Abstract
Nine of 56 patients with mucopolysaccharidoses (MPS) showed small tracheal diameters on their frontal chest radiographs. Autopsy of an MPS I-H (Hurler disease) patient demonstrated that the small calibre was secondary to deposition of glycosaminoglycan (mucopolysaccharide). Autopsies of two patients with other storage diseases, one with geleophysic dysplasia and one with mucolipidosis II, also exhibited compromise of their airways because of storage material accumulation.
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Peters, M.E., Arya, S., Langer, L.O. et al. Narrow trachea in mucopolysaccharidoses. Pediatr Radiol 15, 225–228 (1985). https://doi.org/10.1007/BF02388760
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DOI: https://doi.org/10.1007/BF02388760