Elalfy Mohsen S, Youssef Omneya Ibrahim, Deghedy Marwa M R, Abdel Naby Mariam M
Department of Paediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
J Pediatr Hematol Oncol. 2018 Apr;40(3):171-177. doi: 10.1097/MPH.0000000000001096.
Cardiovascular complications are well recognized in β-thalassemia and sickle cell disease (SCD). The objective of this study was to evaluate left ventricular (LV) structural and functional changes and their relationship to sleep-disordered breathing (SDB) in children with β-thalassemia and SCD. One hundred patients recruited from the hematology clinic were subjected to Pittsburgh Sleep Quality Index score; 26 patients had positive score (Pittsburgh Sleep Quality Index ≥5) (15 β-thalassemia major and 11 SCD) and were compared with 25 age-matched and sex-matched controls. All underwent polysomnography and tissue Doppler echocardiography. SDB was detected in 73% of thalassemia patients (all had increased LV mass index [LVMI], diastolic dysfunction [increased E/Em], and 53% had pulmonary hypertension [tricuspid valve resurgence (TR) velocity ≥2.5 m/s]) and in 46% of SCD patients ( all had increased LVMI, 81.8% had pulmonary hypertension, and 76% had diastolic dysfunction). Sleep O2 saturation of β-thalassemia patients negatively correlated with TR velocity and LVMI (P=0.027, 0.015), and lower asleep O2 saturation was associated with increased E/Em. In SCD patients, sleep and awake O2 saturation negatively correlated with TR velocity and E/Em (P=0.024 and 0.041), and lower sleep O2 saturation was associated with increased LV diameter (P=0.021). SDB is common and associated with LV structural and functional changes in β-thalassemia and SCD.
心血管并发症在β地中海贫血和镰状细胞病（SCD）中已得到充分认识。本研究的目的是评估β地中海贫血和SCD患儿的左心室（LV）结构和功能变化及其与睡眠呼吸障碍（SDB）的关系。从血液科门诊招募的100名患者接受了匹兹堡睡眠质量指数评分；26名患者评分呈阳性（匹兹堡睡眠质量指数≥5）（15名重型β地中海贫血患者和11名SCD患者），并与25名年龄和性别匹配的对照组进行比较。所有患者均接受了多导睡眠图检查和组织多普勒超声心动图检查。73%的地中海贫血患者检测到SDB（所有患者左心室质量指数[LVMI]增加、舒张功能障碍[E/Em增加]，53%患有肺动脉高压[三尖瓣反流（TR）速度≥2.5m/s]），46%的SCD患者检测到SDB（所有患者LVMI增加，81.8%患有肺动脉高压，76%患有舒张功能障碍）。β地中海贫血患者的睡眠氧饱和度与TR速度和LVMI呈负相关（P=0.027，0.015），较低的睡眠氧饱和度与E/Em增加有关。在SCD患者中，睡眠和清醒时的氧饱和度与TR速度和E/Em呈负相关（P=0.024和0.041），较低的睡眠氧饱和度与左心室直径增加有关（P=0.021）。SDB在β地中海贫血和SCD中很常见，并且与左心室结构和功能变化有关。
