Cited by 1,234Hemoglobinopathies and Related DisordersBlood transfusion and managementPneumonia and Respiratory Infections

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Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease

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Vichinsky, Elliott P., Neumayr, Lynne D., Earles, Ann N., Williams, Roger, Lennette, Evelyne T., Dean, Deborah, Nickerson, Bruce, Orringer, Eugene, McKie, Virgil, Bellevue, Rita, Daeschner, Charles, Abboud, Miguel, Moncino, Mark, Ballas, Samir, Ware, Russell, Manci, Elizabeth A.·2000·New England Journal of Medicine

DOI 10.1056/nejm200006223422502

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Abstract

BACKGROUND: The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome. METHODS: In a 30-center study, we analyzed 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease to determine the cause, outcome, and response to therapy. We evaluated a treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy. Samples of blood and respiratory tract secretions were sent to central laboratories for antibody testing, culture, DNA testing, and histopathological analyses. RESULTS: Nearly half the patients were initially admitted for another reason, mainly pain. When the acute chest syndrome was diagnosed, patients had hypoxia, decreasing hemoglobin values, and progressive multilobar pneumonia. The mean length of hospitalization was 10.5 days. Thirteen percent of patients required mechanical ventilation, and 3 percent died. Patients who were 20 or more years of age had a more severe course than those who were younger. Neurologic events occurred in 11 percent of patients, among whom 46 percent had respiratory failure. Treatment with phenotypically matched transfusions improved oxygenation, with a 1 percent rate of alloimmunization. One fifth of the patients who were treated with bronchodilators had clinical improvement. Eighty-one percent of patients who required mechanical ventilation recovered. A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data. Among the specific causes were pulmonary fat embolism and 27 different infectious pathogens. Eighteen patients died, and the most common causes of death were pulmonary emboli and infectious bronchopneumonia. Infection was a contributing factor in 56 percent of the deaths. CONCLUSIONS: Among patients with sickle cell disease, the acute chest syndrome is commonly precipitated by fat embolism and infection, especially community-acquired pneumonia. Among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure. Treatment with transfusions and bronchodilators improves oxygenation, and with aggressive treatment, most patients who have respiratory failure recover.

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Authors: Vichinsky, E. P., Neumayr, L. D., Earles, A. N., Williams, R., Lennette, E. T., Dean, D., Nickerson, B., Orringer, E., McKie, V., Bellevue, R., Daeschner, C., Abboud, M., Moncino, M., Ballas, S., Ware, R., & Manci, E. A.
Affiliations: University of California, San Francisco (US)
Children's Hospital of Orange County (US)
University of North Carolina at Chapel Hill (US)
Augusta University (US)
NewYork–Presbyterian Brooklyn Methodist Hospital (US)
East Carolina University (US)
Duke Medical Center (US)
University of South Alabama Medical Center (US)
ORCID: Vichinsky, Elliott P.
Neumayr, Lynne D.
Dean, Deborah
Abboud, Miguel
Ballas, Samir
Ware, Russell
Manci, Elizabeth A.
Venue: New England Journal of Medicine
Publisher: Massachusetts Medical Society
Published: 2000-06-22
Volume: 342
Issue: 25
Pages: 1855-1865
ISSN: 0028-4793 (ISSN-L)
1533-4406
DOI: 10.1056/nejm200006223422502
OpenAlex: W2332642667
Cited by: 1,234
References: 44
Citations by year: 2026: 35 · 2025: 55 · 2024: 52 · 2023: 62 · 2022: 59 · 2021: 67 · 2020: 75 · 2019: 44 · 2018: 48 · 2017: 38 · 2016: 63 · 2015: 51 · 2014: 57 · 2013: 59 · 2012: 51
FWCI: 16.73
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: New England Journal of Medicine (publishedVersion)
PubMed (publishedVersion)
Carolina Digital Repository (University of North Carolina at Chapel Hill) (submittedVersion)
UNC Libraries
Funders: University of Southern California
University of Miami
Emory University
University of South Alabama
National Institutes of Health (5p60hl020985-25)
National Heart, Lung, and Blood Institute (HL20985, HL 99016)
National Center for Research Resources (M01 RR 01271)
Children's Hospital of Pittsburgh
Topics: Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
Health Sciences › Medicine › Hematology › Blood transfusion and management
Health Sciences › Medicine › Epidemiology › Pneumonia and Respiratory Infections
Keywords: acute chest syndrome, sickle cell disease, pulmonary fat embolism, infection, community-acquired pneumonia, bronchodilators, bronchoscopy, mechanical ventilation, respiratory failure, neurological complications, mortality, hypoxia
MeSH terms: Acute Disease, Adolescent, Adult, Anemia, Sickle Cell, Blood Transfusion, Bronchodilator Agents, Chest Pain, Child, Child, Preschool, Embolism, Fat, Female, Humans, Infections, Lung Diseases, Male, Prospective Studies, Pulmonary Embolism, Respiration, Artificial, Respiratory Insufficiency, Proportional Hazards Models, Community-Acquired Infections

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Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease

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