{"id":29,"date":"2024-11-18T12:14:17","date_gmt":"2024-11-18T12:14:17","guid":{"rendered":"https:\/\/www.naglazyme.com\/en-us\/?page_id=29"},"modified":"2026-06-22T18:46:04","modified_gmt":"2026-06-22T18:46:04","slug":"what-does-mps-vi-do-to-the-body","status":"publish","type":"page","link":"https:\/\/www.naglazyme.com\/en-us\/what-does-mps-vi-do-to-the-body\/","title":{"rendered":"What does MPS VI do to the body?"},"content":{"rendered":"
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What does MPS VI do to the body?<\/h1>\n

GAGs are a key component of tissues throughout the body. In MPS VI, too many GAGs build up and many major organs and systems can be harmed.1,2<\/sup><\/h2>\n

This can lead to a wide range of symptoms as you can see from the table below.\u00a0MPS VI affects people differently<\/a>. Not everyone shows all these symptoms. Some people have obvious symptoms almost from birth. Others may only notice problems over many years. Either way, as GAGs build up in the cells, MPS VI causes serious disabilities over time.<\/p>\n <\/div>\n <\/div>\n <\/div>\n<\/div>\n\n

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Common Symptoms of MPS VI<\/h2>\n <\/div>\n <\/div>\n <\/div>\n<\/div>\n\n
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Ophthalmological\n<\/h2>\n <\/div>\n
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Impaired vision, corneal clouding, glaucoma, optic nerve disease3,4<\/sup><\/p>\n <\/div>\n <\/div>\n

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Dental\n<\/h2>\n <\/div>\n
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Enlarged tongue, small and widely spaced teeth3<\/sup><\/p>\n <\/div>\n <\/div>\n

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Ear, nose, and throat\n<\/h2>\n <\/div>\n
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Impaired hearing, recurrent otitis media, recurrent sinusitis3<\/sup><\/p>\n <\/div>\n <\/div>\n

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Neurological\n<\/h2>\n <\/div>\n
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Spinal cord compression, hydrocephalus, carpal tunnel syndrome3,5,6<\/sup><\/p>\n <\/div>\n <\/div>\n

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Cardiac\n<\/h2>\n <\/div>\n
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Valvular disease, cardiomyopathy, cardiac arrhythmia, systemic and pulmonary hypertension3,7-9<\/sup><\/p>\n <\/div>\n <\/div>\n

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Pulmonary\n<\/h2>\n <\/div>\n
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Obstructive and restrictive airway disease, sleep apnea, recurrent pulmonary infections10,11<\/sup><\/p>\n <\/div>\n <\/div>\n

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Abdominal\n<\/h2>\n <\/div>\n
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Hepatosplenomegaly, umbilical and inguinal hernias3,10<\/sup><\/p>\n <\/div>\n <\/div>\n

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Skeletal\n<\/h2>\n <\/div>\n
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Joint stiffness and contractures, dysostosis multiplex, hip dysplasia3,4,6<\/sup><\/p>\n <\/div>\n <\/div>\n <\/div>\n <\/div>\n <\/div>\n

Learn more about how MPS VI progresses \u00bb<\/a><\/p>\n <\/div>\n \t\t<\/div>\n\t<\/div>\n<\/div>\n\n

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References:<\/strong>\n<\/h4>\n\t\t\t\t\t\t
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  1. Swiedler SJ, Beck M, Bajbouj M, et al. Threshold effect of urinary glycosaminoglycans and the walk test as indicators of disease progression in a survey of subjects with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). Am J Med Genet A.<\/em> 2005;134A(2):144-151.\n<\/span><\/li>\n
  2. Neufeld EF, Muenzer J. The mucopolysaccharidoses. In: Scriver CR, Beaudet AL, Sly WS, et al., eds. The Metabolic and Molecular Bases of Inherited Disease.<\/em> 8th ed. New York, NY: McGraw-Hill; 2001:3421-3452.\n<\/span><\/li>\n
  3. National MPS Society. Maroteaux-Lamy syndrome. A guide to understanding MPS VI [booklet]. Accessed March 24, 2026. https:\/\/mpssociety.org\/wp-content\/uploads\/2025\/03\/MPS_VI_Final_Booklet.pdf\u00a0<\/a>\n<\/span><\/li>\n
  4. Giugliani R, Harmatz P, Wraith JE. Management guidelines for mucopolysaccharidosis VI. Pediatrics.<\/em> 2007;120(2):405-418.\n<\/span><\/li>\n
  5. Giugliani R, Lampe C, Guffon N. Natural history and galsulfase treatment in mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome): 10-year follow-up of patients previously participating in an MPS VI Survey Study. Am J Med Genet A.<\/em> 2014;164A(8):1953-1964.\n<\/span><\/li>\n
  6. D’Avanzo F, Zanetti A, De Filippis C, Tomanin R. Mucopolysaccharidosis type VI: an updated overview of the disease. Int J Mol Sci.<\/em> 2021;22(24):13456.\n<\/span><\/li>\n
  7. Clarke L, Ellaway C, Foster H. Understanding the early presentation of mucopolysaccharidosis disorders: results of a systematic literature review and physician survey. J Inborn Errors Metab Screen.<\/em> 2018;6:1-12.\n<\/span><\/li>\n
  8. Wilcox WR. Lysosomal storage disorders: the need for better pediatric recognition and comprehensive care. J Pediatr.<\/em> 2004;144(suppl 5):S3-S14.\n<\/span><\/li>\n
  9. Harmatz P, Giugliani R, Schwartz I, et al. Enzyme replacement therapy for mucopolysaccharidosis VI: a phase 3 randomized, double-blind, placebo-controlled, multinational study of recombinant human N-acetylgalactosamine-4-sulfatase and follow-on extension. J Pediatr.<\/em> 2006;148(4):533-539.\n<\/span><\/li>\n
  10. Coutinho MF, Lacerda L, Alves S. Glycosaminoglycan storage disorders: a review. Biochem Res Int.<\/em> 2012;2012:471325.\n<\/span><\/li>\n
  11. Akyol MU, Alden TD, Amartino H, et al. 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 <\/ol>\n\t\t\t\t\t<\/div>\n\t<\/div>\n<\/div>\n\n
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