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Case Reports
. 2009 Jan;14(1):134-6.
doi: 10.1111/j.1440-1843.2008.01352.x.

Lung cyst: an unusual manifestation of Niemann-Pick disease

Bruno G Baldi  1 , Alfredo N C Santana, Teresa Y Takagaki, Carmem Fujita, Ronaldo A Kairalla, Carlos R R Carvalho
Affiliations
Case Reports

Lung cyst: an unusual manifestation of Niemann-Pick disease

Bruno G Baldi et al. Respirology. 2009 Jan.

Abstract

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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