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06 August 2020 : Case report  Poland

Combined Emphysema and Interstitial Lung Disease as a Rare Presentation of Pulmonary Involvement in a Patient with Chronic Visceral Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B)

Rare disease

Lucyna Opoka1ABDE*, Dorota Wyrostkiewicz2ABDE, Piotr Radwan-Rohrenschef2ABD, Adriana Roży3ABD, Anna Tylki-Szymańska4ABDE, Witold Tomkowski2ABDG, Monika Szturmowicz2ABDEFG

DOI: 10.12659/AJCR.923394

Am J Case Rep 2020; 21:e923394

Figure 2. (A) High-resolution computed tomography (HRCT) of the lungs, axial plane. Paraseptal emphysema (red arrows) with interlobular septal thickening (blue arrows) predominantly in the upper lobes. (B) HRCT, axial plane. Areas of ground-glass opacities and lung fibrosis with reticulation (yellow arrows) and bronchiectasis (green arrows) predominantly in the middle and lower parts of both lungs. (C) HRCT, coronal reconstruction paraseptal emphysema (red arrow) with interlobular septal thickening (blue arrow) predominantly in the upper lobes. Areas of ground-glass opacities and lung fibrosis with reticulation (yellow arrow) and bronchiectasis (green arrow) predominantly in the middle and lower parts of both lungs. No mediastinal or hilar lymphadenopathies are observed.

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923