Pulmonary functions in children and adolescents with sickle cell disease
- 28 May 2020
- journal article
- research article
- Published by Wiley in Pediatric Pulmonology
- Vol. 55 (8), 2055-2063
- https://doi.org/10.1002/ppul.24871
Abstract
Background Sickle cell disease (SCD) is relatively common in Bahrain, and airway inflammation in patients with SCD is usually multifactorial. This study aimed to evaluate lung function and induced sputum levels of interleukin-6 (IL-6) in Bahraini children and adolescents with SCD and assess their relationship with the recurrence of acute chest syndrome (ACS). Methods A total of 139 children and adolescents with SCD and 123 healthy children (control group) were included in the present study. Patients were further stratified according to age and history of ACS. The patient and control groups underwent pulmonary function tests (PFTs), including spirometry and assessments of lung volume, diffusion of carbon monoxide (DLCO), and induced sputum IL-6 levels. Results Forced expiratory volume in 1 second (FEV1), force vital capacity (FVC), FEV1/FVC, total lung capacity, DLCO, and DLCOc (ie, hemoglobin-corrected DLCO) were significantly lower, while residual volume and sputum IL-6 levels were significantly higher in the patient group than in the control group. PFT parameters were more compromised in the patient subgroup with a history of ACS and older than 12 years compared with the subgroup without a history of ACS and the subgroup under 12 years of age. PFTs revealed significant negative correlations with age, number of ACS events, and sputum IL-6 levels. Conclusion Pulmonary function was observed to worsen with disease progression, and it worsened with older age and repeated occurrence of ACS. Induced sputum IL-6 levels reflected the degree of lung inflammation in affected patients and were associated with more impairment in various PFT parameters.Keywords
This publication has 29 references indexed in Scilit:
- Induced Sputum Substance P in Children with Difficult-to-Treat Bronchial Asthma and Gastroesophageal Reflux: Effect of Esomeprazole TherapyInternational Journal of Pediatrics, 2011
- A Hemodynamic Study of Pulmonary Hypertension in Sickle Cell DiseaseThe New England Journal of Medicine, 2011
- The Impact of Recurrent Acute Chest Syndrome on the Lung Function of Young Adults with Sickle Cell DiseaseLung, 2010
- Protective effects of phosphodiesterase‐4 (PDE‐4) inhibition in the early phase of pulmonary arterial hypertension in transgenic sickle cell miceThe FASEB Journal, 2008
- Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell diseasePediatric Blood & Cancer, 2006
- Interpretative strategies for lung function testsEuropean Respiratory Journal, 2005
- Campaign to Control Genetic Blood Diseases in BahrainPublic Health Genomics, 2005
- Pulmonary function studies in Kuwaiti children with sickle cell disease and elevated Hb FInternational Journal of Clinical Practice, 2004
- Circulating Activated Endothelial Cells in Sickle Cell AnemiaThe New England Journal of Medicine, 1997
- Pulmonary function abnormalities in childhood sickle cell diseaseThe Journal of Pediatrics, 1993