Juvenile Pulmonary Hypertension Associated with Fibromuscular Dysplasia
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概要
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A 20-year-old female diagnosed as idiopathic pulmonary arterial hypertension at 7 years of age was referred with worsening dyspnea and chest pain. Several imaging studies and right cardiac catheterization showed multiple stenoses in the peripheral pulmonary arteries with severe pulmonary hypertension and multiple systemic arterial stenoses lacking in systemic hypertension. No evidence of inflammatory or autoimmune disease was detected. Fibromuscular dysplasia was clinically diagnosed because of the narrowed systemic and pulmonary arterial stenoses which included dilatation and aneurysms that appeared similar to a string of beads. Treatment with sildenafil yielded a temporary improvement in her disease state.
著者
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Sugiura Toshihiko
Department of Respirology, Graduate School of Medicine, Chiba University
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Kitazono Miyako
Department of Respirology, Graduate School of Medicine, Chiba University
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Yano Toshiaki
Department Of Cardiology Shizuoka City Hospital
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Yamauchi Keita
Department Of Clinical Pharmaceutical Science Graduate School Of Natural Science And Technology Okay
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Tanabe Nobuhiro
Department Of Chemistry Faculty Of Science Kyoto University
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Tatsumi Koichiro
Department Of Chest Medicine School Of Medicine Chiba University
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Takiguchi Yuichi
Department Of Chemotherapy Graduate School Of Medicine Chiba University
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Kasahara Yasunori
Department Of Respirology Graduate School Of Medicine Chiba University
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Sakao Seiichiro
Department of Respirology (B2), Graduate School of Medicine, Chiba University, Japan
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Yamauchi Keita
Department of Respirology, Graduate School of Medicine, Chiba University
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