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[肾源性系统性纤维化:一种后果严重的罕见疾病]

[Nephrogenic systemic fibrosis: a rare disease with grave consequences].

作者信息

Kielstein J T, Schiffer M

机构信息

Abteilung Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Carl-Neuberg-Strasse 1, 30625 Hannover, Deutschland.

出版信息

Internist (Berl). 2010 Jan;51(1):39-44. doi: 10.1007/s00108-009-2408-9.

DOI:10.1007/s00108-009-2408-9
PMID:20033388
Abstract

Nephrogenic systemic fibrosis (NSF) is a progressive, potentially fatal systemic multiorgan fibrosing disease related to exposure of patients with advanced renal failure to the gadolinium-based contrast agents (GBCAs) used in magnetic resonance imaging. Because of this relationship between nephrogenic systemic fibrosis and gadolinium-based contrast agents, the U.S. Food and Drug Administration currently warns against using gadolinium-based contrast agents in patients with a glomerular filtration rate less than 30 ml per minute per 1.73 m(2), or any acute kidney injury related to the hepatorenal syndrome or perioperative liver transplantation. Linear non-ionic GBCAs that are more prone to release free gadolinium are the more likely to cause NSF. The mechanism for NSF is not fully understood, yet risk factors have been described. As there is no established therapy for NSF the prevention of exposure to gadolinium is crucial in high risk patients.

摘要

肾源性系统性纤维化(NSF)是一种进行性、潜在致命的系统性多器官纤维化疾病,与晚期肾衰竭患者接触磁共振成像中使用的钆基造影剂(GBCAs)有关。由于肾源性系统性纤维化与钆基造影剂之间的这种关系,美国食品药品监督管理局目前警告,对于肾小球滤过率低于每分钟30毫升/1.73平方米或任何与肝肾综合征或围手术期肝移植相关的急性肾损伤患者,不要使用钆基造影剂。更容易释放游离钆的线性非离子型GBCAs更有可能导致NSF。NSF的发病机制尚未完全明确,但已描述了相关风险因素。由于目前尚无针对NSF的确立疗法,对于高危患者而言,预防接触钆至关重要。

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引用本文的文献

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An Updated Study to Determine Association between Gadolinium-Based Contrast Agents and Nephrogenic Systemic Fibrosis.一项关于确定钆基造影剂与肾源性系统性纤维化之间关联的更新研究。
PLoS One. 2015 Jun 15;10(6):e0129720. doi: 10.1371/journal.pone.0129720. eCollection 2015.
2
[Complications due to contrast agent administration: what has been confirmed in prevention?].
Internist (Berl). 2010 Dec;51(12):1516-24. doi: 10.1007/s00108-010-2760-9.
3
Drug therapy in patients with chronic renal failure.慢性肾衰竭患者的药物治疗。

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UV-A1 therapy for nephrogenic systemic fibrosis.用于治疗肾源性系统性纤维化的UV-A1疗法。
Arch Dermatol. 2009 Oct;145(10):1170-4. doi: 10.1001/archdermatol.2009.245.
2
Nephrogenic systemic fibrosis: change in incidence following a switch in gadolinium agents and adoption of a gadolinium policy--report from two U.S. universities.肾源性系统性纤维化:钆造影剂更换和采用钆造影剂政策后发病率的变化——来自两所美国大学的报告。
Radiology. 2009 Dec;253(3):689-96. doi: 10.1148/radiol.2533090649. Epub 2009 Sep 29.
3
Transforming growth factor beta1 signalling, wound healing and repair: a multifunctional cytokine with clinical implications for wound repair, a delicate balance.
Dtsch Arztebl Int. 2010 Sep;107(37):647-55; quiz 655-6. doi: 10.3238/arztebl.2010.0647. Epub 2010 Sep 17.
转化生长因子β1信号传导、伤口愈合与修复:一种对伤口修复具有临床意义的多功能细胞因子,一种微妙的平衡。
Postgrad Med J. 2009 Jan;85(999):9-14. doi: 10.1136/pgmj.2008.069831.
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Current status of gadolinium toxicity in patients with kidney disease.肾病患者钆毒性的现状
Clin J Am Soc Nephrol. 2009 Feb;4(2):461-9. doi: 10.2215/CJN.06011108.
5
Effects of gadolinium-based magnetic resonance imaging contrast agents on human skin in organ culture and human skin fibroblasts.钆基磁共振成像造影剂对器官培养中的人体皮肤及人体皮肤成纤维细胞的影响。
Invest Radiol. 2009 Feb;44(2):74-81. doi: 10.1097/RLI.0b013e31818f76b5.
6
Nephrogenic systemic fibrosis associated with gadoversetamide exposure: treatment with sodium thiosulfate.与钆塞酸二钠暴露相关的肾源性系统性纤维化:硫代硫酸钠治疗
Am J Kidney Dis. 2009 Jan;53(1):133-7. doi: 10.1053/j.ajkd.2008.09.016. Epub 2008 Nov 20.
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Am J Kidney Dis. 2009 Jan;53(1):129-32. doi: 10.1053/j.ajkd.2008.08.029. Epub 2008 Nov 13.
8
Ultrastructural evidence of dermal gadolinium deposits in a patient with nephrogenic systemic fibrosis and end-stage renal disease.一名患有肾源性系统性纤维化和终末期肾病患者皮肤钆沉积的超微结构证据。
Clin J Am Soc Nephrol. 2008 Jul;3(4):968-75. doi: 10.2215/CJN.00100108. Epub 2008 Apr 2.
9
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AJR Am J Roentgenol. 2008 Mar;190(3):736-41. doi: 10.2214/AJR.07.3115.
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