{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T18:20:17Z","timestamp":1789928417400,"version":"4.0.1"},"reference-count":85,"publisher":"American Academy of Pediatrics (AAP)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,8,1]]},"abstract":"<jats:p>Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) is a lysosomal storage disease that is characterized by systemic clinical manifestations and significant functional impairment. Diagnosis and management are often challenging because of the considerable variability in symptom presentation and rate of progression. The optimal standard of care should be based on evidence from randomized, controlled trials, meta-analyses, systematic reviews, and expert opinion. In support of this goal, comprehensive management guidelines have been drafted by an international group of experts in the management of patients with mucopolysaccharidosis VI. The guidelines provide a detailed outline of disease manifestations by body system, recommendations for regular assessments, and an overview of current treatment options.<\/jats:p>","DOI":"10.1542\/peds.2006-2184","type":"journal-article","created":{"date-parts":[[2007,8,1]],"date-time":"2007-08-01T13:53:25Z","timestamp":1185976405000},"page":"405-418","source":"Crossref","is-referenced-by-count":197,"title":["Management Guidelines for Mucopolysaccharidosis VI"],"prefix":"10.1542","volume":"120","author":[{"given":"Roberto","family":"Giugliani","sequence":"first","affiliation":[{"name":"Medical Genetics Service, Hospital de Cli\u0301nicas de Porto Alegre, Porto Alegre, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Harmatz","sequence":"additional","affiliation":[{"name":"Children's Hospital and Research Center Oakland, Oakland, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James E.","family":"Wraith","sequence":"additional","affiliation":[{"name":"Willink Biochemical Genetics Unit, Royal Manchester Children's Hospital, Manchester, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2007,8,1]]},"reference":[{"key":"2021120901553099300_R1","unstructured":"Maroteaux P, Leve\u0302que B, Marie J, Lamy M. A new dysostosis with urinary elimination of chondroitin sulfate B [in French]. Presse Med.\u20081963;71:1849\u20131852"},{"key":"2021120901553099300_R2","unstructured":"Neufeld E, Muenzer J. The mucopolysaccharidoses. In: Scriver C, Beaudet A, Valle D, Sly W, eds. The Metabolic and Molecular Bases of Inherited Disease. 8th ed. New York, NY: McGraw-Hill; 2001:3421\u20133452"},{"key":"2021120901553099300_R3","unstructured":"Spranger JW, Koch F, McKusick VA, Natzschka J, Wiedemann HR, Zellweger H. Mucopolysaccharidosis VI (Maroteaux-Lamy's disease). Helv Paediatr Acta.\u20081970;25:337\u2013362"},{"key":"2021120901553099300_R4","doi-asserted-by":"crossref","unstructured":"Fong LV, Menahem S, Wraith JE, Chow CW. Endocardial fibroelastosis in mucopolysaccharidosis type VI. Clin Cardiol.\u20081987;10:362\u2013364","DOI":"10.1002\/clc.4960100612"},{"key":"2021120901553099300_R5","doi-asserted-by":"crossref","unstructured":"Hayflick S, Rowe S, Kavanaugh-McHugh A, Olson JL, Valle D. Acute infantile cardiomyopathy as a presenting feature of mucopolysaccharidosis VI. J Pediatr.\u20081992;120:269\u2013272","DOI":"10.1016\/S0022-3476(05)80441-X"},{"key":"2021120901553099300_R6","doi-asserted-by":"crossref","unstructured":"Pilz H, Von Figura K, Goebel H. Deficiency of arylsulfatase B in 2 brothers aged 40 and 38 years (Maroteaux-Lamy Syndrome, type B). Ann Neurol.\u20081979;6:315\u2013325","DOI":"10.1002\/ana.410060405"},{"key":"2021120901553099300_R7","doi-asserted-by":"crossref","unstructured":"Tonnesen T, Gregersen H, Guttler F. Normal MPS excretion, but dermatan sulphaturia, combined with a mild Maroteaux-Lamy phenotype. J Med Genet.