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Nephrogenic Systemic Fibrosis: A New Threat in the Use of Gadolinium-Based Contrast Media

腎因性全身性纖維化症使用含釓之核磁共振顯影劑的新危機

摘要


腎因性全身性纖維化症(Nephrogenic systemic fibrosis, NSF),即之前的腎因性皮膚纖維化症(Nephrogenic fibrosis dermopathy)爲一種全新的病症。其自1997年首次被發現後才陸續被報導。此病之表徵主要爲類硬皮症般的皮膚纖維化,且可能合併心、肺、肝、橫紋肌等全身器官的病變。NSF幾乎只發生在於腎臟功能衰竭的病人,並可能和使用含釓(Gadolinium)的核磁共振顯影劑有關,NSF的個案中以使用Gadodiamide (Omniscan(superscript ®))之後發病最常被報導。此病的標準診斷方式是深層皮膚切片,病理組織學上會顯現CD 34/procollagen雙陽性的紡錘細胞,較獨特的是它沒有發炎現象。在鑑別診斷方面,需要與蜂窩性組織炎,藥物反應,硬皮症,厚皮性粘液水腫及全身性硬化症等疾病等作區分。由於NSF的致病機轉至今不明,加上沒有經證實有效的治療方式,故此病的預防甚爲重要。美國食品及藥物管理局(FDA)建議在腎衰竭的病人,無論是急性或慢性,凡腎絲球濾過率小於30ml/min/1.73m^2者要避免使用含釓之核磁共振顯影劑,除非此檢查極其重要且無法被取代。同時使用含釓顯影劑時,其最大劑量不可超出藥廠建議劑量,並且兩次檢查之間要有充裕的時間讓含釓顯影劑排出體外。在透析病人使用含釓顯影劑後,建議盡快安排血液透析以利釓的排出。

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並列摘要


Nephrogenic systemic fibrosis (NSF) which used to be named nephrogenic fibrosing dermopathy is a new disease appears after 1997. It is characterized by scleroderma-like skin fibrosis and may systemically involve other organs, including heart, lung, liver, and striated muscle. The disease might be triggered by the exposure to Gadolinium (Gd)-based magnetic resonance image contrast agents, and develops exclusively in patients with various stage of renal failure. Gadodiamide (Omniscan(supercrispt ®)) is among them, most frequently reported in the cases of NSF. The gold standard of diagnosis is deep skin biopsy which showed CD 34/procollagen dual positive in spindle cells, and absence of inflammation. There are several differential diagnoses to be made, such as cellulitis, drug reaction, scleroderma, scleromyxedema, and systemic sclerosis. To date, the disease mechanism is still unclear and there is no proven treatment for NSF. Therefore prevention of NSF should be emphasized. Food and Drug Administration recommends avoidance of Gd-containing contrast agents in patients with acute and chronic renal failure (glomerular filtration rate<30ml/min/1.73m^2), unless the diagnostic information is essential and cannot be replaced by other image studies. The dose of Gd should not exceed the recommendation and there must be sufficient time for Gd elimination before readministration. Prompt hemodialysis (HD) should be considered in patients receiving dialysis after Gd exposure as Gd can be effectively eliminated via HD.

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