UCL GOS Institute of Child Health, University College London, London, UK.
King's College Hospital, King's College London, London, UK
Eur Respir Rev. 2024 Sep 18;33(173). doi: 10.1183/16000617.0005-2024. Print 2024 Jul.
Acute chest syndrome (ACS) is a leading cause of respiratory distress and hospitalisation in children with sickle cell disease (SCD). The aetiology is multifactorial and includes fat embolism, venous thromboembolism, alveolar hypoventilation and respiratory infections, with the latter being particularly common in children. These triggers contribute to a vicious cycle of erythrocyte sickling, adhesion to the endothelium, haemolysis, vaso-occlusion and ventilation-perfusion mismatch in the lungs, resulting in the clinical manifestations of ACS. The clinical presentation includes fever, chest pain, dyspnoea, cough, wheeze and hypoxia, accompanied by a new pulmonary infiltrate on chest radiography. Respiratory symptoms may overlap with those of acute asthma, which may be difficult to distinguish. Patients with ACS may deteriorate rapidly; thus prevention, early recognition and aggressive, multidisciplinary team management is essential. In this narrative review, we highlight the current evidence regarding the epidemiology, pathophysiology, treatment and preventative strategies for ACS, focusing on the aspects of major interest for the paediatric pulmonologist and multidisciplinary team who manage children with SCD.
急性胸部综合征（ACS）是导致镰状细胞病（SCD）患儿呼吸窘迫和住院的主要原因。其病因是多因素的，包括脂肪栓塞、静脉血栓栓塞、肺泡通气不足和呼吸道感染，后者在儿童中尤为常见。这些诱因导致红细胞镰状化、黏附在内皮细胞上、溶血、血管阻塞和肺部通气-灌注不匹配的恶性循环，从而导致 ACS 的临床表现。临床表现包括发热、胸痛、呼吸困难、咳嗽、喘息和缺氧，并伴有胸部 X 线新出现的肺部浸润。呼吸症状可能与急性哮喘重叠，这可能难以区分。ACS 患者可能迅速恶化；因此，预防、早期识别和积极的多学科团队管理至关重要。在这篇叙述性综述中，我们重点介绍了 ACS 的流行病学、病理生理学、治疗和预防策略方面的现有证据，重点关注儿科肺科医生和管理 SCD 儿童的多学科团队感兴趣的方面。
