Acute chest syndrome
| Acute chest syndrome | |
|---|---|
| Classification and external resources | |
| Specialty | pulmonology |
| ICD-9-CM | 517.3 |
The acute chest syndrome is a vaso-occlusive crisis of the pulmonary vasculature commonly seen in patients with sickle cell anemia. This condition commonly manifests with pulmonary infiltrate on a chest x-ray.[1]
Signs and symptoms
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
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.
Treatment
Broad spectrum antibiotics to cover common infections like strep pneumoniae and mycoplasma, pain control, and blood transfusion. Acute chest syndrome is an indication for exchange transfusion.
Bronchodilators may be useful but have not been well studied.[3]
Prognosis
It may result in death,[4] and it is one of the most common causes of death for sickle cell patients.[5]
Prevention
Hydroxyurea is a medication that can help to prevent chest syndrome. It may cause a low white blood cell count, which can predispose the patient to some types of infection.[6]
References
- ↑ 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".
- ↑ 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: CD003733. PMID 25086371. doi:10.1002/14651858.CD003733.pub3.
- ↑ "acute chest syndrome" at Dorland's Medical Dictionary
- ↑ Kumar, Abbas, Fausto. Robbins and Cotran: The Pathologic Basis of Disease, Page 631
- ↑ Sickle cell disease (SCD). Centers for Disease Control and Prevention. Retrieved January 7, 2015.