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Usual Interstitial Pneumonia Complicating Dyskeratosis Congenita

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Presentation on theme: "Usual Interstitial Pneumonia Complicating Dyskeratosis Congenita"— Presentation transcript:

1 Usual Interstitial Pneumonia Complicating Dyskeratosis Congenita
James P. Utz, MD, Jay H. Ryu, MD, Jeffrey L. Myers, MD, Virginia V. Michels, MD  Mayo Clinic Proceedings  Volume 80, Issue 6, Pages (June 2005) DOI: / Copyright © 2005 Mayo Foundation for Medical Education and Research Terms and Conditions

2 FIGURE 1 Case 1. Dyskeratotic nails on the second through fifth digits of the right hand. Mayo Clinic Proceedings  , DOI: ( / ) Copyright © 2005 Mayo Foundation for Medical Education and Research Terms and Conditions

3 FIGURE 2 Case 1. Bilateral subpleural reticular changes in the upper and lower lung fields with early subpleural honeycombing and traction bronchiectasis compatible with usual interstitial pneumonia. Mayo Clinic Proceedings  , DOI: ( / ) Copyright © 2005 Mayo Foundation for Medical Education and Research Terms and Conditions

4 FIGURE 3 Case 1. Surgical lung biopsy specimen shows usual interstitial pneumonia. Left, Low-magnification photomicrograph reveals patchy chronic interstitial pneumonia dominated by peripheral and paraseptal collagen deposition with scarring and early honeycomb changes (hematoxylin-eosin, original magnification ×20). Right, Low-magnification photomicrograph shows area of scarring with honeycomb changes and fibroblast focus (arrows) typical of usual interstitial pneumonia (hematoxylin-eosin, original magnification ×40). Mayo Clinic Proceedings  , DOI: ( / ) Copyright © 2005 Mayo Foundation for Medical Education and Research Terms and Conditions

5 FIGURE 4 Case 2. High-resolution computed tomograms show bilateral reticular changes in the upper and lower lung fields with early peripheral honeycombing, traction bronchiectasis, and patchy ground-glass changes compatible with usual interstitial pneumonia. Mayo Clinic Proceedings  , DOI: ( / ) Copyright © 2005 Mayo Foundation for Medical Education and Research Terms and Conditions

6 FIGURE 5 Case 2. Surgical lung biopsy specimen shows usual interstitial pneumonia. Left, Low-magnification photomicrograph illustrates chronic interstitial pneumonia characterized by a combination of inflammation and fibrosis. Fibrosis predominates and is distributed in a patchy, predominantly subpleural pattern characteristic of usual interstitial pneumonia (hematoxylin-eosin, original magnification ×20). Right, At higher magnification, a combination of fibrosis, smooth muscle proliferation, and fibroblast foci (arrows) typical of usual interstitial pneumonia is evident (hematoxylineosin, original magnification ×40). Mayo Clinic Proceedings  , DOI: ( / ) Copyright © 2005 Mayo Foundation for Medical Education and Research Terms and Conditions


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