{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T21:03:52Z","timestamp":1773263032983,"version":"3.50.1"},"reference-count":9,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,2,8]],"date-time":"2013-02-08T00:00:00Z","timestamp":1360281600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:label\/><jats:p>Pulmonary interstitial glycogenosis (PIG) is an idiopathic interstitial lung disease of infants. The underlying pulmonary pathophysiology of PIG has not been well characterized. Herein we report a term\u2010gestatation infant who presented with persistent tachypnea and hypoxia. A chest CT scan demonstrated a diffuse ground glass appearance and lung biopsy demonstrated increased alveolar septae cellularity with glycogen\u2010containing cells, consistent with a diagnosis of PIG. At 3 months of age, pulmonary function testing included: pre\u2010 and post\u2010bronchodilator forced expiratory flows using the raised\u2010volume technique and the ratio of pulmonary diffusing capacity for carbon monoxide to alveolar volume (DL<jats:sub>CO<\/jats:sub>\/V<jats:sub>A<\/jats:sub>). He was prescribed 5 days of oral prednisolone (2\u2009mg\/kg\/day) and pulmonary function testing (PFT) was repeated at 5, 13, and 20 months of age. Initial PFTs demonstrated reduced forced vital capacity (FVC: <jats:italic>Z<\/jats:italic>\u2010score\u2009=\u2009\u22122.36) and an increased ratio of forced expiratory volume in 0.5\u2009sec to FVC (FEV0.5\/FVC: <jats:italic>Z<\/jats:italic>\u2010score\u2009=\u20091.15) with no significant change following an inhaled bronchodilator. There was also a marked reduction in DL<jats:sub>CO<\/jats:sub>\/V<jats:sub>A<\/jats:sub> (<jats:italic>Z<\/jats:italic>\u2010score\u2009=\u2009\u22124.74) compared to age\u2010matched controls. Follow\u2010up demonstrated progressive clinical improvement as well as an increase in Z\u2010FVC and normalization of DL<jats:sub>CO<\/jats:sub>\/V<jats:sub>A<\/jats:sub>. Our in vivo physiological findings are consistent with previous reports that symptom resolution correlated with histological thinning of the alveolar septae upon repeat lung biopsy. The restrictive lung disease we observed is consistent with expected reduced compliance of an alveolar interstitial lung process like PIG, whereas the absence of a reduction in FEV0.5\/FVC confirms the absence of obstructive airway disease. <jats:bold>Pediatr Pulmonol. 2014; 49:E17\u2013E20.<\/jats:bold> \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.22738","type":"journal-article","created":{"date-parts":[[2013,2,8]],"date-time":"2013-02-08T15:35:12Z","timestamp":1360337712000},"source":"Crossref","is-referenced-by-count":14,"title":["An infant with pulmonary interstitial glycogenosis: Clinical improvement is associated with improvement in the pulmonary diffusion capacity"],"prefix":"10.1002","volume":"49","author":[{"given":"Zarmina","family":"Ehsan","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children Indiana University School of Medicine Indianapolis Indiana"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory S.","family":"Montgomery","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children Indiana University School of Medicine Indianapolis Indiana"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina","family":"Tiller","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children Indiana University School of Medicine Indianapolis Indiana"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey","family":"Kisling","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children Indiana University School of Medicine Indianapolis Indiana"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel V.","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children Indiana University School of Medicine Indianapolis Indiana"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert S.","family":"Tepper","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riley Hospital for Children Indiana University School of Medicine Indianapolis Indiana"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,2,8]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.159.3.9803001"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20403"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2105139"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200703-393OC"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-010-1730-7"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-010-1670-2"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.2350\/08-12-0575.1"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1203\/01.PDR.0000103930.93849.B2"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20255"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fppul.22738","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.22738","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T06:53:52Z","timestamp":1696488832000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.22738"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,2,8]]},"references-count":9,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2014,3]]}},"alternative-id":["10.1002\/ppul.22738"],"URL":"https:\/\/doi.org\/10.1002\/ppul.22738","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,2,8]]}}}