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근병증을 동반한 콩팥성전신섬유화증

Nephrogenic Systemic Fibrosis with Myopathy

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초록

Nephrogenic systemic fibrosis (NSF) is a systemic disease that affects the skin and other tissues in patients with renal insufficiency and exposure to gadolinium-containing contrast. A 55-year-old woman with end-stage renal disease on hemodialysis was consulted for progressive general weakness. After she had undergone multiple MRIs with gadolinium-containing contrast media, muscle weakness and skin lesions were developed. Her skin and muscle biopsy specimens showed CD34+ fibroblast entrapping collagen bundles. There are few reports of NSF with myopathy.

Abstract

Nephrogenic systemic fibrosis (NSF) is a systemic disease that affects the skin and other tissues in patients with renal insufficiency and exposure to gadolinium-containing contrast. A 55-year-old woman with end-stage renal disease on hemodialysis was consulted for progressive general weakness. After she had undergone multiple MRIs with gadolinium-containing contrast media, muscle weakness and skin lesions were developed. Her skin and muscle biopsy specimens showed CD34+ fibroblast entrapping collagen bundles. There are few reports of NSF with myopathy.

참고문헌 (10)

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  4. Perazella MA, "Gadolinium use in patients with kidney disease: a cause for concern", Semin Dial, 20, pp.179~185 (2007)
  5. Bardin T, "Nephrogenic systemic fibrosis", Curr Opin Rheumatol, 22, pp.54~58 (2010)
  6. Schieren G, "Nephrogenic systemic fibrosis-a rapidly progressive disabling disease with limited therapeutic options", J Am Acad Dermatol, 61, pp.868~874 (2009)
  7. Jalandhara N, "Nephrogenic Systemic Fibrosis and Gadolinium-Containing Radiological Contrast Agents: An Update", Clin Pharmacol Ther, 6, pp.6~ (2011)
  8. Keyrouz S, "Neuromuscular involvement in nephrogenic systemic fibrosis", J Clin Neuromuscul Dis, 9, pp.297~302 (2007)
  9. Brouns R, "Neurological complications in renal failure: a review", Clin Neurol Neurosurg, 107, pp.1~16 (2004)
  10. Edgar E, "Nephrogenic systemic fibrosis presenting as myopathy: a case report with histopathologic correlation", Neuromuscul Disord, 20, pp.411~413 (2010)

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