{"id":101,"date":"2022-09-26T19:47:25","date_gmt":"2022-09-26T19:47:25","guid":{"rendered":"https:\/\/naglazymehcp-dev-001.azurewebsites.net\/en-us\/naglazyme\/?page_id=101"},"modified":"2023-04-19T16:16:47","modified_gmt":"2023-04-19T16:16:47","slug":"clinical-manifestations-of-mps-vi","status":"publish","type":"page","link":"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/clinical-manifestations-of-mps-vi\/","title":{"rendered":"Clinical manifestations of MPS VI"},"content":{"rendered":"
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Clinical Manifestations of MPS VI<\/h1>\n

Glycosaminoglycans (GAGs) are a major component of connective tissues and organs throughout the body; as a result of excessive GAG buildup in MPS VI, the disease can affect many different organ systems; symptoms may vary widely, due in part to varying rates of disease progression.1,2<\/sup>\u00a0The most common effects of MPS VI are shown below:<\/p>\n <\/div>\n <\/div>\n <\/div>\n<\/div>\n\n

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\n \"Common <\/div>\n <\/figure>\n <\/div>\n \n
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Opthalmologocal\n<\/h2>\n <\/div>\n
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Impaired vision, corneal clouding, glaucoma, optic nerve disease 3,4<\/sup><\/p>\n <\/div>\n <\/div>\n

\n
\n
\n <\/span>\n <\/span>\n <\/div>\n

Dental\n<\/h2>\n <\/div>\n
\n

Enlarged tongue, small and widely spaced teeth 5<\/sup><\/p>\n <\/div>\n <\/div>\n

\n
\n
\n <\/span>\n <\/span>\n <\/div>\n

Ear, nose, and throat\n<\/h2>\n <\/div>\n
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Impaired hearing, recurrent otitis media, recurrent sinusitis 2,3,6<\/sup><\/p>\n <\/div>\n <\/div>\n

\n
\n
\n <\/span>\n <\/span>\n <\/div>\n

Neurological\n<\/h2>\n <\/div>\n
\n

Spinal cord compression, hydrocephalus, carpal tunnel syndrome 3<\/sup><\/p>\n <\/div>\n <\/div>\n

\n
\n
\n <\/span>\n <\/span>\n <\/div>\n

Cardiac\n<\/h2>\n <\/div>\n
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Valvular disease, cardiomyopathy, cardiac arrhythmia, systemic and pulmonary hypertension 3<\/sup><\/p>\n <\/div>\n <\/div>\n

\n
\n
\n <\/span>\n <\/span>\n <\/div>\n

Pulmonary\n<\/h2>\n <\/div>\n
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Obstructive and restrictive airway disease, sleep apnea, recurrent pulmonary infections 6<\/sup><\/p>\n <\/div>\n <\/div>\n

\n
\n
\n <\/span>\n <\/span>\n <\/div>\n

Abdominal\n<\/h2>\n <\/div>\n
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Hepatosplenomegaly, umbilical and inguinal hernias2,7<\/sup><\/p>\n <\/div>\n <\/div>\n

\n
\n
\n <\/span>\n <\/span>\n <\/div>\n

Skeletal\n<\/h2>\n <\/div>\n
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Joint stiffness and contractures, dysostosis multiplex, hip dysplasia 3<\/sup><\/p>\n <\/div>\n <\/div>\n <\/div>\n <\/div>\n <\/div>\n

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

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References:\n<\/h4>\n\t\t\t\t\t\t
    \n
  1. Neufeld EF, Muenzer J. The mucopolysaccharidoses. In: Scriver CR, Beaud et al<\/em>, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. Vol 3<\/em>. 8th ed. New York: McGraw-Hill; 2001:3421-3452.\n<\/span><\/li>\n
  2. 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\u2013Lamy syndrome). Am J Med Genet A<\/em>. 2005;134A(2):144-150.\n<\/span><\/li>\n
  3. 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(1):118.\n<\/span><\/li>\n
  4. Ashworth JL, Kruse FE, Bachmann B, et al<\/em>. Ocular manifestations in the mucopolysaccharidoses\u2014a review. Clin Experiment Ophthalmol<\/em>. 2010;38:12-22.\n<\/span><\/li>\n
  5. Alpoz AR, \u00c7oker M, \u00c7elen E, et al<\/em>. The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod<\/em>. 2006;101(5):632-637.\n<\/span><\/li>\n
  6. Yeung AH, Cowan MJ, Horn B, Rosbe KW. Airway management in children with mucopolysaccharidoses. Arch Otolaryngol Head Neck Surg<\/em>. 2009;135(1):73-79.\n<\/span><\/li>\n
  7. Wilcox WR. Lysosomal storage disorders: the need for better pediatric recognition and comprehensive care. J Pediatr. 2004;144(suppl 5):S3-S14.\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-101","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"\nClinical manifestations of MPS VI - BioMarin Naglazyme HCP EN-US<\/title>\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\/clinical-manifestations-of-mps-vi\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Clinical manifestations of MPS VI - BioMarin Naglazyme HCP EN-US\" \/>\n<meta property=\"og:url\" content=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/clinical-manifestations-of-mps-vi\/\" \/>\n<meta property=\"og:site_name\" content=\"BioMarin Naglazyme HCP EN-US\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/BioMarinOfficial\/\" \/>\n<meta property=\"article:modified_time\" content=\"2023-04-19T16:16:47+00:00\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/clinical-manifestations-of-mps-vi\/\",\"url\":\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/clinical-manifestations-of-mps-vi\/\",\"name\":\"Clinical manifestations of MPS VI - 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