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KMID : 0388220080150030277
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Journal of the Korean Rheumatism Association
2008 Volume.15 No. 3 p.277 ~ p.281
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A Case of Systemic Scleroderma in Triple X Syndrome
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Lee Sang-Yeob
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Abstract
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Systemic scleroderma is a collagen-vascular disease of unknown etiology. Although the pathogenesis is poorly understood, disease progression involves the vasculature, the immune system and extracellular matrix deposition. systemic scleroderma occurs 3∼8 times more frequently in women than men and pathogenesis of systemic scleroderma may be related to female X chromosome. but the role of X chromosome in autoimmunity has not been illustrated yet. Most recently reports, the disturbances in X chromosome and inactivation of X chromosome may be the cause of autoimmunity in abnormal sex chromosome syndrome. Also autoimmune diseases such as systemic scleroderma is increased in Turner’s syndrome. the author had experienced a woman with systemic scleroderma who had been diagnosed to triple X syndrome due to infertility in the past. which was very rare case and not reported yet. So the author report a case of systemic scleroderma with triple X syndrome with literature review.
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KEYWORD
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Systemic scleroderma, Triple X syndrome
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