摘要
目的:探讨抗中性粒细胞胞浆抗体(ANCA)相关性肾炎(AAGN)肾脏风险评分(ARRS)对中国AAGN儿童肾脏预后预测的价值。方法:回顾性分析2013年1月至2019年8月单中心诊断为AAGN的22例患儿的临床及病理特点。以是否进展至慢性肾脏病(CKD)5期(即终末期肾脏病)作为分组依据,分为终点组及非终点组,对导致肾脏不良结局的相关危险因素进行探讨。根据ARRS标准,将患儿分为低、中、高风险组,比较3组患儿肾脏实际生存情况。结果:22例AAGN患儿男女比例为1∶2.67,高峰起病年龄为6~12岁。肾脏受累以蛋白尿最多见,54.55%患儿确诊时估算肾小球滤过率(eGFR)<30 mL/(min·1.73 m2)。16例患儿行肾脏病理检查,肾小球病变以新月体形成为主,其中细胞纤维性新月体最多见;肾间质病变主要表现为炎症细胞浸润。肾外脏器受累按发生率排序依次为肺、皮肤、鼻、黏膜/眼、神经系统等。具备随访资料的20例患儿,中位随访时间10.1个月。随访1、2、5、7年时肾脏累积生存率分别为55.56%、50.00%、37.50%、28.13%。诊断初期低eGFR水平为AAGN儿童患者进展为CKD 5期的高危因素(P=0.019)。14例患儿采用ARRS评分进行评估,随访36个月时,低、中、高风险组肾脏累积存活率分别为100.00%、75.00%、25.00%,3组患儿肾脏累积生存率比较差异无统计学意义(log-rank=3.252,P=0.197)。结论:儿童AAGN肾脏损害程度严重,诊断时eGFR水平较低者更易进展为CKD 5期。当前ARRS系统对中国AAGN儿童肾结局的预测能力存在一定局限性,亟待构建更有效的预测模型,以提升对中国AAGN儿童肾脏预后预测的精准度与有效性。
Objective:To explore the value of anti-neutrophil cytoplasmic antibody(ANCA)-associated glomerulonephritis(AAGN)renal risk score(ARRS)in predicting the renal prognosis of children with AAGN in China.Methods:Retrospective analysis was performed on clinical and pathological characteristics of 22 children diagnosed with AAGN in a single center from Jan.2013 to Aug.2019.Based on whether the children progressed to stage 5 chronic kidney disease(CKD,end-stage renal disease),the children were divided into the end-point group and the non-end-point group.Risk factors leading to adverse renal outcomes were discussed.According to ARRS criterion,the children were divided into the low-risk,medium-risk,and high-risk group,and the actual renal survival situations of three groups were compared.Results:The male to female ratio of the 22 children with AAGN was 1∶2.67,and the peak onset age was 6 to 12 years old.Regarding renal manifestations,proteinuria was the most prevalent indication of renal involvement.Upon diagnosis,the estimated glomerular filtration rate(eGFR)of 54.55%of the children was<30 mL/(min·1.73m2).Renal pathological examinations were performed on 16 children.Glomerular pathologies mainly presented as crescent formation,with cellular-fibrous crescents being the most common.In terms of renal interstitial lesions,inflammatory cell infiltration was the dominant feature.Regarding extrarenal organ involvement,the descending order of frequency was lung,skin,nose,mucosa/eyes,and nervous system.Follow-up information was accessible for 20 children,with a median follow-up duration of 10.1 months.The one-year,two-year,five-year,and seven-year cumulative renal survival rates during the follow-up were respectively 55.56%,50.00%,37.50%,and 28.13%.A lower eGFR value at diagnosis was identified as a high-risk determinant for AAGN-affected children to progress to stage 5 CKD(P=0.019).ARRS was used to evaluate 14 children,at 36-month follow-up,the cumulative renal survival rates of the low-risk,medium-risk,and high-risk groups were respectively 100.00%,75.00%,and 25.00%.No statistically significant variation was observed in the cumulative renal survival rates among three groups(log-rank test=3.252,P=0.197).Conclusion:Children with AAGN have severe renal injury,and those with lower eGFR level at diagnosis are more likely to progress to stage 5 CKD.The current ARRS system has certain limitations in predicting the renal outcomes of children in China.It is urgent to establish a more effective prediction model to improve the accuracy and effectiveness of predicting the renal outcomes of children in China.
作者
陈宵宇
万俊丽
谭力文
焦佳
杨琴
钟诚
张高福
阳海平
李秋
王墨
Chen Xiaoyu;Wan Junli;Tan Liwen;Jiao Jia;Yang Qin;Zhong Cheng;Zhang Gaofu;Yang Haiping;Li Qiu;Wang Mo(Children’s Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,National Clinical Research Center for Child Health and Disorders,Key Laboratory of Children’s Vital Organ Development and Diseases of Chongqing Health Commission,Chongqing Key Laboratory of Pediatric Metabolism and Inflammatory Disease,Chongqing400014,China;Chongqing Yubei District Maternal and Child Health Hospital,Chongqing401120,China)
出处
《儿科药学杂志》
2025年第5期1-6,共6页
Journal of Pediatric Pharmacy
基金
国家重点研发计划“生育健康及妇女儿童健康保障”重点专项,编号2021YFC2702002
国家自然科学基金支持项目,编号82470745。
关键词
儿童
抗中性粒细胞胞浆抗体
抗中性粒细胞胞浆抗体相关性血管炎
抗中性粒细胞胞浆抗体相关性肾炎
children
anti-neutrophil cytoplasmic antibodies
anti-neutrophil cytoplasmic antibodies-associated vasculitis
anti-neutrophil cytoplasmic antibodies-associated glomerulonephritis
