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Pulmonary Function Test Abnormalities in Children with Sickle Cell Anemia: A Cross-Sectional Study from a Tertiary
Sanjay Kumar Sahu1, Manas Ranjan Behera1, Nikhila P Gannavarapu1
1Pediatrics, Kalinga Institute of Medical Sciences, Bhubaneswar, IND.
Insights
Children with sickle cell disease (SCD) often have restrictive lung function abnormalities. Regular pulmonary monitoring and early intervention are crucial for managing these complications in pediatric SCD patients.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Sickle Cell Disease Research
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with potential systemic complications.
- Pulmonary function abnormalities are increasingly recognized in pediatric SCD.
- This study investigates lung function in children with SCD compared to those with other anemias.
Purpose of the Study:
- To assess and compare pulmonary function in children with sickle cell disease (SCD) versus a control group.
- To identify the prevalence of restrictive lung patterns in pediatric SCD.
- To explore potential risk factors associated with pulmonary abnormalities in SCD.
Main Methods:
- Cross-sectional study involving 126 children (63 SCD, 63 controls) aged 6-18 years.
- Pulmonary function tests (PFTs) using spirometry to measure FVC and FEV1.
- Collection of anthropometric, clinical, and laboratory data.
Main Results:
- Children with SCD exhibited significantly lower height and weight compared to controls.
- SCD patients showed lower mean FVC and FEV1, with a predominant restrictive pattern in 63.4% of cases.
- Older age, vaso-occlusive crises, acute chest syndrome, and blood transfusions were associated with restrictive abnormalities.
Conclusions:
- A high prevalence of restrictive lung function abnormalities exists in children with SCD.
- Regular pulmonary monitoring and early intervention are essential for managing SCD-related pulmonary complications.
- Further research is needed on hydroxyurea's impact and cumulative effects of vaso-occlusive events on lung function.
Background:
This cross-sectional study analyzed the pulmonary function in children with sickle cell disease (SCD), assessing the pulmonary abnormalities and comparing these with a control group of children with other forms of anemia.
Materials And Methodology:
This study was conducted from July 2022 to June 2024 at Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India, and included 126 children (63 with SCD, 63 with other forms of anemia) aged between six to 18 years. Anthropometric data, clinical history, and laboratory parameters were collected, and pulmonary function tests (PFTs) were performed using spirometry to evaluate forced vital capacity (FVC) and forced expiratory volume in one second (FEV1).
Results:
Children with SCD had lower height and weight than those in the control group, with height differences reaching statistical significance. Hemoglobin levels were higher in SCD cases than those in the control group, despite both groups exhibiting anemia. The PFTs revealed lower mean FVC and FEV1 in SCD patients, with a predominant restrictive pattern observed in 40 children with SCD (63.4%) compared to 25 children (39.6%) in controls. Only 17 children with SCD (29.6%) showed normal PFT results. Among risk factors, older age, history of vaso-occlusive crises, acute chest syndrome, and blood transfusions were linked to restrictive abnormalities, though not statistically significant. Children on hydroxyurea showed a trend toward restrictive PFT patterns.
Conclusion:
The study underscores the high prevalence of restrictive lung function abnormalities in children with SCD, highlighting the importance of regular pulmonary monitoring and early intervention to address pulmonary complications. Further research is warranted to explore the impact of hydroxyurea and the cumulative effect of vaso-occlusive events on lung function in SCD.
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