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Pulmonary Function Testing in Children with Restrictive Chest Wall Disorders
Gregory J Redding1, Jean-Paul Praud2, Oscar Henry Mayer3
1Pulmonary and Sleep Medicine Division, Department of Pediatrics, University of Washington School of Medicine, Seattle Children's Hospital, Seattle, Washington.
Insights
Chest wall, spine, and muscle disorders cause restrictive lung disease. Spirometry monitors changes, while breathing tests offer insights for new therapies.
Area of Science:
- Pulmonary Medicine
- Respiratory Physiology
Background:
- Thoracic abnormalities, including respiratory muscle weakness and spinal/thoracic growth issues like scoliosis, can cause restrictive lung disease.
- Obesity typically leads to obstructive, not restrictive, lung disease in children.
Purpose of the Study:
- To summarize the impact of chest wall, spine, and respiratory muscle disorders on lung function.
- To differentiate restrictive lung disease causes from obesity-related respiratory issues.
- To highlight the role of advanced respiratory function testing in understanding these conditions.
Main Methods:
- Review of conditions affecting the chest wall, spine, and respiratory muscles.
- Analysis of spirometry's utility in diagnosing and monitoring restrictive lung disease.
- Discussion of specialized tests including respiratory muscle function, sleep, and exercise.
Main Results:
- Disorders of the chest wall, spine, and respiratory muscles are primary causes of restrictive lung disease.
- Spirometry is insufficient for diagnosis but valuable for monitoring disease progression and treatment response.
- Obesity is more commonly associated with obstructive lung disease in children.
Conclusions:
- Understanding the pathophysiology of restrictive lung disease requires comprehensive respiratory function testing.
- Specialized tests provide critical insights into conditions affecting lung mechanics.
- These insights may pave the way for novel therapeutic strategies for restrictive lung disease.
Abstract:
Disorders of the chest wall, spine, and respiratory muscles lead to lung function abnormalities consistent with restrictive lung disease. Common disorders of the thorax include diseases with respiratory muscle weakness, growth abnormalities of the thorax and spine, such as scoliosis. Spirometry alone is not sufficient to diagnose these abnormalities but may be useful to serially monitor changes over time or in response to treatments. In contrast, obesity does not produce restrictive respiratory disease in most children and produces obstructive lung disease more often. Test of respiratory muscle function, breathing during sleep, and during exercise have led to additional insights about the pathophysiology of these conditions that may provide new avenues for future therapies.
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