Potentially life-threatening emergency
Acute chest syndrome
Alerts and Notices
Synopsis

Sickle cell disease is the primary risk factor for ACS. Within the population with sickle cell disease, the following are predictors for increased risk of developing ACS: young age, low fetal hemoglobin, leukocytosis, genotypes HbSS (sickle cell anemia) and HbSβ0, asthma, tobacco exposure, recent surgery, or 3 or more severe veno-occlusive crises in the past year.
Management requires hospitalization for hydration, oxygenation, intravenous (IV) antibiotics, pain control, and blood transfusions and, in some cases, exchange transfusion.
Related topic: sickle cell acute pain crisis
Codes
ICD10CM:J99 – Respiratory disorders in diseases classified elsewhere
SNOMEDCT:
372146004 – Acute chest syndrome
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Pneumonia: viral, bacterial, fungal
- Pulmonary infarction
- Symptomatic anemia (hemolytic or bone marrow hypoproduction [eg, Fanconi anemia])
- Acute coronary syndrome
- Pulmonary embolism
- Upper respiratory infection
- Atelectasis
- Fat embolism
- Asthma exacerbation
- Pneumothorax
- Costochondritis
- Pericarditis
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:03/15/2018
Last Updated:05/27/2021
Last Updated:05/27/2021