Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences

View all Images (2)

Potentially life-threatening emergency
Acute chest syndrome
Other Resources UpToDate PubMed
Potentially life-threatening emergency

Acute chest syndrome

Contributors: Michael W. Winter MD, Eric Ingerowski MD, FAAP, 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, including cough, tachypnea, wheeze, increased work of breathing / shortness of breath, and reduced oxygen saturations. ACS is the second most common reason for hospitalization in children and adults with sickle cell disease and is the leading cause of mortality. 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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

To perform a comparison, select diagnoses from the classic differential

Subscription Required

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:03/15/2018
Last Updated:05/27/2021
Copyright © 2024 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Acute chest syndrome
Print   View all Images (2)
(with subscription)
A medical illustration showing key findings of Acute chest syndrome (Older Child or Adult) : Chest pain, Fever, Arm pain, Leg pain, Dyspnea, Tachypnea
Copyright © 2024 VisualDx®. All rights reserved.