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.
 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.

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.

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 (https://books.go
ogle.com/books?id=mhqswaCtcLQC&pg=PA81) . 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" (http://sickle.bwh.harvard.edu/

acutechest.html) .
 3. Sickle cell disease (SCD) (https://www.cdc.gov/ncbddd/sicklecell/treatments.html) . 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 (https://doi.org/10.1002%2F14651858.CD003733.pub3) .
PMID 25086371 (https://pubmed.ncbi.nlm.nih.gov/25086371) .

5. "acute chest syndrome (https://web.archive.org/web/20090628224336/http://www.mercksource.co

m/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/nine/2071163
8.htm) " at Dorland's Medical Dictionary

. Kumar, Abbas, Fausto. Robbins and Cotran: The Pathologic Basis of Disease, Page 631

External links

Classification ICD-9-CM: 517.3 • MeSH: D056586 D

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