Predominant but silent C1q deposits in mesangium on transplanted kidneys - long-term observational study.
Predominant but silent C1q deposits in mesangium on transplanted kidneys - long-term observational study.
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DOI：
10.1186/s12882-018-0874-9
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发表时间：
2018-04-06
期刊：
影响因子：
2.3
通讯作者：
Hattori M
中科院分区：
文献类型：
--
作者：
Kanai T;Akioka Y;Miura K;Hisano M;Koike J;Yamaguchi Y;Hattori M
C1 q肾病（C1 qN）最早被描述为肾小球疾病，其特征是在无系统性红斑狼疮证据的蛋白尿患者中的主要肾小球C1 q沉积。然而，一些研究揭示了C1 qN的临床异质性，显示了一些尿分析正常的病例。为了证实存在主要系膜C1 q沉积和阴性或轻度蛋白尿和/或血尿的病例，我们研究了显示阴性至轻度蛋白尿（通过试纸试验小于或等于1+）和/或血尿的肾移植活检标本。符合条件的参与者是符合C1 qN标准的肾移植病例，随访时间超过10年。回顾他们的医疗记录，以确定检测主要系膜C1 q沉积时的年龄、性别、原发性肾病和肾移植活检的原因、血压、蛋白尿和血尿的程度以及血清肌酐水平。在414例成人和儿童病例中，5例儿科患者（男女比例为1：1.5）合格。2例系膜区C1 q明显沉积时有轻度蛋白尿，无血尿，其余3例尿常规正常。光镜检查均见肾小球轻度异常。免疫荧光检查显示所有病例的系膜C1 q沉积均以IgG、IgM和C3为主。所选标本均在系膜区出现电子致密沉积。10年后2例尿常规正常，3例轻度蛋白尿无血尿。在随访期间，没有病例出现持续性蛋白尿和/或尿血（试纸试验大于或等于2+）。无一例发生系统性红斑狼疮。2例患者于术后8年复查移植肾活检1次。他们表现出轻微的肾小球异常。1例C1 q存款消失。在另一个病例中，未检查免疫荧光研究。这项对移植肾的长期观察性研究证实了存在着大量但无症状的C1 q沉积在系膜中的病例，这些病例有阴性或轻度蛋白尿。本文的在线版本（10.1186/s12882-018-0874-9）包含补充材料，可供授权用户使用。
C1q nephropathy (C1qN) was first described as glomerular disease characterized by predominant meangial C1q deposits in patients with proteinuria and no evidence of systemic lupus erythematosus. Several studies, however, revealed the clinical heterogeneity of C1qN, showing some cases with normal urinalysis. To confirm the existence of cases with predominant mesangial C1q deposits and negative or mild proteinuria and/or hematuria, we investigated renal graft biopsy specimens showing negative to mild proteinuria (less than or equal to 1+ by dip stick test) and/or hematuria. Eligible participants were kidney transplant cases who corresponded to the criteria for C1qN and were followed more than 10 years. Their medical records were reviewed to determine the age at detection of predominant mesangial C1q deposits, gender, original renal disease and reason for renal graft biopsy, blood pressure, degree of proteinuria and hematuria, and serum creatinine levels. From 414 cases in adults and children, five pediatric patients (the male to female ratio, 1:1.5) were eligible. At the time when predominant mesangial C1q deposits were detected, 2 cases presented with mild proteinuria without hematuria, but the other 3 cases showed normal urinalysis. Light microscopy revealed minor glomerular abnormality in all the cases. Immunofluorescent study showed predominant mesangial C1q deposits with IgG, IgM and C3 in all cases. All selected specimens presented electron dense-depos in the mesangium. Ten years later from the detection, 2 cases continued to be normal urinalysis and 3 cases had mild proteinuria without hematuria. During this follow-up period, no cases presented with persistent proteinuria and/or hematuria greater than or equal to 2+ by dip stick test. And no cases developed systemic lupus erythematosus. Follow-up renal graft biopsies were performed once in 2 cases 8 years later from the detection. They showed minor glomerular abnormalities. C1q deposit disappeared in one case. In another case, immunofluorescent study was not examined. This long-term observational study on transplanted kidneys confirms the existence of cases with predominant but silent C1q deposits in the mesangium who have negative or mild proteinuria. The online version of this article (10.1186/s12882-018-0874-9) contains supplementary material, which is available to authorized users.
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影响因子：
3
作者：
Levart, TK;Kenda, R;Vizjak, A
通讯作者：
Vizjak, A
影响因子：
13.6
作者：
Vizjak, Alenka;Ferluga, Dugan;Jennette, J. Charles
通讯作者：
Jennette, J. Charles
影响因子：
13.2
作者：
JENNETTE, JC;HIPP, CG
通讯作者：
HIPP, CG
影响因子：
2.3
作者：
Nangaku, Masaomi;Couser, William G
通讯作者：
Couser, William G
影响因子：
19.6
作者：
Markowitz, GS;Schwimmer, JA;D'Agati, VD
通讯作者：
D'Agati, VD