\u20081991;28:499\u2013501","DOI":"10.1136\/jmg.28.7.499"},{"key":"2021120901553099300_R8","doi-asserted-by":"crossref","unstructured":"Lowry RB, Applegarth DA, Toone JR, MacDonald E, Thunem NY. An update on the frequency of mucopolysaccharide syndromes in British Columbia. Hum Genet.\u20081990;85:389\u2013390","DOI":"10.1007\/BF00206770"},{"key":"2021120901553099300_R9","doi-asserted-by":"crossref","unstructured":"Meikle PJ, Hopwood JJ, Clague AE, Carey WF. Prevalence of lysosomal storage disorders. JAMA.\u20081999;281:249\u2013254","DOI":"10.1001\/jama.281.3.249"},{"key":"2021120901553099300_R10","doi-asserted-by":"crossref","unstructured":"Nelson J. Incidence of the mucopolysaccharidoses in Northern Ireland. Hum Genet.\u20081997;101:355\u2013358","DOI":"10.1007\/s004390050641"},{"key":"2021120901553099300_R11","doi-asserted-by":"crossref","unstructured":"Baehner F, Schmiedeskamp C, Krummenauer F, et al. Cumulative incidence rates of the mucopolysaccharidoses in Germany. J Inherit Metab Dis.\u20082005;28:1011\u20131017","DOI":"10.1007\/s10545-005-0112-z"},{"key":"2021120901553099300_R12","doi-asserted-by":"crossref","unstructured":"Pinto R, Caseiro C, Lemos M, et al. Prevalence of lysosomal storage diseases in Portugal. Eur J Hum Genet.\u20082004;12:87\u201392","DOI":"10.1038\/sj.ejhg.5201044"},{"key":"2021120901553099300_R13","doi-asserted-by":"crossref","unstructured":"Poorthuis B, Wevers R, Kleijer W, et al. The frequency of lysosomal storage diseases in the Netherlands. Hum Genet.\u20081999;105:151\u2013156","DOI":"10.1007\/s004399900075"},{"key":"2021120901553099300_R14","doi-asserted-by":"crossref","unstructured":"Swiedler S, 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.\u20082005;134:144\u2013150","DOI":"10.1002\/ajmg.a.30579"},{"key":"2021120901553099300_R15","doi-asserted-by":"crossref","unstructured":"Coelho JC, Wajner M, Burin MG, Vargas CR, Giugliani R. Selective screening of 10,000 high-risk Brazilian patients for the detection of inborn errors of metabolism. Eur J Pediatr.\u20081997;156:650\u2013654","DOI":"10.1007\/s004310050685"},{"key":"2021120901553099300_R16","doi-asserted-by":"crossref","unstructured":"Karageorgos L, Brooks D, Pollard A, et al. Mutational analysis of 105 mucopolysaccharidosis type VI patients. Hum Mutat.\u20082007; In press","DOI":"10.1002\/humu.20534"},{"key":"2021120901553099300_R17","doi-asserted-by":"crossref","unstructured":"Simonaro C, Haskins M, Schuchman E. Articular chondrocytes from animals with a dermatan sulfate storage disease undergo a high rate of apoptosis and release nitric oxide and inflammatory cytokines: a possible mechanism underlying degenerative joint disease in the mucopolysaccharidoses. Lab Invest.\u20082001;81:1319\u20131328","DOI":"10.1038\/labinvest.3780345"},{"key":"2021120901553099300_R18","doi-asserted-by":"crossref","unstructured":"Simonaro C, D'Angelo M, Haskins M. Joint and bone disease in mucopolysaccharidoses VI and VII: identification of new therapeutic targets and biomarkers using animal models. Pediatr Res.\u20082005;57:701\u2013707","DOI":"10.1203\/01.PDR.0000156510.96253.5A"},{"key":"2021120901553099300_R19","doi-asserted-by":"crossref","unstructured":"Jezyk PF, Haskins ME, Patterson DF, Mellman WJ, Greenstein M. Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome. Science.\u20081977;198:834\u2013836","DOI":"10.1126\/science.144321"},{"key":"2021120901553099300_R20","doi-asserted-by":"crossref","unstructured":"Abreu S, Hayden J, Berthold P, et al. Growth plate pathology in feline mucopolysaccharidosis VI. Calcif Tissue Int.\u20081995;57:185\u2013190","DOI":"10.1007\/BF00310256"},{"key":"2021120901553099300_R21","doi-asserted-by":"crossref","unstructured":"Byers S, Crawley AC, Brumfield LK, Nuttall JD, Hopwood JJ. Enzyme replacement therapy in a feline model of MPS VI: modification of enzyme structure and dose frequency. Pediatr Res.