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首页> 外文期刊>Seminars in dialysis >Patient characteristics and risk factors for nephrogenic systemic fibrosis following gadolinium exposure.
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Patient characteristics and risk factors for nephrogenic systemic fibrosis following gadolinium exposure.

机译:exposure暴露后肾原性系统性纤维化的患者特征和危险因素。

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摘要

Nephrogenic systemic fibrosis (NSF) is a systemic illness, which only affects patients with kidney failure. NSF risk increases with progressively lower levels of kidney function. It is characterized by red skin areas or plaques that develop over several weeks to painful thickened skin with a "woody" texture, resembling "peau d'orange." It may ultimately cause flexion contractures of joints. Skin biopsy reveals thickened collagen bundles, mucin deposition, proliferation of fibroblasts and elastic fibers, without inflammation. Originally described as nephrogenic fibrosing dermopathy (NFD), because of its primarily cutaneous manifestation, it was renamed NSF because of the involvement of various organs like the lungs, myocardium, or striated muscles. The pathogenesis of the disease is not known yet, but recently we suggested a strong association between development of NSF and exposure to gadolinium-based contrast (GBC) agents, thereafter confirmed by other authors. As a consequence of our recent observations, medical authorities imposed restrictions that exclude patients with advanced levels of renal insufficiency from potentially important magnetic resonance imaging studies with gadolinium. Unfortunately, the only alternatives in many situations (examination of brain, lungs, vasculature) are imaging modalities using iodinated radiocontrast agents. Thus, clinicians are faced with weighing the potential risk of NSF from GBC exposure against the risk of acute kidney injury-associated with radiocontrast media. In this dilemma, clinicians must identify patients at high-risk to develop NSF. Known risk factors critical for the development of NSF after exposure to GBC agents (certain chelates and higher doses) are end-stage renal disease requiring dialysis, especially those with little or no residual renal function, and advanced kidney disease not on dialysis. Other potential risk factors include metabolic acidosis, iron overload/intravenous iron, divalent ion disturbances, endothelial/vascular injury, and high erythropoietin doses. Further studies are required.
机译:肾源性系统性纤维化(NSF)是一种全身性疾病,仅影响肾衰竭患者。随着肾功能的逐渐降低,NSF风险增加。它的特征是红色的皮肤区域或斑块在数周内发展成疼痛的增厚皮肤,具有“木质”质地,类似于“橙皮”。最终可能导致关节屈曲挛缩。皮肤活检显示胶原束增厚,粘蛋白沉积,成纤维细胞和弹性纤维增生,无炎症。最初被描述为肾原性纤维化皮肤病(NFD),由于其主要是皮肤表现,由于涉及诸如肺,心肌或横纹肌等各种器官,因此更名为NSF。该病的发病机理尚不清楚,但最近我们提出了NSF的发展与暴露于g基造影剂(GBC)之间的强烈关联,此后其他作者证实了这一点。由于我们最近的观察结果,医学当局施加了一些限制,将肾功能不全的晚期患者排除在潜在重要的with成像研究中。不幸的是,在许多情况下(检查脑,肺,脉管系统)的唯一替代方法是使用碘化造影剂的成像方式。因此,临床医生面临着权衡GBC暴露引起的NSF潜在风险与放射性造影剂引起的急性肾损伤的风险之间的权衡。在这种困境中,临床医生必须识别出罹患NSF的高危患者。暴露于GBC试剂(某些螯合物和更高剂量)后,NSF发生的关键危险因素是需要透析的终末期肾脏疾病,尤其是那些残余肾功能很少或没有残留的肾脏疾病,以及未经透析的晚期肾脏疾病。其他潜在危险因素包括代谢性酸中毒,铁超负荷/静脉铁,二价离子紊乱,内皮/血管损伤和高促红细胞生成素剂量。需要进一步研究。

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