{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T17:16:41Z","timestamp":1791566201313,"version":"4.3.4"},"reference-count":21,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2018,7,18]],"date-time":"2018-07-18T00:00:00Z","timestamp":1531872000000},"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":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2018,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>We sought to describe the phenotype for patients with P.I.G. including presentation, evaluation, cardiac co\u2010morbidities, high resolution computed tomography findings, and outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>With institutional review board approval, we performed a retrospective review of patients with biopsy\u2010proven P.I.G. Biopsies, high resolution chest computed tomography, and cardiac evaluations were reviewed and characterized by experts in each field.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty\u2010two percent of the patients were male. The median gestational age was 37 weeks (range 27\u201040). The median age at biopsy was 1.6 months (range 0.3\u20106 months). Structural heart disease was present in 63% of patients. Pulmonary hypertension (diagnosed by echocardiogram and\/or cardiac catheterization) was noted in 38% of patients. Alveolar simplification was present in 79% of patients. Fifty percent of available biopsies revealed patchy disease. An increase in age at biopsy was associated with patchy (vs diffuse) disease. Ninety\u2010two percent of patients were treated with systemic corticosteroids. Median age at last follow\u2010up was 1234 days with a range of 37 days to 15 years. At the time of last follow\u2010up, 12 patients were off all support, eight were on supplemental oxygen, two were mechanically ventilated, one underwent lung transplantation, and one died. CT findings commonly included ground glass opacities (86%) and cystic change (50%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The P.I.G. phenotype has not been comprehensively described, and poor recognition and misconceptions about P.I.G. persist. P.I.G. is a disease that presents in early infancy, requires significant medical intervention, and frequently is seen in association with alveolar simplification and\/or cardiovascular disease. CT findings include ground glass opacities and cysts. Patients should be monitored for pulmonary hypertension. Without life\u2010threatening comorbidities, many patients do well over time, although respiratory symptoms may persist into adolescence.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24123","type":"journal-article","created":{"date-parts":[[2018,7,18]],"date-time":"2018-07-18T21:41:18Z","timestamp":1531950078000},"page":"1651-1658","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course"],"prefix":"10.1002","volume":"53","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3667-1856","authenticated-orcid":false,"given":"Deborah R.","family":"Liptzin","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Colorado School of Medicine and Children's Hospital Colorado Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1171-563X","authenticated-orcid":false,"given":"Christopher D.","family":"Baker","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Colorado School of Medicine and Children's Hospital Colorado Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey R.","family":"Darst","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Colorado School of Medicine and Children's Hospital Colorado Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason P.","family":"Weinman","sequence":"additional","affiliation":[{"name":"Department of Radiology University of Colorado School of Medicine Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan K.","family":"Dishop","sequence":"additional","affiliation":[{"name":"Division Chief of Pathology and Laboratory Medicine at Phoenix Children's Medical Group Phoenix Arizona"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Csaba","family":"Galambos","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Colorado School of Medicine and Children's Hospital Colorado Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John T.","family":"Brinton","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Informatics Colorado School of Public Health University of Colorado Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin R.","family":"Deterding","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Colorado School of Medicine and Children's Hospital Colorado Aurora Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,7,18]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.101.4.1065"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2105139"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201509-1809LE"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200703-393OC"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20255"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.2350\/08-12-0575.1"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201305-0923ST"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22738"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.rti.0000203937.82276.ce"},{"key":"e_1_2_8_11_1","unstructured":"Children's Hospital Colorado Community Health Assessment2012: Priorities and opportunities for outreach and advocacy. 2012. (Accessed 3\/24\/2017 athttps:\/\/www.childrenscolorado.org\/contentassets\/b779a1a9e0ef47d7999ca5126233df5a\/2012\u2010chco\u2010community\u2010health\u2010needs\u2010assessment.pdf.)."},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2017.06.026"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-010-1670-2"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201508-1655OC"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20243"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.09.2743"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2431-1-1"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-0637"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1089\/ped.2013.0323"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.028"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23124"},{"key":"e_1_2_8_22_1","first-page":"1","article-title":"High\u2010resolution CT findings of pulmonary interstitial glycogenosis","author":"Weinman JP","year":"2018","journal-title":"Pediatr Radiol"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fppul.24123","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.24123","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T05:10:05Z","timestamp":1694754605000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.24123"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,7,18]]},"references-count":21,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2018,12]]}},"alternative-id":["10.1002\/ppul.24123"],"URL":"https:\/\/doi.org\/10.1002\/ppul.24123","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,7,18]]},"assertion":[{"value":"2017-11-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-06-21","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-07-18","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}