Chida-Nagai Ayako, Tsujioka Takao, Sasaki Daisuke, Izumi Gaku, Yamazawa Hirokuni, Takeda Atsuhito
Department of Pediatrics, Hokkaido University Hospital, Sapporo, Japan.
Front Pediatr. 2022 Jun 17;10:909595. doi: 10.3389/fped.2022.909595. eCollection 2022.
Idiopathic pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting the pulmonary arteries. Epoprostenol, a synthetic prostaglandin analog, is the most potent pharmacological treatment modality used in patients with PAH. However, it requires continuous intravenous infusion, which negatively impacts the patient's quality of life and frequently results in complications, such as catheter-related bloodstream infection. We weaned an adolescent female patient off epoprostenol by gradually introducing oral selexipag over a sustained period, following many years of continuous intravenous epoprostenol use alone. Oral selexipag might have an efficacy comparable to epoprostenol in young patients with PAH.
特发性肺动脉高压(PAH)是一种罕见的、进行性疾病,影响肺动脉。依前列醇,一种合成的前列腺素类似物,是用于PAH患者的最有效的药物治疗方式。然而,它需要持续静脉输注,这对患者的生活质量产生负面影响,并经常导致并发症,如导管相关血流感染。在多年单独持续静脉使用依前列醇后,我们通过在一段持续时间内逐渐引入口服司来帕格,使一名青春期女性患者停用依前列醇。口服司来帕格在年轻PAH患者中的疗效可能与依前列醇相当。