长期管理和治疗急性间歇性病与反复发作,使用药物预防药物治疗
Hung-Chou Kuo1, Long-Sun Ro1, Chia-Ni Lin2,3
1Department of Neurology, Chang Gung Memorial Hospital Linkou Medical Centre and College of Medicine, Chang Gung University, Taoyuan, Taiwan.
Hepatology communications
|December 5, 2023
概括
对于频繁发作的急性间歇性病 (AIP) 患者,在监测1-2年后可以考虑预防性治疗. 需要进一步的研究,以建立关于AIP预防策略的共识.
科学领域:
- 医学科学 医学科学 医学科学
- 罕见疾病 罕见疾病
- 药理学 药理学是指药理学的学科.
背景情况:
- 急性间歇性病 (AIP) 管理缺乏针对反复发作的最终指导方针.
- 患有频繁AIP发作 (年均发作率≥4) 的患者需要特定的治疗策略.
研究的目的:
- 总结针对不同发作频率的AIP患者的管理策略.
- 为了评估预防性治疗的有效性,如血红酸盐和givosiran.
主要方法:
- 在治疗前和治疗后对29名AIP患者的发作率 (AAR) 的回顾性分析.
- 频繁发作 (≥4 AAR) 与较少发作 (<4 AAR) 的患者的结局的比较.
- 对包括血红酸盐和givosiran在内的预防性治疗的评估.
主要成果:
- 所有患者在治疗期间都显示出攻击频率降低.
- 79.3%的患者在最近两年中没有发作.
- 没有预防的患者在最近2年中没有发作的比率更高 (90.0%),相比于接受预防的患者 (55.6%).
结论:
- 在经过1-2年的观察后,可能会考虑对AIP患者进行预防,AIP患者每年≥4次发作.
- 密切监测攻击的严重程度和频率至关重要,以确定预防的必要性.
- 需要进一步的研究来建立关于AIP药理学预防的共识.
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