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Disaccharides were produced from polymer GAGs by digestion with chondroitinase B, heparitinase, and keratanase II. Heparan sulfate (0S, NS), dermatan sulfate (DS) and mono\u2010 and di\u2010sulfated KS were measured by liquid chromatography tandem mass spectrometry (LC\u2010MS\/MS). Median absolute deviation (MAD) was used to determine cutoffs to distinguish patients from controls. Cutoffs were defined as median\u2009+\u20097\u00d7 MAD from general newborns.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The cutoffs were as follows: HS\u20100S\u2009&gt;\u200990 ng\/mL; HS\u2010NS\u2009&gt;\u200923 ng\/mL, DS\u2009&gt;\u200988 ng\/mL; mono\u2010sulfated KS\u2009&gt;\u2009445 ng\/mL; di\u2010sulfated KS\u2009&gt;\u200989 ng\/mL and ratio di\u2010KS in total KS\u2009&gt;\u200932 %. All MPS I and II samples were above the cutoffs for HS\u20100S, HS\u2010NS, and DS, and all MPS III samples were above cutoffs for HS\u20100S and HS\u2010NS. The rate of false positives for MPS I and II was 0.03 % based on a combination of HS\u20100S, HS\u2010NS, and DS, and for MPS III was 0.9 % based upon a combination of HS\u20100S and HS\u2010NS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Combination of levels of two or more different GAGs improves separation of MPS patients from unaffected controls, indicating that GAG measurements are potentially valuable biomarkers for newborn screening for MPS.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s10545-016-9981-6","type":"journal-article","created":{"date-parts":[[2016,10,7]],"date-time":"2016-10-07T15:38:18Z","timestamp":1475854698000},"page":"151-158","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Newborn screening for mucopolysaccharidoses: a pilot study of measurement of glycosaminoglycans by tandem mass spectrometry"],"prefix":"10.1002","volume":"40","author":[{"given":"Francyne","family":"Kubaski","sequence":"first","affiliation":[{"name":"Nemours\/Alfred I. duPont Hospital for Children 1600 Rockland Road Wilmington DE 19899 USA"},{"name":"Department of Biological Sciences University of Delaware Newark DE USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert W.","family":"Mason","sequence":"additional","affiliation":[{"name":"Nemours\/Alfred I. duPont Hospital for Children 1600 Rockland Road Wilmington DE 19899 USA"},{"name":"Department of Biological Sciences University of Delaware Newark DE USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akiko","family":"Nakatomi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nagasaki University Nagasaki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruo","family":"Shintaku","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Osaka City University Graduate School of Medicine Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Xie","sequence":"additional","affiliation":[{"name":"Nemours\/Alfred I. duPont Hospital for Children 1600 Rockland Road Wilmington DE 19899 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naomi N.","family":"van Vlies","sequence":"additional","affiliation":[{"name":"Laboratory Genetic Metabolic Diseases Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather","family":"Church","sequence":"additional","affiliation":[{"name":"Willink Biochemical Genetics Unit Regional Genetics Laboratory Genetic Medicine St Mary's Hospital Manchester Manchester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Giugliani","sequence":"additional","affiliation":[{"name":"Medical Genetics Service, HCPA, Dep. 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