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Updated: Jun 17, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Prevalence, patterns, and factors associated with abnormal lung function among children with sickle cell disease in
Pamella Mwa Aol1, Geriga Fahdil2, Felix Bongomin3
1Department of Paediatrics and Child Health, St. Mary's Hospital Lacor, Gulu, Uganda.
Insights
Over one-third of children with sickle cell disease (SCD) in Uganda exhibit abnormal lung function, including restrictive and obstructive patterns. Factors like high LDH levels and charcoal smoke exposure are associated with these respiratory issues.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Public Health
Background:
- Pulmonary complications are frequent in children with sickle cell disease (SCD).
- Limited data exists on lung function abnormalities in Ugandan children with SCD.
- This study addresses the gap in understanding respiratory health in this population.
Purpose of the Study:
- To determine the prevalence of abnormal lung function in Ugandan children with SCD.
- To identify patterns of lung function abnormalities.
- To investigate factors associated with impaired lung function in this cohort.
Main Methods:
- A cross-sectional study was conducted at a tertiary care hospital in Uganda.
- Spirometry was performed on 332 children aged 6-18 years with SCD.
- Multivariable regression analysis identified factors linked to abnormal lung function.
Main Results:
- 37.9% of participants had abnormal lung function, with restrictive and obstructive patterns being most common.
- Elevated serum lactate dehydrogenase (LDH) levels, history of acute chest syndrome, wasting, and charcoal smoke exposure were associated with abnormal lung function.
- Specific statistical associations were reported for each identified factor.
Conclusions:
- A significant proportion of children with SCD in Uganda experience lung function deficits.
- Interventions focusing on nutrition, reducing exposure to indoor air pollution (charcoal smoke), and monitoring LDH levels are recommended.
- Targeted strategies are needed to manage reversible and irreversible airway obstruction in children with SCD.
Background:
Pulmonary complications are common among children with sickle cell disease (SCD). However, there is little literature on associated lung function abnormalities in Uganda. We aimed to determine the prevalence, patterns, and factors associated with abnormal lung function among children with SCD in a tertiary care hospital in Uganda.
Method:
A cross-sectional study was conducted among children aged 6 to 18 years at the SCD clinic (SCC) of Mulago National Super-Specialized Hospital between January 2020 and April 2021. Data on sociodemographic and clinical characteristics was collected using a standardized questionnaire. Laboratory investigations, including a complete blood count and serum lactate dehydrogenase (LDH), were done. Spirometry was performed following the ATS/ERS standards. Multivariable modified Poisson regression analysis was performed to determine factors associated with abnormal lung function.
Results:
A total of 332 participants were enrolled. The mean age was 11.7 ± 3.4 years, and 184 (55.4%) were female. Overall, 126 (37.9%) participants had abnormal lung function: 67/126 (53.2%) restrictive, 57/126 (45.2%) obstructive, and 2/126 (1.6%) mixed-ventilatory patterns. Factors associated with abnormal lung function were; serum LDH level > 600UL (aIRR: 1.89 95% CI: 1.2 - 7.4, p = 0.049), a history of acute chest syndrome (aIRR: 1.55, 95% CI: 1.06-2.25, p = 0.024), wasting (aIRR: 1.33, 95%CI: 1.02 - 1.72, p = 0.032), and use of charcoal for household cooking (aIRR: 1.49, 95% CI: 1.03-2.15, p = 0.035).
Conclusion:
More than one-third of children with SCD in Uganda have lung function abnormalities. Strategies to improve nutrition, reduce exposure to charcoal smoke, and monitoring serum LDH levels may be important in preventing or managing abnormal lung function in this population. The identification of reversible and irreversible airway obstruction in children with sickle cell disease also highlights the need for targeted interventions to address these specific patterns of abnormal lung function.
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