title: Niemann-Pick disease: high-resolution CT findings in two siblings.
abstract: Niemann-Pick disease is a rare inherited disorder characterized by deposition of sphingomyelin in various organs. We describe the high-resolution computed tomography findings in 2 adult sisters with Niemann-Pick disease and extensive pulmonary involvement. The main abnormalities consisted of thickening of the interlobular septa and patchy areas of ground-glass attenuation.
authors.affiliation: Hospital Universitario Clementino Fraga Filho, Rio de Janeiro, Brazil.
authors.forename: Rosana
authors.initials: R
authors.lastname: Rodrigues
authors.forename: Edson
authors.initials: E
authors.lastname: Marchiori
authors.forename: Nestor L
authors.initials: NL
authors.lastname: Müller
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging*; D008171:Lung Diseases / Q000150:complications / Q000000981:diagnostic imaging*; D009542:Niemann-Pick Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics; D014057:Tomography, X-Ray Computed