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Potentially life-threatening emergency
Acute chest syndrome
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Potentially life-threatening emergency

Acute chest syndrome

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Contributors: Michael W. Winter MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Acute chest syndrome (ACS) is a potentially life-threatening complication of sickle cell disease characterized by lung infiltrates, fever, and respiratory symptoms. Management of ACS in children and adults is similar, although the disease is often more severe in adults and associated with bone marrow and fat emboli. ACS can be triggered by an underlying infection or vaso-occlusive crisis, although in the majority of cases, an identifiable trigger is not determined.

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

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Last Reviewed: 03/16/2018
Last Updated: 04/17/2018
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Potentially life-threatening emergency
Acute chest syndrome
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Acute chest syndrome (Older Child or Adult) : Chest pain, Fever, Arm pain, Leg pain, Dyspnea, Tachypnea
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