Tu J, Chen C Y, Yang H X, Jia Y, Geng H Y, Li H R
Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics,Beijing 100020, China.
Zhonghua Er Ke Za Zhi. 2023 Aug 2;61(8):708-713. doi: 10.3760/cma.j.cn112140-20230104-00007.
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.
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。