在衰老的囊性纤维化患者群体中,未来的共同疾病
Javier Humberto Ticona1, Nicole Lapinel2, Janice Wang2
1Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY 11549, USA.
Life (Basel, Switzerland)
|June 28, 2023
概括
患有囊性纤维化 (CF) 的人因调节器疗法而活得更长,但现在面临与年龄相关的并发症. 本综述考察了这些新的健康挑战的风险因素和流行病学对于老化CF人口.
科学领域:
- 医学科学 医学科学 医学科学
- 遗传学 遗传学 是一个
- 肺部病理学 肺部病理学
背景情况:
- 囊性纤维化 (CF) 是一种由囊性纤维化跨膜导电性调节器 (CFTR) 基因突变引起的自体逆向遗传疾病.
- 高效的调节器疗法显著增加了CF (PwCF) 患者的寿命,导致人口老龄化.
- 延长寿命的增加带来了新的挑战,因为PwCF现在面临着通常在老龄化人口中出现的并发症.
研究的目的:
- 提供PwCF老龄化人口中并发病的风险因素和流行病学概述.
- 突出CFTR基因功能障碍超出呼吸道并发症的多系统影响.
- 强调在PwCF中需要主动管理和预防战略,以确保长期健康.
主要方法:
- 文献审查和流行病学数据分析.
- 专注于风险因素和特定伴随性疾病的流行率.
- 综合有关CF相关并发症的当前知识在一个老龄化的队列.
主要成果:
- 在PwCF中,心血管疾病,脂质失调,CF相关糖尿病,肺高血压,阻塞性睡眠呼吸暂停,CF肝病,骨健康问题和恶性瘤的风险增加.
- CFTR功能障碍的多系统性质有助于急性和慢性非呼吸道并发症.
- 有效的CFTR调节器疗法需要将临床重点转向管理与年龄相关的疾病.
结论:
- 老龄化的PwCF人口需要全面的护理计划,解决更广泛的健康问题.
- 提高对初级和二级预防的认识和关注对于改善长期发病率和死亡率至关重要.
- 在PwCF中管理并发症对于优化生活质量和在调节器治疗时代的生存至关重要.
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