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补体成分C3激活在儿童IgA肾病临床表现及预后中的作用——一项全国性儿童研究

The Role of Complement Component C3 Activation in the Clinical Presentation and Prognosis of IgA Nephropathy-A National Study in Children.

作者信息

Mizerska-Wasiak Małgorzata, Such-Gruchot Agnieszka, Cichoń-Kawa Karolina, Turczyn Agnieszka, Małdyk Jadwiga, Miklaszewska Monika, Drożdż Dorota, Firszt-Adamczyk Agnieszka, Stankiewicz Roman, Rybi-Szumińska Agnieszka, Wasilewska Anna, Szczepańska Maria, Bieniaś Beata, Sikora Przemysław, Pukajło-Marczyk Agnieszka, Zwolińska Danuta, Pawlak-Bratkowska Monika, Tkaczyk Marcin, Zachwieja Jacek, Drożyńska-Duklas Magdalena, Żurowska Aleksandra, Gadomska-Prokop Katarzyna, Grenda Ryszard, Pańczyk-Tomaszewska Małgorzata

机构信息

Department of Pediatrics and Nephrology, Medical University of Warsaw, 02-091 Warsaw, Poland.

Department of Pathology, Medical University of Warsaw, 02-091 Warsaw, Poland.

出版信息

J Clin Med. 2021 Sep 26;10(19):4405. doi: 10.3390/jcm10194405.

DOI:10.3390/jcm10194405
PMID:34640422
原文链接:
https://pmc.ncbi.nlm.nih.gov/articles/PMC8509710/
Abstract

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up. Kidney biopsy was categorized using the Oxford classification, with a calculation of the MEST-C score. The intensity of IgA and C3 deposits were rated from 0 to +4 in immunofluorescence microscopy. The intensity of mesangial C3 > +1 deposits in kidney biopsy has an effect on renal survival with normal GFR in children with IgAN. A reduced serum C3 level has not been a prognostic factor in children but perhaps this finding should be confirmed in a larger group of children.

摘要

本研究的目的是评估肾活检中系膜C3沉积强度和血清C3水平对儿童IgA肾病临床病程及预后的影响。该研究纳入了波兰儿童IgA肾病登记处的148名儿童,均基于肾活检确诊。在研究组中,于基线期和随访结束时对蛋白尿、肌酐、IgA、C3进行了两次评估。肾活检采用牛津分类法进行分类,并计算MEST-C评分。在免疫荧光显微镜下,IgA和C3沉积强度从0至+4进行分级。肾活检中系膜C3> +1沉积强度对IgA肾病儿童正常肾小球滤过率下的肾脏存活率有影响。血清C3水平降低在儿童中并非预后因素,但或许这一发现应在更大规模的儿童群体中得到证实。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/4b8875f0abb5/jcm-10-04405-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/31d383d2a896/jcm-10-04405-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/11967a3d2e95/jcm-10-04405-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/534979bbd1ef/jcm-10-04405-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/4b8875f0abb5/jcm-10-04405-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/31d383d2a896/jcm-10-04405-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/11967a3d2e95/jcm-10-04405-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/534979bbd1ef/jcm-10-04405-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8480/8509710/4b8875f0abb5/jcm-10-04405-g004.jpg

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本文引用的文献

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BMC Nephrol. 2021 Jan 29;22(1):43. doi: 10.1186/s12882-021-02237-w.
2
IgA nephropathy in children and in adults: two separate entities or the same disease?儿童和成人 IgA 肾病:两种独立的实体还是同一种疾病?
J Nephrol. 2020 Dec;33(6):1219-1229. doi: 10.1007/s40620-020-00725-0. Epub 2020 Apr 20.
3
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Pediatr Nephrol. 2023 May;38(5):1439-1448. doi: 10.1007/s00467-022-05644-9. Epub 2022 Aug 2.
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