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Referral is an essential part of MPS VI management14

All patients suspected of having MPS VI should be referred to a geneticist for definitive diagnosis and appropriate counseling.14

A multidisciplinary effort

MPS VI patients are at increased risk for14:

  • Valvular heart disease: refer to a cardiologist
  • Conductive and sensorineural hearing loss: refer to an audiologist for periodic hearing evaluations
  • Corneal clouding, and eventual significant visual impairment: refer to an ophthalmologist for initial and follow-up slitlamp and funduscopic exams
  • Spinal cord compression secondary to thickening of the dura in the cervical canal (leads to myelopathy): refer to a neurosurgeon or orthopodedist
  • Airway obstruction, hypoventilation, sleep apnea: refer to a pulmonologist for regular evaluation

 Next: Managing MPS VI with NAGLAZYME

References

  1. NAGLAZYME [Prescribing Information]. Novato, CA: Biomarin; 2005.
  1. 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 (recombinant human arylsulfatase B or rhASB) and follow-on, open-label extension study. J Pediatr. 2006;148:533-539.
  1. Harmatz RP. Mucopolysaccharidosis type VI. Emedicine. December 18, 2006. Available at: http://www.Emedicine.com/PED/topic1373.htm Accessed: November 4, 2008.
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