{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T13:56:11Z","timestamp":1760622971563},"reference-count":5,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2015,12,9]],"date-time":"2015-12-09T00:00:00Z","timestamp":1449619200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Japanese Ministry of Health, Labour and Welfare"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2016,3]]},"abstract":"Abstract<\/jats:title>A 30\u2010year\u2010old male smoker with congenital amblyopia and oculocutaneous albinism was admitted to our hospital complaining of progressive dyspnea on exertion. Chest computed tomography images revealed diffuse reticular opacities and honeycombing in the bilateral lower lobes with sparing of the subpleural region along with emphysema predominantly in the upper lobes. Lung biopsy specimens showed a mixture of usual interstitial pneumonia and a non\u2010specific interstitial pneumonia pattern with emphysema. Of note, cuboidal epithelial cells with foamy cytoplasm on the alveolar walls and phagocytic macrophages with ceroid pigments in the fibrotic lesions were observed. The patient was diagnosed with H<\/jats:styled-content>ermansky\u2013P<\/jats:styled-content>udlak syndrome (HPS<\/jats:styled-content>) associated with combined pulmonary fibrosis and emphysema (CPFE<\/jats:styled-content>). Six years following the patient's initial admission to our hospital, he died from acute exacerbation (AE<\/jats:styled-content>) of CPFE<\/jats:styled-content> associated with HPS<\/jats:styled-content>. This is one of only few reports available on the clinicopathological characteristics of AE<\/jats:styled-content> in CPFE<\/jats:styled-content> associated with HPS<\/jats:styled-content>.<\/jats:p>","DOI":"10.1002\/rcr2.141","type":"journal-article","created":{"date-parts":[[2015,12,9]],"date-time":"2015-12-09T15:41:39Z","timestamp":1449675699000},"page":"13-15","source":"Crossref","is-referenced-by-count":6,"title":["Acute exacerbation of combined pulmonary fibrosis and emphysema associated with H<\/scp>ermansky\u2013P<\/scp>udlak syndrome"],"prefix":"10.1002","volume":"4","author":[{"given":"Keishi","family":"Sugino","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyoko","family":"Gocho","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naoshi","family":"Kikuchi","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazutoshi","family":"Shibuya","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Toho University Omori Medical Center Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshimasa","family":"Uekusa","sequence":"additional","affiliation":[{"name":"Department of Pathology Labor Health and Welfare Organization Kanto Rosai Hospital Kanagawa Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sakae","family":"Homma","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,12,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1159\/000091609"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2006-0469OC"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1827.2004.01626.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.179.4.1790887"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2011.02.003"}],"container-title":["Respirology Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Frcr2.141","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Frcr2.141","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rcr2.141","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/rcr2.141","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rcr2.141","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,7]],"date-time":"2023-10-07T14:14:27Z","timestamp":1696688067000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/rcr2.141"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,12,9]]},"references-count":5,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2016,3]]}},"alternative-id":["10.1002\/rcr2.141"],"URL":"https:\/\/doi.org\/10.1002\/rcr2.141","archive":["Portico"],"relation":{},"ISSN":["2051-3380","2051-3380"],"issn-type":[{"value":"2051-3380","type":"print"},{"value":"2051-3380","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,12,9]]}}}