{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T17:40:49Z","timestamp":1791999649149,"version":"4.4.0"},"reference-count":26,"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:title>Background<\/jats:title><jats:p>The mucopolysaccharidoses (MPS) comprise a group of inherited lysosomal storage disorders characterized by deficiencies in enzymes catalyzing the degradation of glycosaminoglycans. Impairment of pulmonary function is an important health problem for patients with MPS. However, there are few published reports on the prevalence and severity of pulmonary dysfunction in relation to age and treatment in this disorder.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>To evaluate pulmonary function in patients with MPS, we performed spirometry in 35 patients (22 males and 13 females; 1 with MPS I, 12 with MPS II, 16 with MPS IVA, and 6 with MPS VI; mean age, 14.6\u2009\u00b1\u20095.9 years; age range, 6.4 years to 33 years). Forced vital capacity (FVC), forced expired volume in 1\u2009sec (FEV<jats:sub>1<\/jats:sub>), FEV<jats:sub>1<\/jats:sub> to FVC ratio (FEV1\/FVC), peak expiratory flow (PEF), and mean forced expiratory flow during the middle half of FVC (FEF<jats:sub>25\u201375%<\/jats:sub>) were measured.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Mean FVC, FEV<jats:sub>1<\/jats:sub>, PEF, and FEF<jats:sub>25\u201375%<\/jats:sub> were 74.2%, 73.9%, 64.7%, and 37.1% of the predicted values, respectively. By spirometric classification, 32 patients (91%) had small airway disease (FEF<jats:sub>25\u201375%<\/jats:sub>\u2009&lt;\u200965%), 17 (48%) had restrictive lung disease, and 3 (9%) had obstructive lung disease. Percent predicted FVC, FEV<jats:sub>1<\/jats:sub>, and PEF, as well as FEV<jats:sub>1<\/jats:sub>\/FVC, were all negatively correlated with age (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.01), such that pubertal and post\u2010pubertal patients had significantly lower values than younger patients. Of eight attenuated MPS II and VI patients who underwent follow\u2010up pulmonary function testing after receiving enzyme replacement therapy (ERT) for 1.5\u20137.4 years, six showed improvements in % predicted FVC and five improved in % predicted FEV<jats:sub>1<\/jats:sub>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our additional characterization of the types and prevalence of pulmonary function abnormalities seen in MPS patients should be useful for clinical care. <jats:bold>Pediatr Pulmonol. 2014; 49:277\u2013284.<\/jats:bold> \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.22774","type":"journal-article","created":{"date-parts":[[2013,2,8]],"date-time":"2013-02-08T15:35:21Z","timestamp":1360337721000},"page":"277-284","source":"Crossref","is-referenced-by-count":57,"title":["Characterization of pulmonary function impairments in patients with mucopolysaccharidoses\u2014changes with age and treatment"],"prefix":"10.1002","volume":"49","author":[{"given":"Shuan\u2010Pei","family":"Lin","sequence":"first","affiliation":[{"name":"Department of Pediatrics Mackay Memorial Hospital Taipei Taiwan"},{"name":"Department of Medical Research Mackay Memorial Hospital Taipei Taiwan"},{"name":"Department of Medicine Mackay Medical College New Taipei City Taiwan"},{"name":"Department of Early Infant Care and Education, Mackay Medicine Nursing and Management College Taipei Taiwan"},{"name":"Department of Infant and Child Care National Taipei University of Nursing and Health Sciences Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shou\u2010Chuan","family":"Shih","sequence":"additional","affiliation":[{"name":"Department of Medicine Mackay Medical College New Taipei City Taiwan"},{"name":"Division of Gastroenterology, Department of Internal Medicine Mackay Memorial Hospital Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chih\u2010Kuang","family":"Chuang","sequence":"additional","affiliation":[{"name":"Department of Medical Research Mackay Memorial Hospital Taipei Taiwan"},{"name":"Medical College Fu\u2010Jen Catholic University Taipei Taiwan"},{"name":"Institute of Biotechnology National Taipei University of Technology Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kuo\u2010Sheng","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Medicine Mackay Medical College New Taipei City Taiwan"},{"name":"Department of Early Infant Care and Education, Mackay Medicine Nursing and Management College Taipei Taiwan"},{"name":"Department of Otorhinolaryngology Mackay Memorial Hospital Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming\u2010Ren","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Mackay Memorial Hospital Taipei Taiwan"},{"name":"Department of Early Infant Care and Education, Mackay Medicine Nursing and Management College Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dau\u2010Ming","family":"Niu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Taipei Veterans General Hospital Taipei Taiwan"},{"name":"Institute of Clinical Medicine National Yang\u2010Ming University Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pao Chin","family":"Chiu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Kaohsiung Veterans General Hospital Kaohsiung Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shio Jean","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Chi Mei Medical Center Tainan Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hsiang\u2010Yu","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Mackay Memorial Hospital Taipei Taiwan"},{"name":"Department of Medical Research Mackay Memorial Hospital Taipei Taiwan"},{"name":"Department of Medicine Mackay Medical College New Taipei City Taiwan"},{"name":"Department of Early Infant Care and Education, Mackay Medicine Nursing and Management College Taipei Taiwan"},{"name":"Institute of Clinical Medicine National Yang\u2010Ming University Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,2,8]]},"reference":[{"key":"e_1_2_9_2_1","first-page":"3421","volume-title":"The metabolic and molecular bases of inherited disease","author":"Neufeld EF","year":"2001"},{"key":"e_1_2_9_3_1","first-page":"1","volume-title":"Neurochemistry of metabolic diseases\u2010lysosomal storage diseases, 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