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KMID : 0357820000240010092
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Korean Journal of Legal Medicine
2000 Volume.24 No. 1 p.92 ~ p.97
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A Sudden Death in a Patient with Neurofibromatosis
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Lee Bong-Woo
Seo Joong-Seok
Kwon Tae-Jung
Lee Won-Tae
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Abstract
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Neurofibromatosis type1(von Recklinghausen’s disease) is characterized by multiple skin tumors and abnormal cutaneous pigmentation(cafe au lait spots). Unlikely the solitary neurofibroma, those encountered in von Recklinghausen’s disease may cause significant morbidity. The vasculopathy, although poorly recognized manifestation of von Recklinghausen’s neurofibromatosis is a major cause of fatal cases. One of its most dramatic presentation is the spontaneous hemothorax. We report a case of Von Recklinghausen’s disease with multiple neurofibromatosis accompanying intrathoracic hemorrhage due to vascular rupture, which is the first legal autopsy case in korea.
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KEYWORD
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Neurofibromatosis type1, hemothorax
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