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]
| Acute chest syndrome | |
|---|---|
| Specialty | Pulmonology  |
Signs and symptoms
editThe 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]
Various Etiologies
editAcute 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]
In addition to lung infection as a possible etiology of acute chest syndrome, bone marrow fat embolism, atelectasis, pulmonary edema, or sickle cell aggregation in the intrapulmonary vasculature can precipitate acute chest syndrome. While the instigating factor may not be readily apparent at onset, one distinctive lab value that may set acute chest syndrome from various other causes include a precipitous drop in hemoglobin way below the patient's baseline. [3]
Diagnosis
editThe 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
editHydroxyurea 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.[4]
Treatment
editBroad 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.[5]
Complications
editComplications associated with acute chest syndrome can be delineated into acute and chronic acute chest syndrome complications. Acute complications include respiratory failure, altered mental status, seizures, stroke, intrapulmonary hemolysis, and multi-system organ failure. Chronic complications from recurring acute chest syndrome include cor pulmonale, pulmonary hypertension, chronic sickle lung disease, and restrictive pulmonary function.[6]
Prognosis
editReferences
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".
- ↑ National Heart, Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. Bethesda, MD: National Heart, Lung, and Blood Institute; 2014. https://www.nhlbi.nih.gov/sites/default/files/publications/56-364NFULL.pdf
- ↑ 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.
- ↑ American Lung Association. “Learn About Acute Chest Syndrome.” American Lung Association, https://www.lung.org/lung-health-diseases/lung-disease-lookup/acute-chest-syndrome/learn-about-acute-chest-syndrome
- ↑ "acute chest syndrome" at Dorland's Medical Dictionary
- ↑ Kumar, Abbas, Fausto. Robbins and Cotran: The Pathologic Basis of Disease, Page 631