\u20082000;47:743\u2013749","DOI":"10.1203\/00006450-200006000-00010"},{"key":"2021120901553099300_R22","doi-asserted-by":"crossref","unstructured":"Strauch OF, Stypmann J, Reinheckel T, Martinez E, Haverkamp W, Peters C. Cardiac and ocular pathologies in a mouse model of mucopolysaccharidosis type VI. Pediatr Res.\u20082003;54:701\u2013708","DOI":"10.1203\/01.PDR.0000084085.65972.3F"},{"key":"2021120901553099300_R23","doi-asserted-by":"crossref","unstructured":"Litjens T, Hopwood J. Mucopolysaccharidosis type VI: structural and clinical implications of mutations in N-acetylgalactosamine-4-sulfatase. Hum Mutat.\u20082001;18:282\u2013295","DOI":"10.1002\/humu.1190"},{"key":"2021120901553099300_R24","unstructured":"Human Gene Mutation Database. ARSB. Available at: www.hgmd.cf.ac.uk\/ac\/gene.php?gene=ARSB. Accessed March 12, 2007"},{"key":"2021120901553099300_R25","unstructured":"Jin WD, Jackson CE, Desnick RJ, Schuchman EH. Mucopolysaccharidosis type VI: identification of three mutations in the arylsulfatase B gene of patients with the severe and mild phenotypes provides molecular evidence for genetic heterogeneity. Am J Hum Genet.\u20081992;50:795\u2013800"},{"key":"2021120901553099300_R26","unstructured":"Litjens T, Brooks DA, Peters C, Gibson GJ, Hopwood JJ. Identification, expression and biochemical characterization of N-acetylgalactosamine-4-sulfatase mutations and relationship with clinical phenotype in MPS-VI patients. Am J Hum Genet.\u20081996;58:1127\u20131134"},{"key":"2021120901553099300_R27","doi-asserted-by":"crossref","unstructured":"Karageorgos L, Harmatz P, Simon J, et al. Mutational analysis of mucopolysaccharidosis type VI patients undergoing a trial of enzyme replacement therapy. Hum Mutat.\u20082004;23:229\u2013233","DOI":"10.1002\/humu.10313"},{"key":"2021120901553099300_R28","doi-asserted-by":"crossref","unstructured":"Karageorgos L, Brooks D, Harmatz P, et al. Mutational analysis of mucopolysaccharidosis type VI patients undergoing a phase II trial of enzyme replacement therapy. Mol Genet Metab.\u20082007;90:164\u2013170","DOI":"10.1016\/j.ymgme.2006.10.008"},{"key":"2021120901553099300_R29","doi-asserted-by":"crossref","unstructured":"Bradford TM, Litjens T, Parkinson EJ, Hopwood JJ, Brooks DA. Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): a Y210C mutation causes either altered protein handling or altered protein function of N-acetylgalactosamine-4-sulfatase at multiple points in the vacuolar network. Biochemistry.\u20082002;41:4962\u20134971","DOI":"10.1021\/bi0121149"},{"key":"2021120901553099300_R30","doi-asserted-by":"crossref","unstructured":"Whitley CB, Ridnour MD, Draper KA, Dutton CM, Neglia JP. Diagnostic test for mucopolysaccharidosis. I. Direct method for quantifying excessive urinary glycosaminoglycan excretion. Clin Chem.\u20081989;35:374\u2013379","DOI":"10.1093\/clinchem\/35.3.374"},{"key":"2021120901553099300_R31","doi-asserted-by":"crossref","unstructured":"Albano L, Sugayama S, Bertola D, et al. Clinical and laboratorial study of 19 cases of mucopolysaccharidoses. Rev Hosp Clin Fac Med Sao Paulo.\u20082000;55:213\u2013218","DOI":"10.1590\/S0041-87812000000600004"},{"key":"2021120901553099300_R32","doi-asserted-by":"crossref","unstructured":"Brooks DA, Gibson GJ, Karageorgos L, Hein LK, Robertson EF, Hopwood JJ. An index case for the attenuated end of the mucopolysaccharidosis type VI clinical spectrum. Mol Genet Metab.\u20082005;85:236\u2013238","DOI":"10.1016\/j.ymgme.2005.02.008"},{"key":"2021120901553099300_R33","doi-asserted-by":"crossref","unstructured":"Dierks T, Dickmanns A, Preusser-Kunze A, et al. Molecular basis for multiple sulfatase deficiency and catalytic mechanism for formylglycine generation of the human formylglycine-generating enzyme. Cell.