Which of the following statement is false about Hurler syndrome:
Correct Answer: X linked
Description: Ans. (a) X linkedRef: Harrison's 17th ed. Ch.DisorderEnzymeDef:InheritanceNeurologicOphthalmo- logicHematologicUnique FeaturesHurler syndromea-L- IduronidaseARMental retardation +Corneal cloudingVacuolated lymphocytesCoarse facies, cardiovascular involvement, joint stiffness, wide spaced teethHunter syndromeIduronate sulfatase (ET)X-linkedMental retardation, less in mild formRetinal degeneration, no corneal cloudingGranulated lymphocytesCoarse facies, cardiovascular, joint stiffness, distinctive pebbly skin lesions* Both have hepatosplenomegaly and skeletal dysplasia* In Hurler syndrome, death usually occurs by 10 years because of respiratory infection and heart failure.
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