Nephrogenic systemic fibrosis: an emerging entity

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• 作者：Sandip K. Saxena ; Manjuri Sharma ; Mital Patel and Dimitrios Oreopoulos
• 关键词：NSF ; Nephrogenic systemic fibrosis ; NFD ; Nephrogenic fibrosing dermopathy ; Gadolinium ; related skin diseases ; Gadolinium toxicities ; Gadolinium in kidney diseases
• 刊名：International Urology and Nephrology
• 出版年：2008
• 出版时间：September, 2008
• 年：2008
• 卷：40
• 期：3
• 页码：715-724
• 全文大小：246.9 KB
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Nephrology
  Urology and Andrology
  
• 出版者：Springer Netherlands
• ISSN：1573-2584

文摘

Nephrogenic systemic fibrosis (NSF) is a progressive disorder which has been seen only in patients with chronic kidney disease. It is associated with fibrosis of the skin and connective tissue throughout the body. The skin thickens and becomes hard, rigid, and coarse, which severely restricts movement of the joints. It can also lead to widespread fibrosis of internal organs involving lungs, heart, diaphragm, esophagus, and skeletal muscle, and in some cases may lead to death. Based on case series reports and skin biopsy showing gadolinium (Gd) ions, NSF has been directly linked to Gd contrast exposure given during MRI/MRA. There are over 250 reported cases of NSF worldwide with hundreds still not reported or remaining undiagnosed. Symptoms of NSF appear within 2–75 days, with a mean of 25 days after exposure to Gd contrast. This disorder is not well understood, and more research is needed to obtain information about how Gd causes this condition. Currently there is no effective treatment, so prevention is the only way to avoid this serious illness.