\u20082005;121:541\u2013552","DOI":"10.1016\/j.cell.2005.03.001"},{"key":"2021120901553099300_R34","doi-asserted-by":"crossref","unstructured":"Hein L, Meikle P, Dean C, et al. Development of an assay for the detection of mucopolysaccharidosis type VI patients using dried blood-spots. Clin Chim Acta.\u20082005;353:67\u201374","DOI":"10.1016\/j.cccn.2004.10.009"},{"key":"2021120901553099300_R35","doi-asserted-by":"crossref","unstructured":"Krivit W. Allogeneic stem cell transplantation for the treatment of lysosomal and peroxisomal metabolic diseases. Springer Semin Immunopathol.\u20082004;26:119\u2013132","DOI":"10.1007\/s00281-004-0166-2"},{"key":"2021120901553099300_R36","doi-asserted-by":"crossref","unstructured":"Krivit W, Pierpont M, Ayaz K, et al. Bone marrow transplantation in the Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): biochemical and clinical status 24 months after transplantation. N Engl J Med.\u20081984;311:1606\u20131611","DOI":"10.1056\/NEJM198412203112504"},{"key":"2021120901553099300_R37","doi-asserted-by":"crossref","unstructured":"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 (rhASB) and follow-on open-label extension study. J Pediatr.\u20082006;148:533\u2013539","DOI":"10.1016\/j.jpeds.2005.12.014"},{"key":"2021120901553099300_R38","doi-asserted-by":"crossref","unstructured":"Hoogerbrugge P, Brouwer O, Bordigoni P, et al. Allogeneic bone marrow transplantation for lysosomal storage diseases. The European Group for Bone Marrow Transplantation. Lancet.\u20081995;345:1398\u20131403","DOI":"10.1016\/S0140-6736(95)92597-X"},{"key":"2021120901553099300_R39","doi-asserted-by":"crossref","unstructured":"Herskhovitz E, Young E, Rainer J, et al. Bone marrow transplantation for Maroteaux-Lamy syndrome (MPS VI): long-term follow-up. J Inherit Metab Dis.\u20081999;22:50\u201362","DOI":"10.1023\/A:1005447232027"},{"key":"2021120901553099300_R40","doi-asserted-by":"crossref","unstructured":"Summers C, Purple R, Krivit W, et al. Ocular changes in the mucopolysaccharidoses after bone marrow transplantation: a preliminary report. Ophthalmology.\u20081989;96:977\u2013984; discussion 984\u2013985","DOI":"10.1016\/S0161-6420(89)32795-3"},{"key":"2021120901553099300_R41","doi-asserted-by":"crossref","unstructured":"Gullingsrud E, Krivit W, Summers C. Ocular abnormalities in the mucopolysaccharidoses after bone marrow transplantation: longer follow-up. Ophthalmology.\u20081998;105:1099\u20131105","DOI":"10.1016\/S0161-6420(98)96014-6"},{"key":"2021120901553099300_R42","doi-asserted-by":"crossref","unstructured":"Staba S, Escolar M, Poe M, et al. Cord-blood transplants from unrelated donors in patients with Hurler's syndrome. N Engl J Med.\u20082004;350:1960\u20131969","DOI":"10.1056\/NEJMoa032613"},{"key":"2021120901553099300_R43","doi-asserted-by":"crossref","unstructured":"Peters C, Steward C. Hematopoietic cell transplantation for inherited metabolic diseases: an overview of outcomes and practice guidelines. Bone Marrow Transplant.\u20082003;31:229\u2013239","DOI":"10.1038\/sj.bmt.1703839"},{"key":"2021120901553099300_R44","unstructured":"Cerezyme [package insert]. Cambridge, MA: Genzyme Corp; 1999"},{"key":"2021120901553099300_R45","unstructured":"Fabrazyme [package insert]. Cambridge, MA: Genzyme Corp; 2003"},{"key":"2021120901553099300_R46","unstructured":"Myozyme [package insert]. Cambridge, MA: Genzyme Corp; 2006"},{"key":"2021120901553099300_R47","unstructured":"Aldurazyme [package insert]. Cambridge, MA; Genzyme Corp; 2003"},{"key":"2021120901553099300_R48","unstructured":"Elaprase [package insert]. Hampshire, United Kingdom: Shire Pharmaceuticals; 2006"},{"key":"2021120901553099300_R49","unstructured":"Naglazyme [package insert]. Novato, CA: BioMarin Pharmaceutical Inc; 2005"},{"key":"2021120901553099300_R50","doi-asserted-by":"crossref","unstructured":"Crawley A, Brooks D, Muller V, et al. Enzyme replacement therapy in a feline model of Maroteaux-Lamy syndrome. J Clin Invest.