Clinical and laboratory findings associated with sleep disordered breathing in sickle cell disease.
Clinical and laboratory findings associated with sleep disordered breathing in sickle cell disease.
复制标题
与镰状细胞病睡眠呼吸障碍相关的临床和实验室结果。
DOI：
10.1002/ajh.24892
复制
发表时间：
2017
影响因子：
12.8
通讯作者：
Klings,ElizabethS
中科院分区：
文献类型：
--
作者：
Worsham,ChristopherM;Martin,StephonT;Nouraie,Syed-Mehdi;Cohen,RobynT;Klings,ElizabethS
镰状细胞病是美国最常见的限制生命的遗传性疾病，根据基因型，中位生存期为第5或第6个十年。虽然急性和慢性肺部并发症是发病和死亡的最常见原因之一，但我们对这些疾病的了解仍然有限。睡眠呼吸障碍（SDB）是一种呼吸异常，其真正的患病率和对整体SCD发病机制的影响尚未完全了解。SDB是一个概括性术语，描述了睡眠期间多种类型的异常呼吸;在本文中，SDB指的是阻塞性睡眠呼吸暂停（OSA）和无阻塞情况下的夜间低氧血症（NH）。阻塞性睡眠呼吸暂停（OSA）的定义是睡眠期间反复发作的部分或完全呼吸停止，并伴有氧饱和度下降。在一般人群中，OSA和NH与SCD直接相关的病理学（睡眠质量差、肺动脉和全身性高血压、内皮功能障碍和卒中）相关;然而，SDB与SCD严重程度和进展之间的相关性尚未得到充分研究。1本研究的目的是确定与多导睡眠图诊断的SDB相关的临床和实验室特征。我们对2012年至2016年期间在波士顿医疗中心（BMC）接受过夜多导睡眠图（PSG）作为门诊治疗一部分的SCD患者进行了回顾性图表审查。阻塞性睡眠呼吸暂停（OSA）在该分析中定义为呼吸暂停低通气指数（AHI）5.0次/小时。夜间低氧血症的定义为总睡眠时间（TST）的5%，氧饱和度低于90%。通过审查电子病历获得临床和实验室数据。所有PSG均在患者临床稳定（定义为血管闭塞事件（疼痛或ACS）或输血后至少4周）时进行。使用Stata 14.0（StataCorp.，学院站，TX）。这项研究得到了波士顿大学机构审查委员会的批准。我们的队列包括45名接受PSG的非裔美国人（22名成人和23名儿童）。78%的受试者具有HbSS或HbSb 0基因型（96%的儿童和61%的成人）;其余受试者具有HbSC基因型。虽然51%的患者因打鼾史而接受睡眠研究，但其他适应症包括：夜间遗尿症（43%的儿童）、白天嗜睡、休息或运动时氧饱和度下降，或扁桃体切除术/腺样体切除术后随访。患有OSA的人比没有OSA的人年龄大。OSA与年龄的相关性是由性别差异驱动的，因为男性15岁后OSA的频率稳定增加，而女性OSA的频率没有随着年龄的增加而显著变化（图1）。BMI和OSA的发生率之间没有关联（p5 0.30），尽管肥胖在我们的队列中很少见。在校正年龄和性别的多变量logistic回归模型中，PSG前ACS事件的发生率较高与OSA发生几率增加无关（OR 2. 09，95% CI 0. 39 - 11. 14，P 5. 05）。OSA与未来血管闭塞（疼痛或ACS）发作的可能性增加也不相关。如表1所示，与无夜间低氧血症的个体相比，夜间低氧血症的个体具有较低的日间氧饱和度、较高的网织红细胞百分比、较高的白色血细胞计数和较高的血清天冬氨酸转氨酶（AST）浓度。哮喘患者更有可能...
Sickle cell disease is the most common life-limiting genetic disease in the United States, with median survival in the 5th or 6th decade depending on genotype. While acute and chronic pulmonary complications are among the most common causes of morbidity and mortality, our understanding of each of these conditions remains limited. Sleepdisordered breathing (SDB) is a respiratory abnormality whose true prevalence and impact on overall SCD pathogenesis are not fully understood. SDB is an umbrella term describing many types of abnormal breathing during sleep; for this manuscript, SDB refers to obstructive sleep apnea (OSA) and nocturnal hypoxemia in the absence of obstruction (NH). OSA is defined as repeated episodes of partial or complete cessation of breathing during sleep associated with a decrease in oxygen desaturation. In the general population OSA and NH are associated with pathology (poor sleep quality, pulmonary and systemic hypertension, endothelial dysfunction, and stroke) that have direct relevance to SCD; however, associations between SDB and SCD severity and progression have been inadequately studied. 1 The objective of this study was to identify clinical and laboratory characteristics associated with polysomnogram-diagnosed SDB. We performed a retrospective chart review of patients with SCD who had overnight polysomnography (PSG) as part of their outpatient care at Boston Medical Center (BMC) between 2012 and 2016. Obstructive sleep apnea (OSA) was defined in this analysis as an apneahypopnea index (AHI) 5.0 events per hour. Nocturnal hypoxemia was defined as having 5% of total sleep time (TST) with an oxygen saturation below 90%. Clinical and laboratory data were obtained from review of the electronic medical record. All PSGs were performed while patient was clinically stable as defined as at least 4 weeks after a vaso-occlusive event (pain or ACS) or a blood transfusion. Analyses were conducted using Stata 14.0 (StataCorp., College Station, TX). This study was approved by the Boston University Institutional Review Board. Our cohort included 45 African-American individuals (22 adults and 23 children) with SCD who underwent PSG. Seventy eight percent had the HbSS or HbSb0 genotype (96% of children and 61% of adults); remaining subjects had the HbSC genotype. While 51% of patients were referred for sleep studies because of a history of snoring, additional indications included: nocturnal enuresis (43% of children), daytime somnolence, oxygen desaturation at rest or during exertion, or as follow-up after tonsillectomy/adenoidectomy. Individuals with OSA were older compared to those without OSA. The association of OSA with age was driven by gender differences as there was a steady increase in the frequency of OSA after age 15 in males while the frequency of OSA in females did not change significantly change as age increased (Figure 1). There was no association between BMI and frequency of OSA (p5 0.30), though obesity was rare in our cohort. In a multivariable logistic regression model adjusted for age and gender, a higher rate of ACS events prior to the PSG was not associated with increased odds of having OSA (OR 2.09, 95% CI 0.39–11.14, P 5. 39), nor was OSA associated with increased likelihood of a future vaso-occlusive (pain or ACS) episodes. As shown in Table 1, individuals with nocturnal hypoxemia had a lower daytime oxygen saturation, higher reticulocyte percentage, higher white blood cell counts, and higher serum aspartate aminotransferase (AST) concentrations compared to those without nocturnal hypoxemia. Individuals with asthma were more likely to …