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Published ahead of print on March 23, 2006, doi:10.1164/rccm.200601-125OC

Am. J. Respir. Crit. Care Med., Volume 173, Number 11, June 2006, 1264-1269

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Submitted on January 27, 2006
Accepted on March 23, 2006

Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

Elizabeth S Klings1*, Diego F Wyszynski2, Vikki G Nolan2, and Martin H Steinberg3

1 The Pulmonary Center, Boston University School of Medicine, Boston, Massachusetts, USA; Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA; Boston Comprehensive Sickle Cell Center, Boston University School of Medicine, Boston, Massachusetts, USA, 2 Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA; School of Public Health, Boston University, Boston, Massachusetts, USA, 3 Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA; Boston Comprehensive Sickle Cell Center, Boston University School of Medicine, Boston, Massachusetts, USA

* To whom correspondence should be addressed. E-mail: eklings{at}lung.bumc.bu.edu.

Rationale: Pulmonary complications of sickle cell anemia (Hb-SS) commonly cause morbidity, yet few large studies of pulmonary function tests (PFTs) in this population have been reported. Objectives: PFTs [spirometry, lung volumes and diffusion capacity for carbon monoxide (DLCO)] from 310 Hb-SS adults were analyzed to determine the pattern of pulmonary dysfunction and their association with other systemic complications of sickle cell disease. Methods: Raw PFT data were compared with predicted values. Each subject was sub-classified into 1 of 5 groups: obstructive physiology; restrictive physiology; mixed obstructive/restrictive physiology; isolated low DLCO; or normal. The association of laboratory data between patients with a decreased DLCO or restrictive physiology compared with normals was assessed by multivariate linear regression. Measurements and Main Results: Normal PFTs were present in only 31 of 310 (10%) patients. Overall, adult Hb-SS was characterized by decreased total lung capacities (70.2 ± 14.7% predicted) and DLCO (64.5 ± 19.9 %). The most common PFT patterns were restrictive physiology (74%), and an isolated low DLCO (13%). Decreased DLCO was associated with thrombocytosis (p=0.05), and with hepatic [elevated ALT (p=0.07)] and a trend towards renal dysfunction [elevated BUN and creatinine (p=0.05, 0.07)]. Conclusions: Pulmonary function is abnormal in 90% of adult Hb-SS patients. Common abnormalities include restrictive physiology and a decreased DLCO. A decreased DLCO may indicate more severe sickle vasculopathy characterized by impaired hepatic and renal function
Key words: restrictive disease, dyspnea, airway obstruction




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