\u20081996;97:1864\u20131873","DOI":"10.1172\/JCI118617"},{"key":"2021120901553099300_R51","doi-asserted-by":"crossref","unstructured":"Crawley AC, Niedzielski KH, Isaac EL, Davey RC, Byers S, Hopwood JJ. Enzyme replacement therapy from birth in a feline model of mucopolysaccharidosis type VI. J Clin Invest.\u20081997;99:651\u2013662","DOI":"10.1172\/JCI119208"},{"key":"2021120901553099300_R52","doi-asserted-by":"crossref","unstructured":"Auclair D, Hopwood JJ, Brooks DA, Lemontt JF, Crawley AC. Replacement therapy in mucopolysaccharidosis type VI: advantages of early onset of therapy. Mol Genet Metab.\u20082003;78:163\u2013174","DOI":"10.1016\/S1096-7192(03)00007-6"},{"key":"2021120901553099300_R53","doi-asserted-by":"crossref","unstructured":"Harmatz P, Whitley C, Waber L, et al. Enzyme replacement therapy in mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). J Pediatr.\u20082004;144:574\u2013580","DOI":"10.1016\/j.jpeds.2004.03.018"},{"key":"2021120901553099300_R54","doi-asserted-by":"crossref","unstructured":"Harmatz P, Ketteridge D, Giugliani R, et al. Direct comparison of measures of endurance, mobility, and joint function during enzyme-replacement therapy of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): results after 48 weeks in a phase 2 open-label clinical study of recombinant human N-acetylgalactosamine-4-sulfatase. Pediatrics.\u20082005;115(6). Available at: www.pediatrics.org\/cgi\/content\/full\/115\/6\/e681","DOI":"10.1542\/peds.2004-1023"},{"key":"2021120901553099300_R55","doi-asserted-by":"crossref","unstructured":"Harmatz P, Kramer W, Hopwood J, et al. Pharmacokinetic profile of recombinant human N-acetylgalactosamine-4-sulfatase enzyme replacement therapy in patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): a phase I\/II study. Acta Paediatr Suppl.\u20082005;94:61\u201368; discussion 57","DOI":"10.1080\/08035320510028139"},{"key":"2021120901553099300_R56","doi-asserted-by":"crossref","unstructured":"Smith KS, Hallett KB, Hall RK, Wardrop RW, Firth N. Mucopolysaccharidosis: MPS VI and associated delayed tooth eruption. Int J Oral Maxillofac Surg.\u20081995;24:176\u2013180","DOI":"10.1016\/S0901-5027(06)80097-4"},{"key":"2021120901553099300_R57","doi-asserted-by":"crossref","unstructured":"Alpoz R, Coker M, Celen E, et al. The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod.\u20082006;101:632\u2013637","DOI":"10.1016\/j.tripleo.2005.06.023"},{"key":"2021120901553099300_R58","doi-asserted-by":"crossref","unstructured":"Simmons MA, Bruce IA, Penney S, Wraith E, Rothera MP. Otorhinolaryngological manifestations of the mucopolysaccharidoses. Int J Pediatr Otorhinolaryngol.\u20082005;69:589\u2013596","DOI":"10.1016\/j.ijporl.2005.01.017"},{"key":"2021120901553099300_R59","doi-asserted-by":"crossref","unstructured":"Semenza G, Pyeritz R. Respiratory complications of mucopolysaccharide storage disorders. Medicine (Baltimore).\u20081988;67:209\u2013219","DOI":"10.1097\/00005792-198807000-00002"},{"key":"2021120901553099300_R60","doi-asserted-by":"crossref","unstructured":"Leighton SE, Papsin B, Vellodi A, Dinwiddie R, Lane R, et al. Disordered breathing during sleep in patients with mucopolysaccharidoses. Int J Pediatr Otorhinolaryngol.