Lung cyst: an unusual manifestation of Niemann-Pick 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.
Baldi
Bruno G
BG
Pulmonary Division, University of Sao Paulo Medical School, Sao Paulo, Brazil. bruno.guedes2@terra.com.br
Santana
Alfredo N C
AN
Takagaki
Teresa Y
TY
Fujita
Carmem
C
Kairalla
Ronaldo A
RA
Carvalho
Carlos R R
CR
D000328:Adult; D003560:Cysts / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D008297:Male; D052537:Niemann-Pick Disease, Type B / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed