 children ≤10-year-old as control group synchronously. Chi-square test was used to compare the difference of clinical types, pathologic types, response to steroids and tubulointerstitial changes between the groups. The teenagers with steroid resistant nephrotic syndrome (SRNS) were divided into initial non-responder group and late non-responder group. Kaplan-Meier method was used to compare the difference of persistent proteinuria, and Fisher's exact test for the histological types. There were 118 children >10-year-old, including 74 males and 44 females, with the onset age of 12.1 (10.8, 13.4) years; and 408 children ≤10-year-old with the onset age of 4.5 (3.2, 6.8) years. The proportion of SRNS was significantly higher in patients >10-year-old than those ≤10-year-old (24.6% (29/118) 15.9% (65/408), =4.66, 0.031). There was no statistical difference in the pathological types between >10-year-old and ≤10-year-old (>0.05), with minimal change disease the most common type (56.0% (14/25) 60.5% (26/43)). The percentage of cases with renal tubulointerstitial lesions was significantly higher in children >10-year-old compared to those ≤10-year-old (60.0% (15/25) 23.3% (10/43), =9.18, 0.002). There were 29 cases presented with SRNS in PNS over 10-year-old, including 19 initial non-responders and 10 late non-responders. Analyzed by Kaplan-Meier curve, it was shown that the percentage of persistent proteinuria after 6 months of immunosuppressive treatments was significantly higher in initial non-responders than those of the late non-responders ((22±10)% 0, =14.68, 0.001); the percentage of minimal change disease was significantly higher in patients of late non-responders than those of the initial non-responders (5/6 3/13, 0.041). Of the 63 >10-year-old with steroid-sensitive nephrotic syndrome followed up more than one year, 38 cases (60.3%) had relapse, and 14 cases (22.2%) were frequent relapse nephrotic syndrome and steroid dependent nephrotic syndrome. Among the 45 patients followed up over 18-year-old, 22 cases (48.9%) had recurrent proteinuria continued to adulthood, 3 cases of SRNS progressed to kidney insufficiency, and one of them developed into end stage kidney disease and was administrated with hemodialysis. Cases over 10-year-old with PNS tend to present with SRNS and renal tubulointerstitial lesions. They have a favorable prognosis, but are liable to relapse in adulthood.

摘要

总结青少年原发性肾病综合征(PNS)的临床表现及预后。回顾性分析2010年1月至2020年12月在首都儿科研究所附属儿童医院肾内科治疗的118例10岁以上PNS患儿的临床资料、肾脏病理类型及预后情况,并同步选取408例10岁及以下患儿作为对照组。采用卡方检验比较两组间临床类型、病理类型、对激素的反应及肾小管间质改变的差异。将激素抵抗型肾病综合征(SRNS)青少年患者分为初始无反应组和晚期无反应组。采用Kaplan-Meier法比较持续性蛋白尿的差异,采用Fisher确切概率法比较组织学类型的差异。118例10岁以上患儿中,男74例,女44例,发病年龄为12.1(10.8,13.4)岁;408例10岁及以下患儿发病年龄为4.5(3.2,6.8)岁。10岁以上患者中SRNS的比例显著高于10岁及以下患者(24.6%(29/118)比15.9%(65/408),χ² =4.66,P =0.031)。10岁以上与10岁及以下患者的病理类型差异无统计学意义(P>0.05),最常见类型为微小病变型(56.0%(14/25)比60.5%(26/43))。10岁以上儿童肾小管间质病变的比例显著高于10岁及以下儿童(60.0%(15/25)比23.3%(10/43),χ² =9.18,P =0.002)。10岁以上PNS患者中29例为SRNS,其中19例为初始无反应者,10例为晚期无反应者。通过Kaplan-Meier曲线分析显示,免疫抑制治疗6个月后持续性蛋白尿的比例在初始无反应者中显著高于晚期无反应者((22±10)%比0,χ² =14.68,P =0.001);晚期无反应者中微小病变型的比例显著高于初始无反应者(5/6比3/13,P =0.041)。63例随访1年以上的10岁以上激素敏感型肾病综合征患者中,38例(60.3%)复发,14例(22.2%)为频复发肾病综合征及激素依赖型肾病综合征。45例随访18岁以上患者中,22例(48.9%)蛋白尿复发持续至成年期,3例SRNS进展为肾功能不全,其中1例发展为终末期肾病并接受血液透析治疗。10岁以上PNS患儿易出现SRNS及肾小管间质病变。预后较好,但成年后易复发。

