Acute chest syndrome (ACS) is a frequent complication of sickle cell anemia. It can occur as an isolated event or develop during hospitalization for acute vaso-occlusive crisis (VOC). It is one of the most common causes of death in sickle cell anemia patients.

Clinical symptoms

ACS is characterized by fever, chest pain, difficulty breathing, hypoxia, and lung infiltrates. The condition may progress rapidly from mild hypoxia to respiratory failure and death. Symptoms can differ with age. Pain and difficulty breathing are the predominant symptoms in adults; fever, coughing, and wheezing are more common in children.

The severity of the condition can be predicted by indicators such as worsening hypoxia, increased respiratory rate, decreased platelet count, and decreased hemoglobin concentration.

Cause

ACS can occur due to multiple factors. A low level of oxygen inside the lungs makes it easier for sickled red blood cells to stick to blood vessels, resulting in decreased blood flow. This may cause a blockage in small blood vessels, leading to more severe hypoxia. Other causes include infections, pulmonary fat embolism (a condition in which fat particles enter the blood vessels in the lung), and infarction (tissue death due to blocked blood supply).

Diagnosis

ACS can be diagnosed following thorough investigations that include a chest X‐ray, full blood count, and blood and liver function tests. Blood and sputum should also be tested to identify bacterial or viral infections. Arterial blood gas analyses should be performed to determine hypoxia, a useful predictor of the severity and outcome of the condition.

Treatment

ACD should be treated promptly with pain relief medication, intravenous fluids, aggressive incentive spirometry, which improves lung function, supplemental oxygen or respiratory support, antibiotics, and transfusion therapy. If the patient has a history of asthma, bronchodilators should be considered. Recurrent episodes can be prevented with hydroxyurea treatment.

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