Knight-Madden, Jennifer M.; Lewis, N.A.; Forrester, Terrence E.; Greenough, Anne
Author Affiliation, Ana.
Tropical Medicine Research Institute
A history of acute chest syndrome is associated with decreased pulmonary function in young adults with sickle cell disease
West Indian Medical Journal
Date of Publication
51 (Supplement 2)
Also appears in the Proceedings of the Caribbean Health Research Council's 47th Annual Council and Scientific Meeting. Cara Inn Hotel, Guyana. April 24-27, 2002. Edited by Edward N. Barton.
Objective: To determine whether a history of acute chest syndrome (ACS) and / or asthma decreases the forced respiratory flow in one second (FEVI) in patients with sickle cell diseases. Design and Methods: Eighty patients with sickle cell disease (haemoglobin SS) were randomly selected from the Jamaican Sickle Cell Cohort Study. All respiratory episodes recorded in their charts were coded as acute chest syndrome, asthma episode or other. Eighty AA controls for the study were recruited for comparison. Weight, standing and sitting heights and arms span were measured in all subjects. Spirometry was performed according to European Respiratory Standard. The correlation between anthropometric variables and FEVI was determined. Univariate general linear models were fit for the FEVI with independent variables and the impact off adding number of episodes of ACS and number of episodes of acute bronchospasm determined. Results: All anthropometric variables except height were greater in controls with haemoglobin AA. Correlation of FEVI was highest with arm span (0.703), sitting height (0.677) and height (0.683). A model with all subjects with gender, genotype, and anthropometric variables had an adjusted R2 of 0.654, and in patients with HbSS disease only, an adjusted R2 of 0.588 with the only significant variables being gender (p= 0.003). Addition of the respiratory variables increased the adjusted R@ to 0.675 and the variable which remained significant was ACS (p=0.000). Conclusion: A history of ACS, but not episodes of bronchospasm, is associated with a lower FEVI.....