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羟基脲对镰状细胞病和阻塞性睡眠呼吸暂停患儿的治疗效果。

Hydroxyurea treatment effect on children with sickle cell disease and obstructive sleep apnea.

作者信息

Grady Anthony J, Hankins Jane S, Haberman Brent, Schoumacher Robert, Stocks Rose Mary

机构信息

Department of Otolaryngology, Head and Neck Surgery, University of Tennessee Health Science Center, 910 Madison Avenue, Suite 420, Memphis, TN, 38163, USA.

Department of Hematology, St. Jude Children's Research Hospital, 262 Danny Thomas place, Memphis, TN, 38105, USA.

出版信息

Sleep Breath. 2017 Sep;21(3):697-701. doi: 10.1007/s11325-017-1458-9. Epub 2017 Jan 11.

DOI:10.1007/s11325-017-1458-9
PMID:28078488
Abstract

BACKGROUND

While hydroxyurea is the mainstay of treatment for many of the comorbidities associated with sickle cell disease, its effect on obstructive sleep apnea has not been fully investigated. The purpose of this project is to help characterize the effects of hydroxyurea on obstructive sleep apnea in children with sickle cell disease and determine its therapeutic role in the condition.

METHODS

Chart review was conducted on two pediatric patients with sickle cell disease who experienced resolution of obstructive sleep apnea following hydroxyurea administration.

RESULTS

After undergoing approximately 11 months of hydroxyurea therapy, sleep apnea symptoms improved and obstructive sleep apnea resolution was confirmed by repeat polysomnography in both cases. This resolution was largely secondary to a reduction in the obstructive component of the apnea hypopnea index, highlighting a previously unreported association.

CONCLUSIONS

As adenotonsillectomy is associated with significant risks in patients with sickle cell disease, it appears reasonable to consider a period of observation for improvement of obstructive sleep apnea following hydroxyurea administration rather than directly proceeding with surgery.

摘要

背景

虽然羟基脲是治疗镰状细胞病相关多种合并症的主要药物,但其对阻塞性睡眠呼吸暂停的影响尚未得到充分研究。本项目的目的是帮助明确羟基脲对镰状细胞病患儿阻塞性睡眠呼吸暂停的影响,并确定其在该病中的治疗作用。

方法

对两名镰状细胞病儿科患者进行病历回顾,这两名患者在服用羟基脲后阻塞性睡眠呼吸暂停症状得到缓解。

结果

在接受约11个月的羟基脲治疗后,两名患者的睡眠呼吸暂停症状均有改善,重复多导睡眠图检查证实阻塞性睡眠呼吸暂停得到缓解。这种缓解主要是由于呼吸暂停低通气指数的阻塞成分降低,这突出了一种此前未报道的关联。

结论

由于腺样体扁桃体切除术在镰状细胞病患者中存在重大风险,在服用羟基脲后观察一段时间以改善阻塞性睡眠呼吸暂停,而不是直接进行手术,似乎是合理的。

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Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology.中性粒细胞、血小板和炎症通路在镰状细胞病病理生理学的关联中
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Hydroxyurea treatment does not increase blood viscosity and improves red blood cell rheology in sickle cell anemia.羟基脲治疗不会增加镰状细胞贫血患者的血液粘度,反而会改善红细胞流变学。
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Higher nocturnal and awake oxygen saturations in children with sickle cell disease receiving hydroxyurea therapy.
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Prevention of Morbidity in Sickle Cell Disease (POMS2a)-overnight auto-adjusting continuous positive airway pressure compared with nocturnal oxygen therapy: a randomised crossover pilot study examining patient preference and safety in adults and children.镰状细胞病发病率预防研究(POMS2a)- 自动调节持续气道正压通气与夜间氧疗的对比:一项关于成人和儿童患者偏好及安全性的随机交叉先导研究
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