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Down syndrome: patterns of disturbed lung growth
B L Schloo1, G F Vawter, L M Reid
1Department of Pathology, Harvard Medical School, Children's Hospital, Boston, MA 02115.
Human Pathology
|September 1, 1991
Summary
Lung development in Down syndrome (DS) shows reduced airway branching and altered alveolar growth. These findings suggest prenatal developmental disruptions in DS pulmonary development.
Area of Science:
- Pulmonology
- Developmental Biology
- Genetics
Background:
- Down syndrome (DS) is associated with abnormal pulmonary development, including reduced alveolar complexity.
- Previous studies have indicated potential lung growth disturbances in individuals with DS.
Purpose of the Study:
- To investigate the detailed patterns of pulmonary development in patients with Down syndrome.
- To identify and quantify specific abnormalities in airway branching and alveolar development.
Main Methods:
- Counting axial branches of intrasegmental airways in 13 Down syndrome patients.
- Evaluating radial alveolar counts in 11 lungs from Down syndrome patients.
- Comparing observed airway generation and alveolar counts against predicted or expected values.
Main Results:
- Nine out of 13 Down syndrome patients exhibited a reduction of 25% or more in airway generation.
- Radial alveolar counts varied: five lungs showed counts 143%-162% above expected, five were as expected, and one was below expected (82%).
- No significant correlation was found between the number of airways and radial alveolar count.
Conclusions:
- Reduced airway branching in Down syndrome lungs suggests prenatal developmental interference.
- Disturbances in alveolar multiplication are also evident, indicating complex lung growth abnormalities.
- These findings highlight specific patterns of disturbed lung growth in Down syndrome that warrant further investigation.
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