{"id":85,"date":"2022-09-26T19:28:56","date_gmt":"2022-09-26T19:28:56","guid":{"rendered":"https:\/\/naglazymehcp-dev-001.azurewebsites.net\/en-us\/naglazyme\/?page_id=85"},"modified":"2023-04-19T16:16:15","modified_gmt":"2023-04-19T16:16:15","slug":"about-mps-vi-2","status":"publish","type":"page","link":"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/about-mps-vi-2\/","title":{"rendered":"About MPS VI"},"content":{"rendered":"
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About MPS VI<\/h1>\n

Overview<\/h3>\n

Maroteaux\u2010Lamy syndrome (MPS VI) is a progressive, clinically heterogeneous lysosomal storage disorder (LSD) that results from a deficiency in the arylsulfatase B (ASB) enzyme. MPS VI is one of a group of 7 types of LSDs known as mucopolysaccharidosis disorders. An estimated 1100 people worldwide are living with MPS VI.1<\/sup><\/p>\n

In MPS VI, lysosomes deficient in ASB retain excessive amounts of glycosaminoglycans (GAGs), leading to multisystemic organ damage.1,2<\/sup> MPS VI\u2013related multisystemic abnormalities and significant functional disability are life\u2010altering.2<\/sup> While some patients have rapidly progressing disease, others have symptoms that are not recognized until adolescence or later.3<\/sup><\/p>\n

To help patients with MPS VI maintain quality of life, early diagnosis, referral, and treatment are essential.<\/p>\n

Learn more about the biochemical basis of MPS VI \u00bb<\/a><\/p>\n <\/div>\n <\/div>\n <\/div>\n<\/div>\n\n

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References:\n<\/h4>\n\t\t\t\t\t\t
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  1. Swiedler SJ, Beck M, Bajbouj M, et al<\/em>. Threshold effect of urinary glycosaminoglycans and the walk test as indicators of disease progression in a survey of subjects with mucopolysaccharidosis VI (Maroteaux\u2010Lamy syndrome). Am J Med Genet A.<\/em> 2005;134A(2):144\u2010150.\n<\/span><\/li>\n
  2. Akyol MU, Alden TD, Amartino H, et al<\/em>. Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance. Orphanet J Rare Dis<\/em>. 2019;14:118.\n<\/span><\/li>\n
  3. Valayannopoulos V, Nicely H, Harmatz P, Turbeville S. Mucopolysaccharidosis VI. Orphanet J Rare Dis<\/em>. 2010;5:5\u201020. doi:10.1186\/1750\u20101172\u20105\u20105.\n<\/span><\/li>\n <\/ol>\n\t\t\t\t\t<\/div>\n\t<\/div>\n<\/div>","protected":false},"excerpt":{"rendered":"","protected":false},"author":4,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"inline_featured_image":false,"footnotes":""},"class_list":["post-85","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"\nNaglazyme | HCP About MPS VI<\/title>\n<meta name=\"description\" content=\"Maroteaux-Lamy syndrome (MPS VI) is a progressive lysosomal storage disorder (LSD) that affects an estimated 1100 people worldwide\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/about-mps-vi-2\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta 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