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Updated: Oct 20, 2025

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Variation in pulmonary function tests among children with sickle cell anemia: a systematic review and meta-analysis
Amar Taksande1, Patel Zeeshan Jameel1, Divya Pujari2
1Department of Paediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Sawangi Meghe, Wardha, Maharashtra State, India.
Insights
Children with sickle cell anemia (SCA) show reduced lung function, specifically lower forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC). Routine pulmonary function tests (PFTs) are recommended for early detection of lung decline in SCA patients.
Area of Science:
- Pulmonary Medicine
- Pediatrics
- Hematology
Background:
- Sickle cell anemia (SCA) is associated with various pulmonary complications, including acute chest syndrome (ACS), pulmonary hypertension (PH), and airway hyper-responsiveness (AHR).
- Pulmonary function abnormalities are common in children with SCA, necessitating further investigation.
Purpose of the Study:
- To examine pulmonary function test (PFT) abnormalities in children diagnosed with sickle cell anemia (SCA).
- To assess the impact of SCA on lung function parameters in pediatric patients.
Main Methods:
- A systematic review and meta-analysis of case-control studies was conducted.
- Electronic databases (Cochrane library, PubMed, EMBASE, Scopus, Web of Science) were searched for relevant studies.
- Pulmonary functions were assessed using spirometry, lung volume, and gas diffusion findings in 788 SCA children and 1101 controls.
Main Results:
- Nine studies were analyzed, involving 788 children with SCA and 1101 controls.
- Significant decreases were observed in forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) in children with SCA compared to controls.
- Other assessed parameters like FEV1/FVC ratio, peak expiratory flow rate (PEFR), total lung capacity (TLC), and carbon monoxide diffusing capacity (DLCO) showed non-significant trends towards reduction.
Conclusions:
- Sickle cell anemia is associated with significantly reduced FEV1 and FVC in children.
- These findings support the importance of routine pulmonary function monitoring for early detection of lung function decline in pediatric SCA patients, particularly those with a history of ACS.
Introduction:
the spectrum of pulmonary complications in sickle cell anemia (SCA) comprises mainly of acute chest syndrome (ACS), pulmonary hypertension (PH) and airway hyper-responsiveness (AHR). This study was conducted to examine the abnormalities in pulmonary function tests (PFTs) seen in children with SCA.
Methods:
electronic databases (Cochrane library, PubMed, EMBASE, Scopus, Web of Science) were used as data sources. Two authors independently reviewed studies. All case-control studies with PFT performed in patients with SCA and normal controls were reviewed. Pulmonary functions were assessed with the help of spirometry, lung volume and gas diffusion findings.
Results:
nine studies with 788 SCA children and 1101 controls were analyzed. For all studies, the pooled mean difference for forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, peak expiratory flow rate (PEFR), total lung capacity (TLC) and carbon mono-oxide diffusing capacity (DLCO) were -12.67, (95% CI: -15.41,-9.94), -11.69, (95% CI: -14.24, -9.14), -1.90, (95% CI: -4.32, 0.52), -3.36 (95% CI: -6.69, -0.02), -7.35, (95% CI: -14.97, -0.27) and -4.68, (95% CI -20.64, -11.29) respectively. FEV1 and FVC and were the only parameters found to be significantly decreased.
Conclusion:
sickle cell anemia was associated with lower FEV1 and FVC, thus, supporting the role of routine monitoring for the progression of lung function decline in children with SCA with ACS. We recommend routine screening and lung function monitoring for early recognition of pulmonary function decline.
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