Tracheobronchial Involvement of Mucopolysaccharidosis Type VI
A 20-year-old male with Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome) presented with severe mitral valve stenosis, obstructive sleep apnea, and progressive respiratory compromise due to extensive tracheobronchial involvement. Chest CT revealed diffuse tracheal-bronchial wall thickening, fixed tracheal angulation, and significant airway narrowing consistent with glycosaminoglycan accumulation characteristic of MPS VI.
Authors
- Furkan Ufuk (ORCID: https://orcid.org/0000-0002-8614-5387)
Institutions
- University of Chicago (US)
Publication Details
- Journal
- Pediatric Pulmonology
- Published
- 2025-02-01
- DOI
- https://doi.org/10.1002/ppul.71007
- Primary Topic
- Lysosomal Storage Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00