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一名非亚洲男性中与免疫性血小板减少症相关的肾病综合征并发木村病

Nephrotic syndrome associated with immune thrombocytopenia revealing Kimura's disease in a non-Asian male.

作者信息

Noel Nicolas, Meignin Véronique, Rosenstingl Sophie, Ronco Pierre, Boffa Jean-Jacques

机构信息

Department of Nephrology, AP-HP , Hôpital Tenon , F-75020.

Department of Pathology, AP-HP , Hôpital Saint-Louis , Paris.

出版信息

NDT Plus. 2009 Dec;2(6):452-4. doi: 10.1093/ndtplus/sfp099. Epub 2009 Aug 7.

DOI:10.1093/ndtplus/sfp099
PMID:25949378
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4421322/
Abstract

We report the case of a young Caucasian man presenting with diffuse oedema and nephrotic syndrome. Clinical examination revealed multiple lymphadenopathies. Histological examination was consistent with the diagnosis of Kimura's disease. A renal biopsy showed focal segmental glomerulosclerosis. Immune thrombocytopenia and signs of humoral autoimmunity were discovered. Corticosteroid treatment induced remission of nephrotic syndrome but relapses occurred 12 and 18 months after onset of treatment while the patient was receiving 20 mg prednisone once a day. To our knowledge, this is the first case of Kimura's disease and nephrotic syndrome associated with B-cell autoreactivity.

摘要

我们报告了一例年轻的白种男性患者，其表现为弥漫性水肿和肾病综合征。临床检查发现多处淋巴结肿大。组织学检查结果符合木村病的诊断。肾活检显示局灶节段性肾小球硬化。还发现了免疫性血小板减少症和体液自身免疫的迹象。皮质类固醇治疗使肾病综合征缓解，但在治疗开始后的12个月和18个月出现复发，当时患者每天服用20毫克泼尼松。据我们所知，这是首例与B细胞自身反应性相关的木村病和肾病综合征病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a83/4421322/98682c435856/sfp099fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a83/4421322/733b0a5c8372/sfp099fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a83/4421322/98682c435856/sfp099fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a83/4421322/733b0a5c8372/sfp099fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a83/4421322/98682c435856/sfp099fig2.jpg

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Nephrotic syndrome in Kimura's disease: apropos a case of the glomerular tip lesion in an African-Caribbean male.木村病中的肾病综合征：关于一名非洲加勒比男性肾小球顶端病变的病例
NDT Plus. 2011 Feb;4(1):60-2. doi: 10.1093/ndtplus/sfq167. Epub 2010 Oct 4.
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A novel approach to investigation of the pathogenesis of active minimal-change nephrotic syndrome using subtracted cDNA library screening.一种利用消减cDNA文库筛选研究活动性微小病变型肾病综合征发病机制的新方法。
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