Table 2.
Studies (year) | Study Designs | Populations | Findings |
---|---|---|---|
Ivankovich et al39, 2019 | Cross sectional case-control | 22 infants with SCD, 37 healthy control infants aged 6–18 months | Normal lung function in 77% of infants with SCA but those w SCA had lower FVC, FEF0.5, FEF25–75 compared with controls |
Koumbourlis et al40, 1997 | Cross sectional | 20 infants with SCD aged 3–30 months (12 with HbSS) | Elevated FRC, decreased maximum expiratory flow at FRC, and increased time to maximum expired flow/total expiratory time, suggesting hyperinflation and lower airway obstruction |
Cohen et al41 2016 | Cross sectional and longitudinal cohort | 149 children aged 6–19 years with HbSS with spirometry and plethysmography; 139 had morbidity data from birth, 136 with prospective morbidity data (Sleep and Asthma Cohort Study) | 70% with normal lung function, 16%, 7%, and 6% with obstructive, restrictive, nonspecific, and mixed patterns, respectively. Lung function pattern not associated with prior or future pain or ACS episodes |
Arteta et al42, 2014 | Cross sectional | 146 children with HbSS or HbSβ0 (PUSH study) | 19% had obstruction (44% of whom had asthma), 9% had restriction, 11% had nonspecific abnormal lung function, and 19% had reduced DLCO |
MacLean et al46, 2008 | Single center longitudinal cohort study | 312 children with SCD aged 8–18 years | Average decline in FEV1: 2.93% per year for males, 2.95% per year for females; average decline in TLC: 2.15% per year for males, 2.43% per year for females |
Lunt et al47, 2016 | Longitudinal case-control study in 2 cohorts |
Cohort 1: 47 children with SCD, 26 controls; mean age 8.8 (range 3–14) years, followed up for 2 years Cohort 2: 45 children with SCD, 24 controls; mean age 10.2 (range 4–17) years, followed up for 10 years |
Cohort 1: At baseline: 34% with obstruction, 2% with restriction. FEV1 decline 1.45% per year Cohort 2 at baseline: 24% with obstruction, 11% with restriction. FEV1 decline 0.9% per year. |
Klings et al53, 2006 | Multicenter longitudinal cohort study | 310 adults with HbSS (Cooperative Study of SCD) | 74% had restriction, mean TLC 70% of predicted; 13% had isolated reduced DLCO. DLCO inversely associated with age. |
Field etal52, 2008 | Single center longitudinal cohort study | 92 adults with SCD, lung function testing obtained for clinical reasons | 35.7% with restriction, 18% with obstruction. FEV1 decreased by 49 cc per year |
Hodges et al51, 2022 | Single center longitudinal cohort study | 193 adults with SCD 309 adults with cystic fibrosis Age 18–65 years |
FEV1 declined 23 and 26 mL/year in those with SCD and cystic fibrosis, respectively, no difference between these two cohorts |
Abbreviations: ACS, acute chest syndrome; DLCO, diffusion capacity for carbon monoxide; FEF0.5, forced expiratory flow in 0; 5 seconds; FEF25–75, forced expiratory flow from the 25th to 75%ile of the maneuver; FEV1, forced expiratory volume in 1 second; FRC, functional residual capacity; FVC, forced vital capacity; SCA, sickle cell anemia; SCD, sickle cell disease; TLC, total lung capacity.