Premium
Lung cyst: An unusual manifestation of Niemann–Pick disease
Author(s) -
BALDI Bruno G.,
SANTANA Alfredo N.C.,
TAKAGAKI Teresa Y.,
FUJITA Carmem,
KAIRALLA Ronaldo A.,
CARVALHO Carlos R.R.
Publication year - 2009
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/j.1440-1843.2008.01352.x
Subject(s) - medicine , niemann–pick disease , pathology , lung , bone marrow , cyst , spleen , radiology , disease
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.