Alzahrani Fatma, Albaz Ghaidaa F, AlSinan Fatima, Alzuhayri Jumana, Barnawi Zahra M, Melebari Nouf, Al Twairgi Tethkar M
Pediatrics, King Abdulaziz University Hospital, Jeddah, SAU.
Medicine, King Abdulaziz University Hospital, Jeddah, SAU.
Cureus. 2021 Feb 20;13(2):e13453. doi: 10.7759/cureus.13453.
Background Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that is very common and causes a great burden in Saudi Arabia and worldwide. This study aims to determine the frequency and benefits of using hydroxyurea in treating children with SCD in King Abdulaziz University Hospital (KAUH) in Saudi Arabia. Method This retrospective observational study included all SCD patients, males and females, who were following up in KAUH and were less than 18 years old. Patients on chronic transfusion therapy or who had previous stem cell transplantation were excluded from the study. The study was approved by the unit of biomedical ethics in KAUH, Jeddah. Result The study included 102 SCD patients; the median age of the sample was 7.5±4.2, with the majority being female (58 patients; 56.9%). Hydroxyurea users among participants in the study were 62 patients (60.8%). Thirty-seven (37) of the patients using hydroxyurea had an increase in the mean corpuscular volume (MCV). There was a decrease in the level of white blood cells (WBCs) and neutrophil count from 15.81±10.24 and 48.31±23.23% to 12.48±5.48 and 40.81±15.78%, respectively. Platelet count showed an increase from 345.4±2.1096 to 359.162±199.87 after starting hydroxyurea. The incidence of vaso-occlusive crisis (VOC) increased after hydroxyurea initiation from 4.555±4.08 to 6.288±9.80. Moreover, the frequency of blood transfusion in patients using hydroxyurea was statistically significant as p-value = 0.048. Conclusion In conclusion, our results showed significant clinical and laboratory benefits of hydroxyurea in children with SCD. Hydroxyurea has been shown to reduce the frequency of VOC and blood transfusion in patients with SCD.
镰状细胞病(SCD)是一种常染色体隐性血红蛋白病,在沙特阿拉伯及全球都非常常见且造成了沉重负担。本研究旨在确定在沙特阿拉伯阿卜杜勒阿齐兹国王大学医院(KAUH)使用羟基脲治疗儿童SCD的频率及益处。
这项回顾性观察性研究纳入了所有在KAUH接受随访且年龄小于18岁的SCD患者,男女不限。接受慢性输血治疗或曾接受过干细胞移植的患者被排除在研究之外。该研究获得了吉达KAUH生物医学伦理部门的批准。
该研究纳入了102例SCD患者;样本的中位年龄为7.5±4.2岁,大多数为女性(58例;56.9%)。研究参与者中使用羟基脲的有62例患者(60.8%)。使用羟基脲的37例患者平均红细胞体积(MCV)有所增加。白细胞(WBC)水平和中性粒细胞计数分别从15.81±10.24和48.31±23.23%降至12.48±5.48和40.81±15.78%。开始使用羟基脲后血小板计数从345.4±2.1096增加到359.162±199.87。开始使用羟基脲后血管闭塞性危机(VOC)的发生率从4.555±4.08增加到6.288±9.80。此外,使用羟基脲的患者输血频率具有统计学意义,p值 = 0.048。
总之,我们的结果显示羟基脲对儿童SCD具有显著的临床和实验室益处。已证明羟基脲可降低SCD患者VOC的频率和输血频率。