Youssry Ilham, Mostafa Abla S, Hamed Dina H, Hafez Yasmin F Abdel, Bishai Irene E, Selim Yasmeen M M
Department of Pediatric Hematology, Faculty ofMedicine, Cairo University, Giza, Egypt.
Department of Pediatric Pulmonology, Faculty of Medicine, Cairo University, Giza, Egypt.
BMC Pediatr. 2024 Oct 1;24(1):626. doi: 10.1186/s12887-024-05066-6.
Endothelial dysfunction is an integral pathophysiologic mechanism in sickle cell disease (SCD), and can lead to many complications. Sleep-disordered breathing (SDB) is a SCD complication with diverse incidence and pathophysiology. This study aimed to determine the prevalence of SDB in children with SCD and to assess its relation to endothelial dysfunction.
Sixty children with SCD and 60 healthy controls were enrolled. The levels of TNF-α, IL-6, and IL-17A were evaluated in the entire cohort using enzyme-linked immunosorbent assay (ELISA) kits. Polysomnography (PSG) was performed for all SCD patients after completion of the Pediatric Sleep Questionnaire (PSQ).
TNF-α, IL-6, and IL-17A levels were significantly greater in children with SCD than in controls (p-values < 0.001, < 0.001, and 0.006, respectively). The PSQ revealed symptoms suggestive of SDB in 50 children with SCD (83.3%), and PSG revealed obstructive sleep apnea (OSA) in 44 children with SCD (73.3%); 22 patients had mild OSA, and 22 had moderate-to-severe OSA according to the apnea-hypopnea index (AHI). TNF-α was significantly greater in SCD children who reported heavy or loud breathing, trouble breathing or struggle to breathe, and difficulty waking up in the morning (p-values = 0.002, 0.002, and 0.031, respectively). The IL-6 levels were significantly greater in SCD children who stopped growing normally (p-value = 0.002). The levels of IL-6 and IL-17A were significantly greater in SCD children with morning headaches (p-values = 0.007 and 0.004, respectively).
Children with SCD showed a high prevalence of SDB with significantly elevated levels of markers of endothelial function, highlighting the interplay of SDB and endothelial dysfunction in SCD.
内皮功能障碍是镰状细胞病（SCD）的一个重要病理生理机制，可导致多种并发症。睡眠呼吸紊乱（SDB）是一种SCD并发症，其发病率和病理生理机制各不相同。本研究旨在确定SCD患儿中SDB的患病率，并评估其与内皮功能障碍的关系。
招募了60名SCD患儿和60名健康对照。使用酶联免疫吸附测定（ELISA）试剂盒在整个队列中评估肿瘤坏死因子-α（TNF-α）、白细胞介素-6（IL-6）和白细胞介素-17A（IL-17A）的水平。在完成儿童睡眠问卷（PSQ）后，对所有SCD患者进行多导睡眠图（PSG）检查。
SCD患儿的TNF-α、IL-6和IL-17A水平显著高于对照组（p值分别<0.001、<0.001和0.006）。PSQ显示50名SCD患儿（83.3%）有提示SDB的症状，PSG显示44名SCD患儿（73.3%）有阻塞性睡眠呼吸暂停（OSA）；根据呼吸暂停低通气指数（AHI），22例患者为轻度OSA，22例为中度至重度OSA。报告有呼吸沉重或声音大、呼吸困难或呼吸费力以及早晨醒来困难的SCD患儿的TNF-α水平显著更高（p值分别为0.002、0.002和0.031）。正常生长停止的SCD患儿的IL-6水平显著更高（p值=0.002）。有早晨头痛的SCD患儿的IL-6和IL-17A水平显著更高（p值分别为0.007和0.004）。
SCD患儿中SDB的患病率很高，内皮功能标志物水平显著升高，突出了SDB与SCD中内皮功能障碍之间的相互作用。
