Ray Patricio E
Children's Research Institute, Children's National Medical Center, Washington, D.C. 20010, USA.
Pediatr Nephrol. 2009 Nov;24(11):2109-19. doi: 10.1007/s00467-009-1155-4. Epub 2009 Mar 14.
Childhood human immunodeficiency virus-associated nephropathy (HIVAN) is defined by the presence of proteinuria associated with mesangial hyperplasia and/or global-focal segmental glomerulosclerosis, in combination with the microcystic transformation of renal tubules. This review discusses the pathogenesis of childhood HIVAN and explores how the current pathological paradigm for HIVAN in adults can be applied to children. The Human Immunodeficiency Virus-1 (HIV-1) induces renal epithelial injury in African American children with a genetic susceptibility to develop HIVAN. The mechanism is not well understood, since renal epithelial cells harvested from children with HIVAN do not appear to be productively infected. Children with HIVAN show a renal up-regulation of heparan sulphate proteoglycans and a recruitment of circulating heparin-binding growth factors, chemokines, and mononuclear cells. Macrophages appear to establish a renal HIV-reservoir and transfer viral particles to renal epithelial cells. All of these changes seem to trigger an aberrant and persistent renal epithelial proliferative response. The paradigm that viral products produced by infected renal epithelial cells per se induce the proliferation of these cells is not supported by data available in children with HIVAN. More research is needed to elucidate how HIV-1 induces renal epithelial injury and proliferation in HIV-infected children.
儿童人类免疫缺陷病毒相关性肾病(HIVAN)的定义为:存在与系膜增生和/或全球局灶节段性肾小球硬化相关的蛋白尿,并伴有肾小管的微囊性改变。本综述讨论了儿童HIVAN的发病机制,并探讨了目前成人HIVAN的病理模式如何应用于儿童。人类免疫缺陷病毒1型(HIV-1)在具有发生HIVAN遗传易感性的非裔美国儿童中诱发肾上皮损伤。其机制尚不完全清楚,因为从HIVAN患儿采集的肾上皮细胞似乎未被有效感染。HIVAN患儿表现出肾硫酸乙酰肝素蛋白聚糖上调,以及循环中肝素结合生长因子、趋化因子和单核细胞的募集。巨噬细胞似乎建立了肾HIV储存库,并将病毒颗粒转移至肾上皮细胞。所有这些变化似乎都引发了异常且持续的肾上皮增殖反应。感染的肾上皮细胞本身产生的病毒产物诱导这些细胞增殖的模式,并未得到HIVAN患儿现有数据的支持。需要更多研究来阐明HIV-1如何在HIV感染儿童中诱发肾上皮损伤和增殖。