{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T03:28:12Z","timestamp":1790479692290,"version":"4.1.0"},"reference-count":21,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T00:00:00Z","timestamp":1696982400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Multiple lung cysts are one of the major features of Birt\u2010Hogg\u2010Dub\u00e9 syndrome (BHD), but little is known about their nature and pathogenesis. We report a case of a woman diagnosed with BHD lung cysts who exhibited pulmonary interstitial glycogenosis (PIG), a mesenchymal abnormality hitherto undescribed in this disease, in specimens resected at 14 and 29 years of age. Histopathologically, oval to spindle clear cells were seen in the subepithelial interstitial tissue of septal structures and the walls of the cysts. They had abundant periodic acid\u2013Schiff\u2010positive cytoplasmic glycogen. Immunohistochemically, these cells were positive for a few markers of mesenchymal stem cell\u2010like lineage, including vimentin, CD44, and CD10, and negative for markers of epithelial or specific mesenchymal differentiation; these results were consistent with the reported immunophenotype of PIG cells. These PIG cells were more abundant in her specimen at age 14 years than in the second specimen from adulthood. The present case suggests that BHD lung cysts belong to a group of pulmonary developmental disorders characterized by combined PIG and alveolar simplification\/cystic change. Disorders with PIG may persist until adulthood and may be of clinical and pathological significance.<\/jats:p>","DOI":"10.1111\/pin.13383","type":"journal-article","created":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T10:51:42Z","timestamp":1697021502000},"page":"601-608","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Pulmonary interstitial glycogenosis in Birt\u2010Hogg\u2010Dub\u00e9 syndrome\u2010associated lung cysts: A new insight into the pathogenesis?"],"prefix":"10.1111","volume":"73","author":[{"given":"Riken","family":"Kawachi","sequence":"first","affiliation":[{"name":"Division of Respiratory Surgery Nihon University School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1241-8610","authenticated-orcid":false,"given":"Yukio","family":"Nakatani","sequence":"additional","affiliation":[{"name":"Department of Pathology Yokosuka Kyosai Hospital  Yokosuka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsuko","family":"Furuya","sequence":"additional","affiliation":[{"name":"Pathology Center, Genetic Lab Co., Ltd. Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoya","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Pathology Tokai University Hospital  Isehara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Kondo","sequence":"additional","affiliation":[{"name":"Department of Pathology Tokai University Hospital  Isehara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6536-648X","authenticated-orcid":false,"given":"Yoji","family":"Nagashima","sequence":"additional","affiliation":[{"name":"Department of Surgical Pathology Tokyo Women's Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohiro","family":"Nakayama","sequence":"additional","affiliation":[{"name":"Laboratory Medicine Nihon University School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiro","family":"Okada","sequence":"additional","affiliation":[{"name":"Radiology Nihon University School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Sakurai","sequence":"additional","affiliation":[{"name":"Division of Respiratory Surgery Nihon University School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinobu","family":"Masuda","sequence":"additional","affiliation":[{"name":"Pathology Nihon University School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,11]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1977.01640120042005"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1535-6108(02)00104-6"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200610-1483OC"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12807"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1827.2009.02434.x"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182475240"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/pin.12752"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2105139"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2017.06.026"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24123"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22871"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24324"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0040-1708500"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/mgg3.373"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25548"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.2350\/08-12-0575.1"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.12368"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00853-2020"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2019-214112"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddz158"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-021-03963-9"}],"container-title":["Pathology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pin.13383","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,6]],"date-time":"2024-01-06T08:35:48Z","timestamp":1704530148000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pin.13383"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,11]]},"references-count":21,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2023,12]]}},"alternative-id":["10.1111\/pin.13383"],"URL":"https:\/\/doi.org\/10.1111\/pin.13383","archive":["Portico"],"relation":{},"ISSN":["1320-5463","1440-1827"],"issn-type":[{"value":"1320-5463","type":"print"},{"value":"1440-1827","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,11]]},"assertion":[{"value":"2023-05-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-17","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-10-11","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}