title: How I treat acute chest syndrome in children with sickle cell disease.
abstract: Acute chest syndrome describes new respiratory symptoms and findings, often severe and progressive, in a child with sickle cell disease and a new pulmonary infiltrate. It may be community-acquired or arise in children hospitalized for pain or other complications. Recognized etiologies include infection, most commonly with atypical bacteria, and pulmonary fat embolism (PFE); the cause is often obscure and may be multifactorial. Initiation of therapy should be based on clinical findings. Management includes macrolide antibiotics, supplemental oxygen, modest hydration and often simple transfusion. Partial exchange transfusion should be reserved for children with only mild anemia (Hb > 9 g/dL) but deteriorating respiratory status. Therapy with corticosteroids may be of value; safety, efficacy and optimal dosing strategy need prospective appraisal in a clinical trial. On recovery, treatment with hydroxyurea should be discussed to reduce the likelihood of recurrent episodes.
authors.affiliation: State University of New York/Downstate Medical Center, Kings County Hospital Center, 450 Clarkson Ave., Brooklyn, NY 11203, USA. scott.miller@downstate.edu
authors.forename: Scott T
authors.initials: ST
authors.lastname: Miller
mesh_terms: D056586:Acute Chest Syndrome / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy*; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000755:Anemia, Sickle Cell / Q000150:complications*; D001803:Blood Transfusion; D002648:Child; D002690:Chlamydia Infections / Q000150:complications; D016993:Chlamydophila pneumoniae; D004620:Embolism, Fat / Q000150:complications; D005078:Exchange Transfusion, Whole Blood; D006801:Humans; D018410:Pneumonia, Bacterial / Q000150:complications; D011019:Pneumonia, Mycoplasma / Q000150:complications; D011024:Pneumonia, Viral / Q000150:complications; D011655:Pulmonary Embolism / Q000150:complications; D012008:Recurrence
chemical_list: D000305:Adrenal Cortex Hormones