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Lung Function Patterns in Sickle Cell Anemia
Assessing patterns for early mortality
Understanding the pathophysiology of a low forced expiratory volume in 1 second (FEV1% predicted) in people with sickle cell anemia (SCA) enables early intervention for those at risk, according to a study of 430 patients with SCA. Researchers found:
• 63 deaths occurred in the cohort; their lung function patterns included: 47% normal, 29% restrictive, 8% obstructive, 2% mixed, and 14% non-specific.
• Lower FEV1% predicted was associated with increased hazard of death (HR per %-predicted, 1.02) as was older age (HR, 1.07), male sex (HR, 2.09), higher lactate dehydrogenase levels (HR per mg/dl, 1.002), and higher acute chest syndrome incidence (HR per event/yr, 10.4).
• Presence of obstructive (HR, 1.18) and restrictive (HR, 1.31) pulmonary function patterns were not associated with earlier death.
Citation: Kassim AA, Payne AB, Rodeghier M, Macklin EA, Strunk RC, DeBaun MR. Forced expiratory volume in 1 second is associated with earlier death in sickle cell anemia. [Published online ahead of print August 10, 2015]. Blood. doi: http://dx.doi.org/10.1182/blood-2015-05-644435.