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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Longitudinal changes in lung function and somatic growth in children with sickle cell disease
Anastassios C Koumbourlis1, Donna J Lee, Ada Lee
1Division of Pediatric Pulmonology, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center, New York, NY, USA. Akoumbou@LIJ.edu
Pediatric Pulmonology
|May 1, 2007
Summary
Children with sickle cell disease (SCD) show worsening lung function over time, with an increase in obstructive patterns. Somatic growth did not appear to directly impact lung function changes in this study.
Area of Science:
- Pulmonology
- Pediatrics
- Hematology
Background:
- Sickle cell disease (SCD) significantly impacts multiple organ systems, including the lungs.
- Longitudinal studies are crucial to understand the progression of lung function changes in pediatric SCD patients.
Purpose of the Study:
- To investigate the longitudinal changes in lung function and somatic growth patterns in children and adolescents with hemoglobin SS (Hb-SS) sickle cell disease (SCD).
Main Methods:
- 45 children with Hb-SS SCD underwent two lung function and somatic growth assessments, separated by approximately 42 months.
- Spirometry was used to evaluate lung volumes and airflow indices, including FEV1, FVC, and FEF25-75.
- Body Mass Index (BMI) was assessed to categorize somatic growth patterns.
Main Results:
- Lung volumes slightly decreased but remained borderline normal; however, significant declines in spirometric indices indicated developing lower airway obstruction (P<0.001).
- The prevalence of obstructive lung function patterns increased from 22% to 44%, while normal patterns decreased from 56% to 29% over time.
- Abnormal BMI (high or low) was associated with abnormal lung function, but only normal BMI patients exhibited lung function decline.
Conclusions:
- Sickle cell disease is associated with a progressive obstructive pattern of lung function in children and adolescents.
- No direct causal relationship was identified between somatic growth patterns and the observed changes in lung function in this cohort.
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