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Acute Chest Syndrome in Children with Sickle Cell Disease.
Pediatric Allergy, Immunology, and Pulmonology ( IF 0.9 ) Pub Date : 2017-12-28 , DOI: 10.1089/ped.2017.0814
Shilpa Jain 1 , Nitya Bakshi 2 , Lakshmanan Krishnamurti 2
Affiliation  

Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray. The spectrum of clinical manifestations can range from mild respiratory illness to acute respiratory distress syndrome. The presence of severe hypoxemia is a useful predictor of severity and outcome. The etiology of ACS is often multifactorial. One of the proposed mechanisms involves increased adhesion of sickle red cells to pulmonary microvasculature in the presence of hypoxia. Other commonly associated etiologies include infection, pulmonary fat embolism, and infarction. Infection is a common cause in children, whereas adults usually present with pain crises. Several risk factors have been identified in children to be associated with increased incidence of ACS. These include younger age, severe SCD genotypes (SS or Sβ0 thalassemia), lower fetal hemoglobin concentrations, higher steady-state hemoglobin levels, higher steady-state white blood cell counts, history of asthma, and tobacco smoke exposure. Opiate overdose and resulting hypoventilation can also trigger ACS. Prompt diagnosis and management with intravenous fluids, analgesics, aggressive incentive spirometry, supplemental oxygen or respiratory support, antibiotics, and transfusion therapy, are key to the prevention of clinical deterioration. Bronchodilators should be considered if there is history of asthma or in the presence of acute bronchospasm. Treatment with hydroxyurea should be considered for prevention of recurrent episodes. This review evaluates the etiology, pathophysiology, risk factors, clinical presentation of ACS, and preventive and treatment strategies for effective management of ACS.

中文翻译:

镰状细胞病患儿的急性胸综合症。

急性胸部综合症(ACS)是镰状细胞疾病(SCD)儿童急性肺部疾病的常见原因。患者可能因急性血管闭塞性危机(VOC)住院期间出现ACS或出现这种并发症。ACS与住院时间延长,呼吸衰竭的风险增加以及发生慢性肺病的可能性有关。SCD中的ACS定义为出现发烧和/或新的呼吸道症状,并在胸部X光片上出现新的肺部浸润。临床表现范围从轻度呼吸系统疾病到急性呼吸窘迫综合征。严重低氧血症的存在是严重程度和预后的有用预测指标。ACS的病因通常是多因素的。所提出的机制之一涉及在缺氧的情况下镰状红细胞对肺微血管的粘附增加。其他通常相关的病因包括感染,肺脂肪栓塞和梗塞。感染是儿童的常见病因,而成年人通常会出现疼痛危机。在儿童中已发现几种与ACS发生率增加相关的危险因素。这些因素包括年龄较小,严重的SCD基因型(SS或Sβ0地中海贫血),较低的胎儿血红蛋白浓度,较高的稳态血红蛋白水平,较高的稳态白细胞计数,哮喘病史和烟草烟雾暴露。阿片类药物过量和通气不足也会触发ACS。通过静脉输液,镇痛药,积极的肺活量测定法迅速诊断和管理,补充氧气或呼吸支持,抗生素和输血疗法,是预防临床恶化的关键。如果有哮喘病史或存在急性支气管痉挛,应考虑使用支气管扩张剂。应考虑用羟基脲治疗以预防复发。这篇综述评估了ACS的病因,病理生理,危险因素,临床表现以及有效管理ACS的预防和治疗策略。
更新日期:2019-11-01
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