Acute chest syndrome

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]

Acute chest syndrome
Specialty	Pulmonology

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.^{[citation needed]}

Diagnosis

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

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

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

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

1. 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.
2. Johnson, CS (1995). "Sickle-Cell Disease: The Acute Chest Syndrome".
3. Sickle cell disease (SCD). Centers for Disease Control and Prevention. Retrieved January 7, 2015.
4. 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.
5. "" at Dorland's Medical Dictionary
6. Kumar, Abbas, Fausto. Robbins and Cotran: The Pathologic Basis of Disease, Page 631

External links