Acute chest syndrome
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| Acute chest syndrome | |
|---|---|
| Specialty | Pulmonology  |
The acute chest syndrome is a vaso-occlusive crisis of the pulmonary vasculature commonly seen in people with sickle cell anemia. This condition commonly manifests with a new opacification of the lung(s) on a chest x-ray.[1]
Signs and symptoms[edit]
The crisis is a common complication in sickle-cell patients and can be associated with one or more symptoms including fever, cough, excruciating pain, sputum production, shortness of breath, or low oxygen levels.[2]
Cause[edit]
Acute chest syndrome is often precipitated by a lung infection, and the resulting inflammation and loss of oxygen saturation leads to further sickling of red cells, thus exacerbating pulmonary and systemic hypoxemia, sickling, and vaso-occlusion.[citation needed]
Diagnosis[edit]
The diagnosis of acute chest syndrome is made difficult by its similarity in presentation with pneumonia. Both may present with a new opacification of the lung on chest x-ray. The presence of fevers, low oxygen levels in the blood, increased respiratory rate, chest pain, and cough are also common in acute chest syndrome. Diagnostic workup includes chest x-ray, complete cell count, reticulocyte count, ECG, and blood and sputum cultures. Patients may also require additional blood tests or imaging (e.g. a CT scan) to exclude a heart attack or other pulmonary pathology.[citation needed]
Prevention[edit]
Hydroxyurea is a medication that can help to prevent acute chest syndrome. It may cause a low white blood cell count, which can predispose the person to some types of infection.[3]
Treatment[edit]
Broad spectrum antibiotics to cover common infections such as Streptococcus pneumoniae and mycoplasma, pain control, and blood transfusion. Acute chest syndrome is an indication for exchange transfusion.[citation needed]
Bronchodilators may be useful but have not been well studied.[4]
Prognosis[edit]
It may result in death,[5] and it is one of the most common causes of death for people with sickle cell anemia.[6]
References[edit]
- ^ Betty Pace (2007). Renaissance of Sickle Cell Disease Research in the Genome Era. Imperial College Press. pp. 81–. ISBN 978-1-86094-645-5. Retrieved 15 June 2010.
- ^ Johnson, CS (1995). "Sickle-Cell Disease: The Acute Chest Syndrome".
- ^ Sickle cell disease (SCD). Centers for Disease Control and Prevention. Retrieved January 7, 2015.
- ^ Knight-Madden, JM; Hambleton, IR (Aug 2, 2014). "Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease". The Cochrane Database of Systematic Reviews. 8 (8): CD003733. doi:10.1002/14651858.CD003733.pub3. PMID 25086371.
- ^ "acute chest syndrome" at Dorland's Medical Dictionary
- ^ Kumar, Abbas, Fausto. Robbins and Cotran: The Pathologic Basis of Disease, Page 631