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Cited by 573Hemoglobinopathies and Related DisordersPneumonia and Respiratory InfectionsMalaria Research and Control

Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course

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Vichinsky, Elliott P., Styles, Lori A., Colangelo, Linda H., Wright, Elizabeth C., Castro, Oswaldo, Nickerson, Bruce, the Cooperative Study of Sickle Cell Disease·1997·Blood
DOI 10.1182/blood.v89.5.1787
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Abstract

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.

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  • Infection is the most common acute chest syndrome trigger in children; common isolates include atypical microorganisms and Streptococcus pneumoniae.

    “Infection is the most common ACS trigger in children; common isolates include atypical microorganisms and Streptococcus pneumoniae (208).”

    From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [208]

    The Hospital course section says childhood ACS most likely results from infectious causes, but describes this as an inference rather than a confirmed ranking of triggers. The Results section identifies Streptococcus pneumoniae as a common isolate; it does not identify atypical microorganisms as common isolates. Page numbers are unavailable in our copy. (LLM, conf=0.88)

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  • Elevated initial platelet count and dramatic drops during vaso-occlusive crisis have been found to portend a poor prognosis in acute chest syndrome.

    “Elevated initial platelet count and dramatic drops during VOC have been found to portend a poor prognosis in ACS (29, 208, 219).”

    From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [208]

    The Laboratory findings passage compares hemoglobin and white blood cell counts with baseline values, but does not report platelet counts. The Hospital course says there were no clinical predictors of which patients would die, but it does not assess elevated initial platelet counts or platelet drops during vaso-occlusive crisis. Searches for platelet and platelet-count changes found no passage addressing the claimed prognostic association. (LLM, conf=0.73)

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Authors
Vichinsky, E. P., Styles, L. A., Colangelo, L. H., Wright, E. C., Castro, O., Nickerson, B., & Disease, T. C. S. O. S. C.
Affiliations
Children's Hospital of Orange County (US)
ORCID
Venue
Blood
Publisher
American Society of Hematology
Published
1997-03-01
Volume
89
Issue
5
Pages
1787-1792
ISSN
  • 0006-4971 (ISSN-L)
  • 1528-0020
DOI
10.1182/blood.v89.5.1787
OpenAlex
W1272191569
Cited by
573
References
30
Citations by year
2026: 13 · 2025: 18 · 2024: 13 · 2023: 17 · 2022: 22 · 2021: 23 · 2020: 20 · 2019: 27 · 2018: 22 · 2017: 20 · 2016: 32 · 2015: 22 · 2014: 26 · 2013: 28 · 2012: 25
FWCI
10.28
Type
journal article
Language
en
Open access
Locations
  • Blood (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
  • Health Sciences › Medicine › Epidemiology › Pneumonia and Respiratory Infections
  • Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Malaria Research and Control
Keywords
acute chest syndrome, sickle cell disease, clinical features, age differences, bacteremia, hypoxia, respiratory failure, pain, fever, hospital course, mortality, seasonal variation, blood transfusion
MeSH terms
Adolescent, Adult, Aged, Anemia, Sickle Cell, Chest Pain, Child, Child, Preschool, Humans, Infant, Middle Aged, Prospective Studies, Respiratory Insufficiency, Syndrome

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