Acute Chest Syndrome of Sickle Cell Disease
نویسنده
چکیده
Hospital Physician January 2007 15 T he acute chest syndrome (ACS) is an acute pulmonary illness that occurs in patients with sickle cell disease. ACS is currently defined as a new infiltrate on chest radiograph in conjunction with 1 other new symptom or sign: chest pain, cough, wheezing, tachypnea, and/or fever (> 38.5°C).1 The term acute chest syndrome was first suggested in 1979 by Charache et al2 and was developed to reflect the unique nature of acute pulmonary illness in patients with sickle cell disease. ACS can be caused by a variety of mechanisms, both infectious and noninfectious. Diagnostic considerations and treatment modalities are not typical of any other specific pulmonary illness experienced by the general population. Furthermore, the typical course, possible complications, and outcomes are unique. For these reasons, the terminology persists and remains useful for both research purposes and effective clinical communication. As a leading cause of hospitalization and death in adults with sickle cell disease, the importance of ACS in sickle cell patients cannot be overstated.3 Emergency physicians, family practitioners, pediatricians, internists, and hematologists who encounter these patients on a regular basis have the potential to prevent significant morbidity and mortality through early recognition and aggressive treatment of ACS. This article reviews the etiology, pathogenesis, clinical characteristics, treatment, and prevention of ACS.
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