Division of Pediatric Pulmonology, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, United States of America.
Department of Pediatrics, Columbia University Irving Medical Center, New York, NY, United States of America.
PLoS One. 2023 Mar 30;18(3):e0283349. doi: 10.1371/journal.pone.0283349. eCollection 2023.
Acute chest syndrome (ACS) is an acute complication in SCD but its effects on lung function are not well understood. Inflammation is a key component of SCD pathophysiology but with an unclear association with lung function. We hypothesized that children with ACS had worse lung function than children without ACS and aimed to investigate the association of lung function deficits with inflammatory cytokines.
Patients enrolled in a previous 2-year randomized clinical trial who had consented to future data use, were enrolled for the present exploratory study. Patients were categorized into ACS and non-ACS groups. Demographic and clinical information were collected. Serum samples were used for quantification of serum cytokines and leukotriene B4 levels and pulmonary function tests (PFTs) were assessed.
Children with ACS had lower total lung capacity (TLC) at baseline and at 2 years, with a significant decline in forced expiratory volume in 1 sec (FEV1) and mid-maximal expiratory flow rate (FEF25-75%) in the 2 year period (p = 0.015 and p = 0.039 respectively). For children with ACS, serum cytokines IL-5, and IL-13 were higher at baseline and at 2 years compared to children with no ACS. IP-10 and IL-6 were negatively correlated with PFT markers. In multivariable regression using generalized estimating equation approach for factors predicting lung function, age was significantly associated FEV1 (p = 0.047) and ratio of FEV1 and forced vital capacity (FVC)- FEV1/FVC ratio (p = 0.006); males had lower FEV1/FVC (p = 0.035) and higher TLC (p = 0.031). Asthma status was associated with FEV1 (p = 0.017) and FVC (p = 0.022); history of ACS was significantly associated with TLC (p = 0.027).
Pulmonary function abnormalities were more common and inflammatory markers were elevated in patients with ACS, compared with those without ACS. These findings suggest airway inflammation is present in children with SCD and ACS, which could be contributing to impaired pulmonary function.
急性胸部综合征（ACS）是 SCD 的一种急性并发症，但人们对其对肺功能的影响了解甚少。炎症是 SCD 病理生理学的一个关键组成部分，但与肺功能的关联尚不清楚。我们假设 ACS 患儿的肺功能比无 ACS 患儿差，并旨在研究肺功能缺陷与炎症细胞因子的相关性。
本研究纳入了先前一项为期 2 年的随机临床试验中同意未来数据使用的患者，这些患者被纳入本探索性研究。患者分为 ACS 组和非 ACS 组。收集人口统计学和临床信息。检测血清细胞因子和白三烯 B4 水平，并进行肺功能测试（PFT）。
ACS 患儿的基础肺总量（TLC）和 2 年时的 TLC 均较低，在 2 年内用力呼气 1 秒量（FEV1）和中最大呼气流量率（FEF25-75%）显著下降（p=0.015 和 p=0.039）。与无 ACS 的患儿相比，ACS 患儿的基础期和 2 年期血清细胞因子 IL-5 和 IL-13 更高。IP-10 和 IL-6 与 PFT 标志物呈负相关。使用广义估计方程方法对预测肺功能的因素进行多变量回归分析，结果显示年龄与 FEV1 显著相关（p=0.047），与 FEV1 与用力肺活量的比值（FEV1/FVC）显著相关（p=0.006）；男性的 FEV1/FVC 较低（p=0.035），TLC 较高（p=0.031）。哮喘状态与 FEV1（p=0.017）和 FVC（p=0.022）相关；ACS 史与 TLC 显著相关（p=0.027）。
与无 ACS 的患儿相比，ACS 患儿的肺功能异常更常见，炎症标志物水平更高。这些发现表明，SCD 和 ACS 患儿的气道炎症存在，这可能导致肺功能受损。
