کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3209065 1587601 2009 7 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Nephrogenic systemic fibrosis–a rapidly progressive disabling disease with limited therapeutic options
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی امراض پوستی
پیش نمایش صفحه اول مقاله
Nephrogenic systemic fibrosis–a rapidly progressive disabling disease with limited therapeutic options
چکیده انگلیسی

BackgroundNephrogenic systemic fibrosis (NSF) affects some patients on dialysis after gadolinium contrast agent–enhanced magnetic resonance imaging. It is characterized by progressive skin fibrosis of the extremities, sometimes including the trunk and internal organs.MethodsThe clinical course of 10 patients with biopsy-proven NSF was analyzed retrospectively with regard to gadolinium exposition, disease onset, and progression of NSF with special emphasis on physical mobility and impact of different therapeutic approaches.ResultsDespite physiotherapy and different additional therapeutic approaches (eg, immunosuppression, ultraviolet A-1 phototherapy, or extracorporal photopheresis) all patients developed progressive skin fibrosis of the lower extremities, sometimes including the trunk and arms. Kidney transplantation led to a slow improvement of skin lesions in one patient. Nine patients developed progressive joint contractures, and 8 patients became wheelchair bound within 12 months after disease onset and became dependent on the support of family members or a nursing service.LimitationsRetrospective analysis in a relatively small number of patients is a limitation.ConclusionNSF appears to be a rapidly progressive disabling disease with limited therapeutic options.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of the American Academy of Dermatology - Volume 61, Issue 5, November 2009, Pages 868–874
نویسندگان
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