关键词: Malnutrition Medical management Pulmonary hypertension Respiratory medicine Vitamins and supplements

Mesh : Humans Pulmonary Arterial Hypertension / complications Scurvy / complications Familial Primary Pulmonary Hypertension / complications Hypertension, Pulmonary / drug therapy Ascorbic Acid Deficiency / complications

来  源:   DOI:10.1136/bcr-2023-254730   PDF(Pubmed)

Abstract:
Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that results in precapillary pulmonary hypertension. PAH is caused by a group of clinical conditions involving multiple organ systems. Several cases have been reported in the literature demonstrating an association between vitamin C deficiency and PAH. Low endothelial nitric oxide levels in the pulmonary vasculature, combined with the inappropriate activation of hypoxia-inducible transcription factors, seen in patients with ascorbic acid deficiency, are believed to be the main contributors to the pathogenesis of pulmonary vasculopathy and the exaggerated pulmonary vasoconstrictive response seen in patients with scurvy-induced PAH. Vitamin C supplementation is considered the definitive treatment.
摘要:
肺动脉高压(PAH)是导致毛细血管前肺动脉高压的肺血管系统的进行性疾病。PAH是由一组涉及多个器官系统的临床病症引起的。文献中已经报道了一些病例,表明维生素C缺乏与PAH之间存在关联。肺血管内皮一氧化氮水平低,结合缺氧诱导转录因子的不适当激活,可见于抗坏血酸缺乏的患者,被认为是引起肺血管病变的发病机理的主要原因,并且在患有镰刀性PAH的患者中看到了肺血管收缩反应。补充维生素C被认为是决定性的治疗方法。
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