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British Journal of Radiology (2003) 76, 838-840
© 2003 British Institute of Radiology
doi: 10.1259/bjr/33503694
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Pulmonary involvement in an adult male affected by type B Niemann-Pick disease

E González-Reimers, MD 1 M J Sánchez-Pérez, MD 1 A Bonilla-Arjona, MD 2 M Rodríguez-Gaspar, MD 1 J L Carrasco-Juan, MD 3 H Alvarez-Argüelles, MD 3 and F Santolaria-Fernández, MD 1

1 Departments of Internal Medicine, 2 Radiology and 3 Pathology, Hospital Universitario, Tenerife, Canary Islands, Spain



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  		Figure 1. Plain radiograph of the thorax, showing a mainly bibasal reticulonodular infiltrate.

 


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  		Figure 2. Detailed image of the reticulonodular infiltrate affecting the lower fields of the right lung.

 


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  		Figure 3. High resolution CT thoracic scan, with a ground glass pattern accompanied by septal and peribronchovascular interstitial thickening in the lung bases.

 


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  		Figure 4. Lung biopsy specimen, showing (a) massive infiltration of alveoli by foamy histiocytes (HE, x 40), (b) massive infiltration by histiocytes which stain sea-blue (arrows) with May-Grünwald Giemsa ( x 200), (c) moderate fibrosis (arrows) of alveolar walls and interlobular septae (Masson-Goldner, x 100) and (d) ultrastructural examination, showing (left) the sea blue histiocytes with numerous dense granules (arrows), which correspond to secondary lysosomes with abundant myelin figures (right).

 




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