Niemann-Pick disease: high-resolution CT findings in two siblings.
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.
Rodrigues
Rosana
R
Hospital Universitario Clementino Fraga Filho, Rio de Janeiro, Brazil.
Marchiori
Edson
E
Müller
Nestor L
NL
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