\u20082001;58:127\u2013138","DOI":"10.1016\/S0165-5876(01)00417-7"},{"key":"2021120901553099300_R61","doi-asserted-by":"crossref","unstructured":"Wippermann CF, Beck M, Schranz D, Huth R, Michel-Behnke I, Jungst BK. Mitral and aortic regurgitation in 84 patients with mucopolysaccharidoses. Eur J Pediatr.\u20081995;154:98\u2013101","DOI":"10.1007\/BF01991908"},{"key":"2021120901553099300_R62","doi-asserted-by":"crossref","unstructured":"Azevedo A, Schwartz I, Kalakun L, et al. Clinical and biochemical study of 28 patients with mucopolysaccharidosis type VI. Clin Genet.\u20082004;66:208\u2013213","DOI":"10.1111\/j.1399-0004.2004.00277.x"},{"key":"2021120901553099300_R63","doi-asserted-by":"crossref","unstructured":"Hinek A, Wilson S. Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly. Am J Pathol.\u20082000;156:925\u2013938","DOI":"10.1016\/S0002-9440(10)64961-9"},{"key":"2021120901553099300_R64","doi-asserted-by":"crossref","unstructured":"Chan D, Li AM, Yam MC, Li CK, Fok TF. Hurler's syndrome with cor pulmonale secondary to obstructive sleep apnoea treated by continuous positive airway pressure. J Paediatr Child Health.\u20082003;39:558\u2013559","DOI":"10.1046\/j.1440-1754.2003.00218.x"},{"key":"2021120901553099300_R65","doi-asserted-by":"crossref","unstructured":"Dangel J. Cardiovascular changes in children with mucopolysaccharide storage diseases and related disorders: clinical and echocardiographic findings in 64 patients. Eur J Pediatr.\u20081998;157:534\u2013538","DOI":"10.1007\/s004310050872"},{"key":"2021120901553099300_R66","doi-asserted-by":"crossref","unstructured":"Brosius F III, Roberts W. Coronary artery disease in the Hurler syndrome: qualitative and quantitative analysis of the extent of coronary narrowing at necropsy in six children. Am J Cardiol.\u20081981;47:649\u2013653","DOI":"10.1016\/0002-9149(81)90550-6"},{"key":"2021120901553099300_R67","doi-asserted-by":"crossref","unstructured":"Braunlin E, Hunter D, Krivit W, et al. Evaluation of coronary artery disease in the Hurler syndrome by angiography. Am J Cardiol.\u20081992;69:1487\u20131489","DOI":"10.1016\/0002-9149(92)90908-H"},{"key":"2021120901553099300_R68","doi-asserted-by":"crossref","unstructured":"Taylor DB, Blaser SI, Burrows PE, Stringer DA, Clarke JT, Thorner P. Arteriopathy and coarctation of the abdominal aorta in children with mucopolysaccharidosis: imaging findings. AJR Am J Roentgenol.\u20081991;157:819\u2013823","DOI":"10.2214\/ajr.157.4.1909834"},{"key":"2021120901553099300_R69","unstructured":"Miller G, Partridge A. Mucopolysaccharidosis type VI presenting in infancy with endocardial fibroelastosis and heart failure. Pediatr Cardiol.\u20081983;4:61\u201362"},{"key":"2021120901553099300_R70","doi-asserted-by":"crossref","unstructured":"Tarantino M, Lail A, Donfield S, et al. Surveillance of infectious complications associated with central venous access devices in children with haemophilia. Haemophilia.\u20082003;9:588\u2013592","DOI":"10.1046\/j.1365-2516.2003.00793.x"},{"key":"2021120901553099300_R71","doi-asserted-by":"crossref","unstructured":"Tan CT, Schaff HV, Miller FA Jr, Edwards WD, Karnes PS. Valvular heart disease in four patients with Maroteaux-Lamy syndrome. Circulation.\u20081992;85:188\u2013195","DOI":"10.1161\/01.CIR.85.1.188"},{"key":"2021120901553099300_R72","doi-asserted-by":"crossref","unstructured":"Ashworth JL, Biswas S, Wraith E, Lloyd IC. The ocular features of the mucopolysaccharidoses. Eye.\u20082006;20:553\u2013563","DOI":"10.1038\/sj.eye.6701921"},{"key":"2021120901553099300_R73","doi-asserted-by":"crossref","unstructured":"Ashworth JL, Biswas S, Wraith E, Lloyd IC. Mucopolysaccharidoses and the eye. Surv Ophthalmol.\u20082006;51:1\u201317","DOI":"10.1016\/j.survophthal.2005.11.007"},{"key":"2021120901553099300_R74","doi-asserted-by":"crossref","unstructured":"Del Monte M, Maumenee I, Edwards R. Glycosaminoglycan degradation by cultured retinal pigment epithelium from patients with retinitis pigmentosa. Curr Eye Res.