Journal
RESPIROLOGY
Volume 14, Issue 1, Pages 134-136Publisher
WILEY-BLACKWELL PUBLISHING, INC
DOI: 10.1111/j.1440-1843.2008.01352.x
Keywords
chest CT; cyst; ground-glass opacity; Niemann-Pick disease; subtype B
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Niemann-Pick disease is a rare inherited autosomal recessive disorder, currently classified into six subtypes and characterized by the intracellular accumulation of sphingomyelin in the liver, spleen, lungs, bone marrow or brain. The main pulmonary abnormalities described in high-resolution computed tomography (HRCT) of the chest consist of thickening of the interlobular septa and ground-glass opacities. This case report describes a patient with subtype B Niemann-Pick disease characterized by cysts and ground-glass opacities that were detected on HRCT of the chest.
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