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Niemann-Pick disease: high-resolution CT findings in two siblings.

作者信息

Rodrigues Rosana, Marchiori Edson, Müller Nestor L

机构信息

Hospital Universitario Clementino Fraga Filho, Rio de Janeiro, Brazil.

出版信息

J Comput Assist Tomogr. 2004 Jan-Feb;28(1):52-4. doi: 10.1097/00004728-200401000-00008.

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.

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