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The acute chest syndrome in children and adolescents with sickle cell disease

Authors
Matthew Heeney, MD
Donald H Mahoney, Jr, MD
Section Editors
George B Mallory, MD
Stanley L Schrier, MD
Deputy Editor
Jennifer S Tirnauer, MD

INTRODUCTION

Acute and chronic pulmonary complications occur frequently in patients with sickle cell disease (SCD). The acute chest syndrome (ACS) is one of the acute pulmonary complications seen in patients with SCD. ACS is a major cause of morbidity and mortality, and its prevention would reduce the death rate and sequelae in patients with SCD.

The etiology, diagnosis, and management of ACS in children and adolescents will be reviewed here. ACS in adults, and other pulmonary manifestations of SCD in adults and children, are discussed separately. (See "Overview of the pulmonary complications of sickle cell disease" and "Overview of the management and prognosis of sickle cell disease" and "Acute chest syndrome in adults with sickle cell disease".)

EPIDEMIOLOGY

ACS is a common complication in children with SCD. In a report from the Cooperative Study of Sickle Cell Disease (CSSCD), the peak incidence for ACS was in children between two and four years of age (25.3 per 100 patient-years among children with hemoglobin SS), with a higher prevalence during the winter months [1].

For patients with SCD, ACS is the second most common cause of hospitalization (second to vasoocclusive pain) with a reported rate of 12.8 hospitalizations per 100 patient years [1]. It is the most common cause of death [2], with one-fourth of SCD-related deaths due to ACS [3]. In a report from the CSSCD, the death rate in patients with ACS is 1.8 percent in children and 4.3 percent in adults [4].

Nearly half of patients with ACS develop this disorder during hospitalization for another complication of SCD, most frequently vasoocclusive crisis. Other causes of hypoxia during hospitalization that can precipitate ACS include postoperative atelectasis and bronchospasm due to asthma.

                                

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Literature review current through: Nov 2016. | This topic last updated: Wed Dec 09 00:00:00 GMT+00:00 2015.
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