\u20081991;10:241\u2013248","DOI":"10.3109\/02713689109003446"},{"key":"2021120901553099300_R75","unstructured":"Leung L, Weinstein G, Hobson R. Further electroretinographic studies of patients with mucopolysaccharidoses. Birth Defects Orig Artic Ser.\u20081971;7:32\u201340"},{"key":"2021120901553099300_R76","doi-asserted-by":"crossref","unstructured":"Sato S, Maeda N, Watanabe H, et al. Multiple iridociliary cysts in patients with mucopolysaccharidoses. Br J Ophthalmol.\u20082002;86:933\u2013934","DOI":"10.1136\/bjo.86.8.933"},{"key":"2021120901553099300_R77","doi-asserted-by":"crossref","unstructured":"Vestermark S, Tonnesen T, Andersen MS, Guttler F. Mental retardation in a patient with Maroteaux-Lamy. Clin Genet.\u20081987;31:114\u2013117","DOI":"10.1111\/j.1399-0004.1987.tb02779.x"},{"key":"2021120901553099300_R78","unstructured":"Mabe P, Leistner S, Schwartz I, Matte U, Guigliani R. Mucopolysaccharidoses [in Spanish]. In: Colombo M, Cornejo V, Raimann E, eds. Inborn Errors in Pediatric Metabolism. Santiago, Chile: Editorial Universitaria; 2003:225\u2013256"},{"key":"2021120901553099300_R79","unstructured":"Schwartz I, Matte U, Leistner S, et al. Mucopolysaccharidoses [in Portugese]. In: Carakushansky G, ed. Genetic Diseases in Pediatrics. Rio de Janeiro, Brazil: Guanabara Koogan; 2001:180\u2013184"},{"key":"2021120901553099300_R80","doi-asserted-by":"crossref","unstructured":"Van Heest AE, House J, Krivit W, Walker K. Surgical treatment of carpal tunnel syndrome and trigger digits in children with mucopolysaccharide storage disorders. J Hand Surg [Am].\u20081998;23:236\u2013243","DOI":"10.1016\/S0363-5023(98)80120-2"},{"key":"2021120901553099300_R81","unstructured":"Schwartz GP, Cohen EJ. Hydrocephalus in Maroteaux-Lamy syndrome. Arch Ophthalmol.\u20081998;116:400"},{"key":"2021120901553099300_R82","doi-asserted-by":"crossref","unstructured":"Mut M, Cila A, Varli K, Akalan N. Multilevel myelopathy in Maroteaux-Lamy syndrome and review of the literature. Clin Neurol Neurosurg.\u20082005;107:230\u2013235","DOI":"10.1016\/j.clineuro.2004.05.003"},{"key":"2021120901553099300_R83","doi-asserted-by":"crossref","unstructured":"Boor R, Miebach E, Bruhl K, Beck M. Abnormal somatosensory evoked potentials indicate compressive cervical myelopathy in mucopolysaccharidoses. Neuropediatrics.\u20082000;31:122\u2013127","DOI":"10.1055\/s-2000-7495"},{"key":"2021120901553099300_R84","doi-asserted-by":"crossref","unstructured":"Walker R, Allen D, Rothera M. A fibreoptic intubation technique for children with mucopolysaccharidoses using the laryngeal mask airway. Paediatr Anaesth.\u20081997;7:421\u2013426","DOI":"10.1046\/j.1460-9592.1997.d01-102.x"},{"key":"2021120901553099300_R85","doi-asserted-by":"crossref","unstructured":"ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute-walk test. Am J Respir Crit Care Med.\u20082002;166:111\u2013117","DOI":"10.1164\/ajrccm.166.1.at1102"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/120\/2\/405\/1119764\/zpe00807000405.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/120\/2\/405\/1119764\/zpe00807000405.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,9]],"date-time":"2021-12-09T03:20:29Z","timestamp":1639020029000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/120\/2\/405\/70470\/Management-Guidelines-for-Mucopolysaccharidosis-VI"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,8,1]]},"references-count":85,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2007,8,1]]},"published-print":{"date-parts":[[2007,8,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2006-2184","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,8,1]]}}}