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. 2014 May 28;14:91. doi: 10.1186/1471-2466-14-91

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Copyright © 2014 Enomoto et al.; licensee BioMed Central Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Heterogeneous deposition of elastic fibers in the lungs of patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE). Lung specimens were obtained from the both upper lobe and lower lobes in five of the six patients with IPPFE. Elastic fiber scores (EF scores) in each lobe are shown (A). EF scores in lower lobes were significantly lower than those in the upper lobes in patients with IPPFE (median, 24.2% [range 20.3-26.5%] vs. 32.4% [range 24.9-39.6%], p = 0.048). Values in the same patient are connected by solid lines. The horizontal bar represents the median. Even in the lower lobes, the EF scores in patients with IPPFE were still higher than those in patients with IPF (C, p < 0.0001) as well as in the upper lobes (B, p = 0.0007).