相似文献

1
[Clinical presentation and prognosis in children over 10-year-old with primary nephrotic syndrome].[10岁以上儿童原发性肾病综合征的临床表现及预后]
Zhonghua Er Ke Za Zhi. 2023 Aug 2;61(8):708-713. doi: 10.3760/cma.j.cn112140-20230104-00007.
2
[Multicenter study on present status of diagnosis and treatment of steroid-resistant nephrotic syndrome in children].[儿童激素抵抗型肾病综合征诊治现状的多中心研究]
Zhonghua Er Ke Za Zhi. 2014 Jul;52(7):483-7.
3
Follow-up of steroid-resistant nephrotic syndrome: tubular proteinuria and enzymuria.激素抵抗型肾病综合征的随访:肾小管蛋白尿和酶尿
Pediatr Nephrol. 2000 Dec;15(3-4):252-8. doi: 10.1007/s004670000472.
4
Correlation of fractional excretion of magnesium with steroid responsiveness in children with nephrotic syndrome.肾病综合征患儿镁排泄分数与类固醇反应性的相关性
Saudi J Kidney Dis Transpl. 2014 Jul;25(4):830-6. doi: 10.4103/1319-2442.135173.
5
[Four cases of nephrotic syndrome with TRPC6 gene variations and literature review].[4例伴有瞬时受体电位阳离子通道蛋白6(TRPC6)基因变异的肾病综合征病例及文献复习]
Zhonghua Er Ke Za Zhi. 2021 Mar 2;59(3):223-227. doi: 10.3760/cma.j.cn112140-20200824-00822.
6
[Multicenter survey of diagnostic and therapeutic status in Chinese childhood patients with steroid-sensitive, relaping/steroid-dependent nephrotic syndrome].[中国儿童激素敏感、复发/激素依赖型肾病综合征诊断与治疗现状的多中心调查]
Zhonghua Er Ke Za Zhi. 2014 Mar;52(3):194-200.
7
Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis.环孢素治疗儿童激素耐药性肾病的 5 年前瞻性随访。
Pediatr Nephrol. 2013 May;28(5):765-71. doi: 10.1007/s00467-012-2393-4. Epub 2013 Jan 13.
8
Baseline characteristics and long-term outcomes of steroid-resistant nephrotic syndrome in children: impact of initial kidney histology.儿童激素耐药性肾病综合征的基线特征和长期结局:初始肾脏组织学的影响。
Pediatr Nephrol. 2020 Dec;35(12):2377-2381. doi: 10.1007/s00467-020-04760-8. Epub 2020 Sep 22.
9
[Establishment and validation of clinical prediction model for steroid-resistant nephrotic syndrome in children].[儿童激素抵抗型肾病综合征临床预测模型的建立与验证]
Zhonghua Er Ke Za Zhi. 2023 Apr 2;61(4):333-338. doi: 10.3760/cma.j.cn112140-20220924-00837.
10
Steroid-resistant idiopathic childhood nephrosis: overdiagnosed and undertreated.类固醇抵抗型儿童特发性肾病:诊断过度与治疗不足。
Nephrol Dial Transplant. 2007 Aug;22(8):2183-93. doi: 10.1093/ndt/gfm092. Epub 2007 May 15.

引用本文的文献

1
Associations between demographic and disease related factors and anxiety and depression among adolescents with chronic kidney disease.慢性肾病青少年的人口统计学和疾病相关因素与焦虑和抑郁之间的关联。
Front Pediatr. 2025 Aug 22;13:1616765. doi: 10.3389/fped.2025.1616765. eCollection 2025.