title: Sub-pleural bullous changes in two adults with Mucopolysaccharidosis type I (Hurler-Scheie).
abstract: Two unrelated adult patients with MPS I (Hurler-Scheie) demonstrated sub-pleural bullous emphysema. This complication of MPS I should be looked for due to the risk of spontaneous pneumothorax.
authors.affiliation: Department of Genetic Medicine, Westmead Hospital, Westmead, NSW, Australia. michelt@chw.edu.au
authors.forename: Michel C
authors.initials: MC
authors.lastname: Tchan
authors.forename: Nicole
authors.initials: N
authors.lastname: Graf
authors.forename: David O
authors.initials: DO
authors.lastname: Sillence
mesh_terms: D000328:Adult; D004549:Elastin / Q000378:metabolism; D004646:Emphysema / Q000175:diagnosis / Q000000981:diagnostic imaging; D056947:Enzyme Replacement Therapy / Q000379:methods; D006801:Humans; D008059:Mucopolysaccharidosis I / Q000175:diagnosis / Q000473:pathology*; D010641:Phenotype; D010994:Pleura / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D004549:Elastin