Wang Karen, Olave Nelida, Aggarwal Saurabh, Oh Joo-Yeun, Patel Rakesh P, Rahman A K M Fazlur, Lebensburger Jeffrey, Alishlash Ammar Saadoon
Heersink School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Department of Pediatrics, Heersink School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Eur J Haematol. 2025 Feb;114(2):325-333. doi: 10.1111/ejh.14342. Epub 2024 Nov 5.
Acute Chest Syndrome (ACS) is the leading cause of death in children with sickle cell disease (SCD) in the US-about half of the children who develop ACS present initially with pain.
Here, we studied biomarkers to differentiate ACS from vaso-occlusive crises (VOC) in children with SCD who presented with pain to the emergency department (ED). We conducted a prospective cohort study of consecutive patients who presented to the ED with pain and were discharged with ACS or VOC between March, 2017 and February, 2020.
We identified 7 patients with ACS and 19 patients with VOC. The two groups were comparable in age and sex. All patients with ACS had asthma versus 42% of the VOC group. The ACS group had lower weight and BMI z-scores. Patients with ACS compared to VOC had significantly higher respiratory rates, lower O saturation, and longer hospital stays. They also had higher white blood cell count, glucose level (> 99 mg/dL), anion gap (> 9 mEq/L), sPLA2 (> 7 pg/mL), IFN-γ (> 17.8 pg/mL), IL-10 (1.54 pg/mL), and IL-12 (> 0.5 pg/mL) levels.
We identified biomarkers associated with ACS development in children with SCD presenting with pain that allow for earlier ACS interventions to reduce mortality and morbidity.
急性胸综合征（ACS）是美国镰状细胞病（SCD）患儿的主要死因——约一半发生ACS的患儿最初表现为疼痛。
在此，我们研究了生物标志物，以区分急诊科（ED）中因疼痛就诊的SCD患儿的ACS与血管闭塞性危机（VOC）。我们对2017年3月至2020年2月期间连续因疼痛到急诊科就诊并出院诊断为ACS或VOC的患者进行了一项前瞻性队列研究。
我们确定了7例ACS患者和19例VOC患者。两组在年龄和性别上具有可比性。所有ACS患者均患有哮喘，而VOC组这一比例为42%。ACS组的体重和BMI z评分较低。与VOC患者相比，ACS患者的呼吸频率显著更高、血氧饱和度更低、住院时间更长。他们的白细胞计数、血糖水平（>99mg/dL）、阴离子间隙（>9mEq/L）、sPLA2（>7pg/mL）、IFN-γ（>17.8pg/mL）、IL-10（1.54pg/mL）和IL-12（>0.5pg/mL）水平也更高。
我们确定了与因疼痛就诊的SCD患儿ACS发生相关的生物标志物，这些生物标志物有助于早期进行ACS干预，以降低死亡率和发病率。
