Correlations Between Lung, Cardiac Functions and Hematologic Parameters in SCA-Chidren

• Published in: Blood 2011-11, Vol.118 (21), p.1067-1067
• Main Authors: Tassel, Clément, Arnaud, Cécile, Kamdem, Annie, Médejel, Nadia, Fauveau, Elodie, Kulpa, Marc, Madhi, Fouad, Epaud, Ralph, Bernaudin, Francoise
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V118.21.1067.1067

Abstract 1067▪▪This icon denotes a clinically relevant abstract Elevation of tricuspid regurgitant jet velocity (TRJV) predicts high systolic pulmonary artery pressure and early mortality in adults with sickle cell anemia (SCA). To date, few studies have reported the concomitant analysis of lung alterations and high TRJV in SCA-children. To evaluate the relationship between lung function, TRJV and hematologic parameters in SCA-children. SCA-children of the Creteil-CHIC cohort were assessed at steady state on the same day by cardiac echocardiography, pulmonary function tests (PFT), clinical and biological parameters. All data were recorded in the CHIC database. Forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1), total lung capacity (TLC) and diffusing capacity for carbon monoxide (DLCO) were measured with the single-breath technique. DLCO was adjusted to hemoglobin and to the alveolar volume (KCOc). Univariate and multivariate linear regression analyses were performed to evaluate factors correlating with TRJV and KCOc and logistic regression was applied to evaluate the risk factors associated with elevated TRJV (≥ 2.5 m/s). A total of 228 check-ups with cardiac and lung assessment, performed in 163 SCA-patients (160 SS, 3 SB0) was analyzed. Check-ups were performed at the median age of 13.2 years (range: 5.7–19.9). Among the 228 check-ups, 151 were performed after intensification (59 on hydroxyurea, 57 on transfusion program and 38 after hematopoietic stem cell transplantation (HSCT). TRJV (median value: 2.2 m/s, range 1.4–3.1) was significantly positively correlated with age (r=0.164, p=0.013), was not correlated with TLC but was significantly and negatively correlated with FVC before b2 (r=−0.145, p=0.03) and after b2 (r=−0.184, p=0.008), FEV1 before b2 (r=−0.213, p=0.001) and after b2 (r=−0.178, p=0.012), FEV1/FVC before b2 (r=23?0.165, p=0.013) and positively correlated with KCOc (r=0.379, p