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镰状细胞病慢性输血成年患者的夜间血红蛋白饱和度下降:一项回顾性研究

Nocturnal hemoglobin desaturation in chronically transfused adults with sickle cell disease: a retrospective study.

作者信息

Obadina Mofiyin A, Owens Iman, Chang Ada, Miller Vanessa, Little Jane A

机构信息

Division of Hematology, Department of Medicine, University of North Carolina, Chapel Hill, NC 27514, United States.

UNC Blood Research Center, University of North Carolina, Chapel Hill, NC 27514, United States.

出版信息

J Sick Cell Dis. 2025 Jan 31;2(1):yoaf003. doi: 10.1093/jscdis/yoaf003. eCollection 2025.

DOI:10.1093/jscdis/yoaf003
PMID:40045981
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11878521/
Abstract

High-risk SCD may be managed with chronic red blood cell exchange transfusions. We examined the prevalence of sleep-associated hypoxemia (≥5 minutes at SpO ≤88%) or nocturnal Hb desaturation (NHD) in chronically transfused adults with SCD. Of 41 identified participants, 15 (36.6%) had tested positive for NHD at some point prior to enrollment. The median duration of desaturation (SpO ≤88%) in those that tested positive was 88.3 minutes (IQR 27.9-226.0 minutes). Participants with and without NHD were compared using non-parametric statistical tests. Compared to participants without NHD, those with NHD had higher absolute reticulocyte counts ( = .018) and white blood counts ( = .05) and tended to be older. They had more anemia ( = .11) and higher lactate dehydrogenase levels ( = .072). Older age at initiation of chronic red blood cell transfusions associated the strongest with a longer duration of NHD (ρ = 0.4253,  = .0067), while prior history of cerebrovascular events associated with a shorter duration of NHD ( = .0315). Our results demonstrate that NHD is common in adults being treated with red blood cell exchange for high-risk SCD and associates with laboratory evidence of increased disease activity. Increased awareness of this complication and appropriate screening may provide an additional simple, low-cost, and physiologically relevant treatment intervention, that is oxygen therapy.

摘要

高危镰状细胞病(SCD)可通过慢性红细胞置换输血进行治疗。我们研究了接受慢性输血的成年SCD患者中睡眠相关低氧血症(SpO₂≤88%持续≥5分钟)或夜间血红蛋白饱和度降低(NHD)的患病率。在41名确定的参与者中,15名(36.6%)在入组前的某个时间点NHD检测呈阳性。检测呈阳性者的饱和度降低(SpO₂≤88%)的中位持续时间为88.3分钟(四分位间距27.9 - 226.0分钟)。使用非参数统计检验对有和没有NHD的参与者进行比较。与没有NHD的参与者相比,有NHD的参与者网织红细胞绝对计数更高(P = 0.018)、白细胞计数更高(P = 0.05),且年龄往往更大。他们贫血更严重(P = 0.11),乳酸脱氢酶水平更高(P = 0.072)。开始慢性红细胞输血时年龄较大与NHD持续时间较长关联最强(ρ = 0.4253,P = 0.0067),而脑血管事件既往史与NHD持续时间较短相关(P = 0.0315)。我们的结果表明,NHD在接受红细胞置换治疗高危SCD的成年人中很常见,并且与疾病活动增加的实验室证据相关。提高对这种并发症的认识并进行适当筛查可能会提供一种额外的简单、低成本且生理相关的治疗干预措施,即氧疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0750/11878521/b04384a0394a/yoaf003f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0750/11878521/279ff60d9713/yoaf003f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0750/11878521/23e6b8a35c8d/yoaf003f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0750/11878521/b04384a0394a/yoaf003f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0750/11878521/279ff60d9713/yoaf003f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0750/11878521/23e6b8a35c8d/yoaf003f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0750/11878521/b04384a0394a/yoaf003f3.jpg

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本文引用的文献

1
Nocturnal hypoxaemia is common in adults with sickle cell anaemia.镰状细胞贫血症患者在夜间常发生低血氧症。
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Nocturnal Hypoxemia Rather Than Obstructive Sleep Apnea Is Associated With Decreased Red Blood Cell Deformability and Enhanced Hemolysis in Patients With Sickle Cell Disease.镰状细胞病患者中,夜间低氧血症而非阻塞性睡眠呼吸暂停与红细胞变形性降低及溶血增强有关。
Front Physiol. 2021 Sep 24;12:743399. doi: 10.3389/fphys.2021.743399. eCollection 2021.
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The burden of sleep disordered breathing in children with sickle cell disease.
儿童镰状细胞病睡眠呼吸障碍负担。
Pediatr Pulmonol. 2021 Dec;56(12):3607-3633. doi: 10.1002/ppul.25632. Epub 2021 Aug 25.
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Red blood cell adhesion to ICAM-1 is mediated by fibrinogen and is associated with right-to-left shunts in sickle cell disease.红细胞与细胞间黏附分子-1的黏附由纤维蛋白原介导,并与镰状细胞病中的右向左分流有关。
Blood Adv. 2020 Aug 11;4(15):3688-3698. doi: 10.1182/bloodadvances.2020001656.
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Whole blood viscosity and red blood cell adhesion: Potential biomarkers for targeted and curative therapies in sickle cell disease.全血黏度和红细胞黏附:镰状细胞病靶向和治愈疗法的潜在生物标志物。
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Blood Adv. 2020 Jan 28;4(2):327-355. doi: 10.1182/bloodadvances.2019001143.
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Hypoxia in sickle cell disease due to right to left shunting.镰状细胞病中因右向左分流导致的缺氧。
Am J Hematol. 2019 Feb;94(2):E53-E55. doi: 10.1002/ajh.25360. Epub 2018 Dec 5.
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Simultaneous polymerization and adhesion under hypoxia in sickle cell disease.镰状细胞病中缺氧下的聚合和黏附的同步化。
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The association of nocturnal hypoxia and an echocardiographic measure of pulmonary hypertension in children with sickle cell disease.夜间缺氧与超声心动图评估镰状细胞病患儿肺动脉高压的相关性。
Pediatr Res. 2019 Mar;85(4):506-510. doi: 10.1038/s41390-018-0125-6. Epub 2018 Jul 24.
10
Intracardiac or intrapulmonary shunts were present in at least 35% of adults with homozygous sickle cell disease followed in an outpatient clinic.在一家门诊诊所随访的纯合子镰状细胞病成年患者中,至少35%存在心内或肺内分流。
Haematologica. 2019 Jan;104(1):e1-e3. doi: 10.3324/haematol.2018.196212. Epub 2018 Jul 13.