Hurler syndrome medical therapy: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Hurler syndrome}} | {{Hurler syndrome}} | ||
{{CMG}} | {{CMG}}; {{AE}} {{ADI}} | ||
==Overview== | ==Overview== | ||
| Line 10: | Line 8: | ||
==Medical Therapy== | ==Medical Therapy== | ||
===Enzyme Replacement Therapy=== | ===Enzyme Replacement Therapy=== | ||
* Patient is | * Patient is treated with the drug Laronidase, or Aldurazyme. | ||
* This is is a polymorphic variant of Alpha-L-iduronidase (lysosomal enzyme), produced by recombinant DNA technology. | * This is is a polymorphic variant of Alpha-L-iduronidase (lysosomal enzyme), produced by recombinant DNA technology. | ||
* This enzyme is required for the hydrolysis of terminal Alpha-L-iduronic acid residues of heparan and dermatan sulfate. | * This enzyme is required for the hydrolysis of terminal Alpha-L-iduronic acid residues of heparan and dermatan sulfate. | ||
* Enzyme replacement therapy is useful in dealing with problems of growth, breathing, heart and joints. | |||
* It is not proven effective in cases of neurological symptoms or mental development problems. | |||
* It is a good option for children with [[Scheie syndrome]] or [[Hurler-Scheie syndrome]] that does not cause [[mental retardation]]. | |||
==References== | ==References== | ||
{{Reflist|2}} | |||
[[Category:Gastroenterology]] | [[Category:Gastroenterology]] | ||
[[Category:Hepatology]] | [[Category:Hepatology]] | ||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
[[Category:Psychiatry]] | [[Category:Psychiatry]] | ||
[[Category:Disease]] | [[Category:Disease]] | ||
{{WS}} | |||
{{WH}} | |||
Latest revision as of 13:43, 19 July 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Govindavarjhulla, M.B.B.S. [2]
Overview
Treatment of Hurler syndrome involves supplementing the body with the deficient enzyme so that it can break down the GAG's. Enzyme replacement therapy has proven useful in reducing non-neurological symptoms and pain.
Medical Therapy
Enzyme Replacement Therapy
- Patient is treated with the drug Laronidase, or Aldurazyme.
- This is is a polymorphic variant of Alpha-L-iduronidase (lysosomal enzyme), produced by recombinant DNA technology.
- This enzyme is required for the hydrolysis of terminal Alpha-L-iduronic acid residues of heparan and dermatan sulfate.
- Enzyme replacement therapy is useful in dealing with problems of growth, breathing, heart and joints.
- It is not proven effective in cases of neurological symptoms or mental development problems.
- It is a good option for children with Scheie syndrome or Hurler-Scheie syndrome that does not cause mental retardation.