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Acute chest syndrome in sickle cell disease: clinical presentation and
course. Cooperative Study of Sickle Cell Disease
EP Vichinsky, LA Styles, LH Colangelo, EC Wright, O Castro and B Nickerson
Children's Hospital Oakland, CA 94609, USA.
Acute chest syndrome (ACS) is an important cause of morbidity and mortality
in sickle cell disease (SCD). Previous studies reported conflicting
pictures of ACS making therapeutic interventions difficult. The Cooperative
Study of Sickle Cell Disease prospectively followed 3,751 patients enrolled
from birth to 66 years of age for ACS. Data on presenting signs and
symptoms, laboratory findings, and hospital course were collected. There
were 1,722 ACS episodes in 939 patients. Young children (age 2 to 4 years)
presented with fever and cough, a negative physical exam, and rarely had
pain. Adults were often afebrile and complained of shortness of breath,
chills, and severe pain. Upper lobe disease was more common in children;
multilobe and lower lobe disease affected adults more often. Severe hypoxia
occurred in 18% of adults tested and could not be predicted by examination
or laboratory findings. Bacteremia was documented in 3.5% of episodes, but
was strongly influenced by age (14% of infants and 1.8% of patients > 10
years). ACS was most common in winter with children having the most
striking increase. Transfusion was used less frequently, but earlier in
children. Young children were hospitalized for 5.4 days versus 9 days for
adults. Fifty percent of adults had a pain event in the 2 weeks preceding
ACS and children were more likely to have febrile events. The death rate
was four times higher in adults than in children. Fatal cases generally
developed rapid pulmonary failure and one third were associated with
bacteremia. Age has a striking effect on the clinical picture of ACS. In
children, ACS was milder and more likely due to infection, whereas in
adults ACS was severe, associated with pain and had a higher mortality
rate.
Volume 89,
Issue 5,
pp. 1787-1792,
03/01/1997
Copyright © 1997 by The American Society of Hematology
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