Related Experiment Videos
Airway changes in children with mucopolysaccharidoses
Shin-Lin Shih1, Y-J Lee, S-P Lin
1Department of Radiology, Mackay Memorial Hospital, 92 Section 2, Chung San North Road, Taipei, Taiwan.
Acta Radiologica (Stockholm, Sweden : 1987)
|April 26, 2002
Summary
Children with mucopolysaccharidoses (MPS) exhibit significant airway abnormalities, including vocal cord and trachea shape changes. Their tracheal surface area (TSA) is notably smaller compared to healthy children, indicating airway narrowing.
Area of Science:
- Medical Imaging
- Pediatric Pulmonology
- Genetics
Background:
- Mucopolysaccharidoses (MPS) are a group of rare genetic disorders characterized by the accumulation of glycosaminoglycans in various tissues.
- Airway compromise is a recognized complication of MPS, impacting respiratory function and quality of life.
Purpose of the Study:
- To evaluate the specific computed tomography (CT) findings of the airway in pediatric patients diagnosed with mucopolysaccharidoses (MPS).
Main Methods:
- A cohort of 13 pediatric patients with various types of MPS underwent airway CT scans.
- Airway morphology, including vocal cord and tracheal shape, was assessed.
- Tracheal surface area (TSA) at the T1 level was measured and compared to age-matched controls.
Main Results:
- CT revealed vocal cord abnormalities in 7 of 13 patients, with common shapes including elliptical and star-shaped.
- Tracheal abnormalities were observed in 8 of 13 patients, frequently presenting as U-shaped or worm-shaped.
- Patients with MPS demonstrated a significantly smaller TSA compared to control subjects, with reductions evident across different age groups.
Conclusions:
- Pediatric patients with MPS exhibit distinct and significant alterations in vocal cord and tracheal morphology.
- The U-shaped trachea was the most prevalent abnormal configuration identified.
- Reduced TSA in MPS patients suggests airway narrowing, potentially linked to submucosal storage of substances like keratan or dermatan sulfate.