{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T10:50:21Z","timestamp":1777891821843,"version":"3.51.4"},"reference-count":9,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2014,10,14]],"date-time":"2014-10-14T00:00:00Z","timestamp":1413244800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Respir Care"],"published-print":{"date-parts":[[2015,2]]},"abstract":"<jats:p>Pompe disease is a rare autosomal recessive disorder caused by \u03b1-glucosidase deficiency. Lower airway involvement and management are rare in patients with late-onset Pompe disease. We describe the case of a 16-y-old girl with late-onset Pompe disease who presented with obvious progressive deterioration in respiratory function. Pulmonary hypertension was also apparent on echocardiography. She had been on enzyme replacement therapy and nighttime CPAP ventilation for several years. Flexible bronchoscopy was used for diagnosis and subsequent implantation of a bronchial airway stent. Following implantation of the stent, the patient's pulmonary function stabilized, and her pulmonary hypertension resolved. The patient continued on enzyme replacement therapy and nighttime CPAP ventilation. This case highlights that lower airway involvement may occur with late-onset Pompe disease and that flexible bronchoscopy can be an effective tool for both diagnosis and management of lower airway collapse in late-onset Pompe disease.<\/jats:p>","DOI":"10.4187\/respcare.03419","type":"journal-article","created":{"date-parts":[[2014,10,15]],"date-time":"2014-10-15T03:08:23Z","timestamp":1413342503000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":18,"title":["Late-Onset Pompe Disease With Left-Sided Bronchomalacia"],"prefix":"10.1177","volume":"60","author":[{"given":"Chia-Feng","family":"Yang","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dau-Ming","family":"Niu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan."},{"name":"Department of Pediatrics, School of Medicine, National Yang-Ming University, Taipei, Taiwan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mei-Jy","family":"Jeng","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yu-Sheng","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pei-Chen","family":"Taso","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wen-Jue","family":"Soong","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan."},{"name":"Department of Pediatrics, School of Medicine, National Yang-Ming University, Taipei, Taiwan."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2014,10,14]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2008.12.009"},{"key":"e_1_3_2_3_2","first-page":"304","article-title":"[Concerning idiopathic hypertrophy of the heart]","volume":"76","author":"Pompe JC","year":"1932","unstructured":"Pompe JC. [Concerning idiopathic hypertrophy of the heart]. Ned Tijdschr Geneeskd, 1932; 76:304-311. Article in Dutch.","journal-title":"Ned Tijdschr Geneeskd"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.112.2.332"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.11.033"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2007.06.002"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21025"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2010.11.053"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2011.12.010"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2011.06.012"}],"container-title":["Respiratory Care"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.4187\/respcare.03419","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.4187\/respcare.03419","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.4187\/respcare.03419","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T06:12:23Z","timestamp":1777615943000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.4187\/respcare.03419"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,10,14]]},"references-count":9,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2015,2]]}},"alternative-id":["10.4187\/respcare.03419"],"URL":"https:\/\/doi.org\/10.4187\/respcare.03419","relation":{},"ISSN":["0020-1324"],"issn-type":[{"value":"0020-1324","type":"print"}],"subject":[],"published":{"date-parts":[[2014,10,14]]}}}