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Niemann-Pick disease: high-resolution CT findings in two siblings
Rosana Rodrigues1, Edson Marchiori, Nestor L Müller
1Hospital Universitario Clementino Fraga Filho, Rio de Janeiro, Brazil.
Journal of Computer Assisted Tomography
|January 13, 2004
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.