Q:

Enzyme replacemen is the main therapy of Hurler disease, it improve all the following EXCEPT

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Enzyme replacemen is the main therapy of Hurler disease, it improve all the following EXCEPT


  1. growth rate
  2. joint mobility
  3. organomegaly
  4. cognition functions
  5. episodes of sleep apnea

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(D). Enzyme replacement using recombinant a-L-iduronidase has been approved for patients with MPS-l. It reduces organomegaly and ameliorates rate of growth, joint mobility, reduces the number of episodes of sleep apnea and urinary GAG excretion. The enzyme does not cross the blood-brain barrier and does not prevent deterioration of cognition and other neurologic functions

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