Ehlers-Danlos Syndrome with Recurrent Spontaneous Pneumothoraces and Cavitary Lesion on Chest X-ray as the Initial Complications
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概要
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A 17-year-old-man developed left-sided pneumothorax in 1995. Chest computed tomography (CT) showed a thick-walled cavity in the left lower lobe. Video-assisted thoracic surgery was performed, and pathologic findings of the resected lung showed a cavity, organizing hematoma, and a fibrous nodule. Fragility of connective tissue was suspected, and biochemical and molecular analysis showed reduction of type III collagen production and point mutation of the COL3A1 gene. The patient was diagnosed as having vascular-type Ehlers-Danlos syndrome (EDS). From 2002, the patient developed hemoptysis and bloody sputum once a year. Chest CT detected several nodules and cavities, which were regarded as hematomas with or without excretion. Several vascular changes including aneurysmal formations have been found since 2002, and an aneurysm of the left ulnar artery was resected. The patient continues to be followed regularly on an outpatient basis. We report a rare case of vascular-type EDS who developed pulmonary symptoms as an initial complication.
著者
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Takayanagi Noboru
Department Of Respiratory Medicine Saitama Cardiovascular And Respiratory Center
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Aoki Fumiaki
Department Of Medicine And Biological Science Gunma University Graduate School Of Medicine
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Saito Hiroo
Department Of Mathematical Informatics Graduate School Of Information Science And Technology Univers
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Ubukata Mikio
Department Of Respiratory Medicine Saitama Cardiovascular And Respiratory Center
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Tokunaga Daido
Department Of Respiratory Medicine Saitama Cardiovascular And Respiratory Center
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Yanagisawa Tsutomu
Department Of Respiratory Medicine Saitama Cardiovascular And Respiratory Center
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Kurashima Kazuyoshi
Department Of Respiratory Medicine Saitama Cardiovascular And Respiratory Center
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Yoshii Yutaka
Department Of Agricultural Chemistry Kyushu University
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Okita Hiroshi
Department Of Dermatology Dokkyo University School Of Medicine
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Hatamochi Atsushi
Department Of Dermatology Chiba University School Of Medicine
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Kawabata Yoshinori
Division Of Pathology The Research Institute Of Tuberculosis
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Sugita Yutaka
Department Of Respiratory Medicine Saitama Cardiovascular And Respiratory Center
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Ishiguro Takashi
Department Of 2nd Physiology Showa University School Of Medicine
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Yamaguchi Shozaburo
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Japan
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Kagiyama Naho
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center
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Matsushima Hidekazu
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center
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Harasawa Keiji
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center
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Yoneda Koichiro
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center
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Miyahara Yousuke
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center
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Yamaguchi Shozaburo
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center
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Yoshii Yutaka
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center
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Okita Hiroshi
Department of Dermatology, Dokkyo Medical University
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