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- W2071547297 abstract "症例は53歳女性. 40歳時甲状腺機能低下症(橋本病)治療開始. 42歳時レイノー症状出現, 50歳頃より労作時呼吸困難・口腔内乾燥感を自覚.平成11年1月失神発作.同年5月呼吸困難の増悪と肝障害のため入院.心エコーにて右心系の拡大肥厚と僧帽弁の逆流を認め,肺動脈圧平均120mmHgと推定された.橋本病,門脈圧充進症を伴わない原発性胆汁性肝硬変(PBC)および原発性シェーグレン症候群(SjS)に合併した肺高血圧症(PH)と診断.PHに対して酸素投与,抗凝固療法(ワーファリン),経口PGI2製剤(プロスタサイクリン)投与したが,右心不全,黄疸が進行し,同年12月緊急入院後,突然心停止し死亡.病理解剖で右心肥大と末梢肺動脈の内膜の中心性肥厚,中膜の線維化, plexiform lesionを認めたが,肺血管壁には免疫グロブリンの沈着は見られなかった. PH合併膠原病症例は予後不良であることが多く,治療法の開発のために病因をさらに解析していく必要があると考えた." @default.
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- W2071547297 date "2000-01-01" @default.
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- W2071547297 title "Pulmonary hypertension in a patient with primary Sjogren's syndrome, Hashimoto's disease, and primary biliary cirrhosis." @default.
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