Minimal change disease: a dysregulation of the podocyte CD80-CTLA-4 axis?

Minimal change disease: a dysregulation of the podocyte CD80-CTLA-4 axis?
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DOI:
10.1007/s00467-014-2874-8
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发表时间:
2014-12
影响因子:
3
通讯作者:
Garin, Eduardo H.
Garin, Eduardo H.
中科院分区:
医学3区
文献类型:
--
作者:
Cara-Fuentes, Gabriel;Wasserfall, Clive H.;Wang, Heiman;Johnson, Richard J.;Garin, Eduardo H.

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微小病变病(MCD)与足细胞中的CD 80表达和活动性疾病期间尿CD 80排泄升高相关。我们评估了MCD患者肾病综合征不同阶段的CTLA-4和CD 80尿排泄,以检验以下假设:复发时MCD患者尿CD 80排泄持续增加是由于宿主对CTLA-4无效应答减少CD 80活化所致。在复发和/或缓解期间研究了32例经活检证实的MCD儿童。11例健康体检者作为对照组。与缓解期MCD患者(p<0.001)和对照组(p<0.001)相比,复发期MCD患者的尿CD 80排泄量显著增加。尽管复发的MCD患者的尿CTLA-4排泄高于缓解的MCD患者(p=0.01)和对照组(p=0.03),但在复发时的MCD患者中未观察到尿CD 80和尿CTLA-4之间的显著相关性(p=0.06)。在缓解时,所有患者的CD 80均显著降低,但除5例患者外,所有患者的CTLA-4均降低或保持不变,并且在尿CD 80和CTLA-4之间未观察到相关性(p=0.7)。尿CTLA-4水平与尿CD 80排泄不相关,提示CTLA-4应答可能在这种疾病复发期间是次优的。
Minimal Change Disease (MCD) is associated with CD80 expression in podocytes and elevated urinary CD80 excretion during active disease. We have evaluated the CTLA-4 and CD80 urinary excretion during different stages of the nephrotic syndrome in patients with MCD to test the hypothesis that the persistent increased urinary CD80 excretion in MCD in relapse is due to an ineffectual CTLA-4 response of the host to curtail the CD80 activation. 32 children with biopsy-proven MCD were studied during relapse and/or remission. 11 healthy subjects served as controls. Urinary CD80 excretion was significantly increased in MCD patients in relapse when compared to MCD patients in remission (p<0.001) and controls (p<0.001). Although urinary CTLA-4 excretion was higher in MCD patients in relapse than in MCD patients in remission (p=0.01) and controls (p=0.03), no significant correlation was observed between urinary CD80 and urinary CTLA-4 in MCD patients at the time of relapse (p=0.06). At the time of remission, CD80 has decreased significantly in all patients but CTLA-4 either decreased or remained unchanged in all but five patients and no correlation was observed between urinary CD80 and CTLA-4 (p=0.7). Urinary CTLA-4 levels do not correlate with urinary CD80 excretion, suggesting the possibility that the CTLA4 response may be suboptimal in this disease during relapse.
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