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Subjects were divided into a younger (5\u20109 years) and older cohort (10\u201018 years) for the analysis. Upright and supine forced vital capacity (FVC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fourteen patients, all cross\u2010reactive immunologic material (CRIM)\u2010positive, met the inclusion criteria and were included in this study. Mean upright and supine FVC were 70.3% and 64.9% predicted, respectively, in the 5\u2010 to 9\u2010year\u2010old cohort; and 61.5% and 52.5% predicted, respectively, in the 10\u2010 to 18\u2010year\u2010old group. Individual patient trends showed stability in FVC overtime in six of the 14 patients. MIPs and MEPs were consistent with inspiratory and expiratory muscle weakness in the younger and older age group but did not decline with age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Data from this cohort of CRIM\u2010positive patients with IPD showed that ERT is able to maintain respiratory function in a subgroup of patients whereas others had a steady decline. There was a statistically significant decline in FVC from the upright to a supine position in both the younger and older age groups of CRIM\u2010positive ERT\u2010treated patients. Before ERT, patients with IPD were unable to maintain independent ventilation beyond the first few years of life.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24621","type":"journal-article","created":{"date-parts":[[2020,1,3]],"date-time":"2020-01-03T17:24:10Z","timestamp":1578072250000},"page":"674-681","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Pulmonary outcome measures in long\u2010term survivors of infantile Pompe disease on enzyme replacement therapy: A case series"],"prefix":"10.1002","volume":"55","author":[{"given":"Mai K.","family":"ElMallah","sequence":"first","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Pediatrics Duke University School of Medicine Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ankit K.","family":"Desai","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Duke 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