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Comment
. 2016 Mar 15;193(6):694-6.
doi: 10.1164/rccm.201509-1809LE.

Lipofibroblast Phenotype in Pulmonary Interstitial Glycogenosis

Gail H Deutsch  1 , Lisa R Young  2
Affiliations
Comment

Lipofibroblast Phenotype in Pulmonary Interstitial Glycogenosis

Gail H Deutsch et al. Am J Respir Crit Care Med. .
No abstract available

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Figures

Figure 1.
Figure 1.
Lipofibroblast phenotype in pulmonary interstitial glycogenosis. Characteristic spindle cells expand the alveolar interstitium in the biopsy from case 4, which also showed poor lung growth and hypertensive change of the pulmonary arteries (A). Transmission electron microscopy of interstitial cells in the alveolar wall from case 3 (B, original magnification, ×8,500) and case 2 (C, original magnification ×18,200), showing some of these cells contain lipid bodies (arrows), as well as dispersed cytoplasmic glycogen. Oil-red-O staining (D) demonstrates lipid droplets of variable size in scattered alveolar interstitial cells; cytokeratin (Dako M3515) immunostaining denotes epithelial cells. (E and F) Some interstitial cells in pulmonary interstitial glycogenosis express adipocyte differentiation–related protein (Fitzgerald 10R-A117ax; E developed with 3,3′-diaminobenzidine; F developed with fluorescein isothiocyanate-green), which colocalizes with vimentin (red; Dako M7020; nuclei in blue, 4′,6-diamidino-2-phenylindole counterstain). No significant oil-red-O positive or adipocyte differentiation-related protein immunopositive interstitial cells were seen within lung biopsies from age-matched patients without histologic findings of pulmonary interstitial glycogenosis (data not shown).
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