Division of Cardiology, Department of Medicine, Helsinki University Central Hospital, Helsinki, Finland.
Division of Cardiology, Department of Medicine, Helsinki University Central Hospital, Helsinki, Finland.
Chest. 2012 Jul;142(1):225-227. doi: 10.1378/chest.11-1857.
We describe the case of a 40-year-old female patient who developed severe pulmonary hypertension and life-threatening right-sided heart failure in association with dietary scurvy and iron deficiency. Supplementation with oral vitamin C and iron very likely contributed to her complete cure. Scurvy-associated pulmonary arterial hypertension could result from impaired availability of endothelial nitric oxide, but inappropriate activation of the hypoxia-inducible family (HIF) of transcription factors could play an even more important role. HIF coordinates the body's responses to hypoxia, and its activity is regulated by oxygen-dependent prolyl hydroxylases, which need vitamin C and iron as cofactors. Deficiency of these cofactors could lead to uncontrolled HIF activity and pulmonary vasoconstriction responsive to vitamin C and iron administration.
我们描述了一例 40 岁女性患者的病例，该患者因饮食性坏血病和缺铁而并发严重肺动脉高压和威胁生命的右侧心力衰竭。口服维生素 C 和铁的补充极有可能促成了她的完全康复。坏血病相关的肺动脉高压可能是由于内皮一氧化氮的可用性受损所致，但缺氧诱导因子（HIF）转录因子家族的不适当激活可能发挥更为重要的作用。HIF 协调身体对缺氧的反应，其活性受氧依赖性脯氨酰羟化酶调节，而这些羟化酶需要维生素 C 和铁作为辅助因子。这些辅助因子的缺乏可能导致 HIF 活性不受控制，并对维生素 C 和铁的给药产生肺血管收缩反应。
