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一名16岁肺结核女孩的塌陷性肾小球病:全身炎症介质的作用

Collapsing glomerulopathy in a 16-year-old girl with pulmonary tuberculosis: the role of systemic inflammatory mediators.

作者信息

Coventry Susan, Shoemaker Lawrence R

机构信息

Department of Pathology, Kosair Children's Hospital, Louisville, KY 40202, USA.

出版信息

Pediatr Dev Pathol. 2004 Mar-Apr;7(2):166-70. doi: 10.1007/s10024-003-3027-4. Epub 2004 Mar 17.

Abstract

Idiopathic collapsing glomerulopathy is an aggressive variant of focal segmental glomerulosclerosis (FSGS) seen primarily in adults. Its etiology is unknown. Nearly identical pathology is seen in association with nephrotic syndrome in human immunodeficiency virus type 1 (HIV-1)-infected patients, raising the possibility that viral infection plays a role in pathogenesis. This is supported by the recent discovery of parvovirus B19 DNA in some cases of idiopathic collapsing glomerulopathy. We report a case of collapsing glomerulopathy in a 16-year-old girl who presented with steroid-resistant nephrotic syndrome and pulmonary tuberculosis. In the absence of the usual associations (adult age group, African-American race, or history of intravenous drug abuse), infection is the sole known risk factor in this case. This lends support to the hypothesis that immune dysregulation due to infection per se, rather than infection by specific viral agents, may lead to collapsing glomerulopathy in susceptible individuals.

摘要

特发性塌陷性肾小球病是局灶节段性肾小球硬化(FSGS)的一种侵袭性变异型,主要见于成年人。其病因不明。在感染人类免疫缺陷病毒1型(HIV-1)的患者中,与肾病综合征相关的病理表现几乎相同,这增加了病毒感染在发病机制中起作用的可能性。在一些特发性塌陷性肾小球病病例中发现细小病毒B19 DNA,这支持了上述观点。我们报告一例16岁女孩发生塌陷性肾小球病,该女孩表现为激素抵抗型肾病综合征和肺结核。在没有常见相关因素(成人年龄组、非裔美国人种族或静脉药物滥用史)的情况下,感染是该病例中唯一已知的危险因素。这支持了以下假说:感染本身导致的免疫失调,而非特定病毒病原体的感染,可能在易感个体中导致塌陷性肾小球病。

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