title: Imaging of pulmonary manifestations in subtype B of Niemann-Pick disease.
abstract: We report the radiological and high resolution CT (HRCT) findings of the pulmonary involvement in a case of subtype B Niemann-Pick disease, diagnosed in an asymptomatic 22-year-old man, which was proved by bone marrow biopsy and sphingomyelinase assessment. In the present case, HRCT showed smooth interlobular septa thickening and a subtle ground-glass pattern.
authors.affiliation: Department of Medical Imaging, Université Catholique de Louvain, Cliniques Universitaires Saint Luc, B-1200 Brussels, Belgium.
authors.forename: F
authors.initials: F
authors.lastname: Duchateau
authors.forename: S
authors.initials: S
authors.lastname: Dechambre
authors.forename: E
authors.initials: E
authors.lastname: Coche
mesh_terms: D000328:Adult; D001854:Bone Marrow Cells / Q000473:pathology; D004796:Clinical Enzyme Tests; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging*; D008297:Male; D009542:Niemann-Pick Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D013108:Sphingomyelin Phosphodiesterase / Q000172:deficiency; D014057:Tomography, X-Ray Computed
chemical_list: D013108:Sphingomyelin Phosphodiesterase