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Imaging of pulmonary manifestations in subtype B of Niemann-Pick disease.

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Duchateau, F [UCL] Dechambre, S [UCL] Coche, Edgard [UCL]

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.

Document type Article de périodique (Journal article) – Case Reports, Journal Article
Access type Accès restreint
Publication date 2001
Language Anglais
Journal information "The British journal of radiology" - Vol. 74, no. 887, p. 1059-61 (2001)
Peer reviewed yes
issn 0007-1285
Publication status Publié
Affiliations UCL - (SLuc) Département d'imagerie médicale
UCL - MD/RAIM - Département de radiologie et d'imagerie médicale
MESH Subject Adult ; Bone Marrow Cells - pathology ; Clinical Enzyme Tests ; Histiocytes - pathology ; Humans ; Lung Diseases - radiography ; Male ; Niemann-Pick Diseases - pathology - radiography ; Sphingomyelin Phosphodiesterase - deficiency ; Tomography, X-Ray Computed
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Bibliographic reference Duchateau, F ; Dechambre, S ; Coche, Edgard. Imaging of pulmonary manifestations in subtype B of Niemann-Pick disease.. In: The British journal of radiology, Vol. 74, no. 887, p. 1059-61 (2001)
Permanent URL http://hdl.handle.net/2078.1/8991