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Previous Volume 342:1855-1865 June 22, 2000 Number 25 Next

	Full Text PDF Editorial by Platt, O. S. Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited Related Article PubMed Citation

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.

Source Information

From the Departments of Hematology–Oncology (E.P.V., L.D.N., A.N.E.) and Pathology (R.W.), Children's Hospital Oakland, Oakland, Calif.; Virolab, Berkeley, Calif. (E.T.L.); the Department of Medicine, University of California School of Medicine at San Francisco, San Francisco (D.D.); the Department of Pediatric Pulmonary Medicine, Children's Hospital of Orange County, Orange, Calif. (B.N.); the Department of Hematology, University of North Carolina, Chapel Hill (E.O.); the Department of Pediatric Hematology–Oncology, Medical College of Georgia, Augusta (V.M.); the Department of Medicine, New York Methodist Hospital, Brooklyn (R.B.); the Department of Pediatric Hematology–Oncology, East Carolina University, Greenville, N.C. (C.D.); and the Department of Pathology, University of Southern Alabama Doctors' Hospital, Mobile (E.A.M.). Other authors were Miguel Abboud, M.D. (Medical University of South Carolina, Charleston); Mark Moncino, M.D. (Scottish Rite Children's Medical Center, Atlanta); Samir Ballas, M.D. (Thomas Jefferson University, Philadelphia); and Russell Ware, M.D. (Duke University Medical Center, Durham, N.C.).

Address reprint requests to Dr. Vichinsky at Children's Hospital Oakland, 747–52nd St., Oakland, CA 94609, or at evichinsky{at}mail.cho.org.

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Related Letters:

Acute Chest Syndrome in Sickle Cell Disease
Avila P., Jay S. J., Dean D., Neumayr L. D., Vichinsky E. P.
Extract | Full Text  
N Engl J Med 2000; 343:1336-1337, Nov 2, 2000. Correspondence

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