Acute Chest Syndrome in Children with Sickle Cell Disease

Abstract

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.

Low‐risk factors for severe bacterial infection and acute chest syndrome in children with sickle cell disease

“…Children were defined as having possible SBI if they had a clinical syndrome compatible with a bacterial infection not microbiologically confirmed. For the purposes of this study, both pneumonia without a bacterial confirmation and acute chest syndrome (ACS) (defined as the presence of fever and acute respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X‐ray) were considered equally in the group of possible SBI. Mild bacterial infections, such as group A streptococcal pharyngitis or bacterial gastroenteritis without bacteremia, were not included in the study.…”

Soluble receptor for advanced glycation end products as a vasculopathy biomarker in sickle cell disease

“…Acute chest syndrome (ACS) is a frequent cause of acute lung injury in children with SCD. 29 In the physiological state, pulmonary tissue displays a relatively high expression of RAGE, with alveolar type I(ATI) pneumocytes accounting for the vast majority of RAGE expression. Accordingly, alteration in RAGE levels and RAGE-ligand interaction have been involved in damage to ATI pneumocytes which is an important feature of acute lung injury.…”

Multidisciplinary management of homozygous sickle cell patients: Dental treatment pathologies and needs.

“…[11] The Vaso-occlusive crisis characterizes the critical phase of SSD and first clinical signs are pains in the child's limbs and in the adolescent's abdomen. [12,13] Tested patients from this study had dental pains during vasoocclusive and hemolytic attacks. The pulp vaso-occlusive crisis causes an ischemia which describes the dental pain etiology.…”