title: The role of secretory phospholipase A2 in acute chest syndrome.
abstract: Acute chest syndrome (ACS) is the leading cause of death in sickle cell disease. Severe ACS often develops in the course of a vasoocclusive crisis (VOC), and frequently involves pulmonary fat embolism. Secretory phospholipase A2 (sPLA2), a potent inflammatory mediator, is elevated in ACS, and sPLA2 levels in serum or plasma predict impending ACS. In addition sPLA2 may play a major role in the actual damage to the lung resulting in a new pulmonary infiltrate on chest radiography, respiratory symptoms, and ultimately alveolar collapse and the impairment of gas exchange. The data indicate that measurement of sPLA2 can be useful in alerting the clinician to patients with impending ACS, and suggest that instituting early therapies based on sPLA2 levels, including inhibition of sPLA2 activity, may be useful to prevent or reduce the clinical morbidity of ACS in sickle cell disease.
authors.affiliation: Children's Hospital Oakland Research Institute, Oakland, CA, USA. fkuypers@chori.org
authors.forename: F A
authors.initials: FA
authors.lastname: Kuypers
authors.forename: L A
authors.initials: LA
authors.lastname: Styles
mesh_terms: D000208:Acute Disease; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D006801:Humans; D008171:Lung Diseases / Q000097:blood / Q000175:diagnosis / Q000201:enzymology* / Q000209:etiology*; D010741:Phospholipases A / Q000097:blood / Q000378:metabolism*; D054467:Phospholipases A2; D013577:Syndrome
chemical_list: D010741:Phospholipases A; D054467:Phospholipases A2