A Pair of Unrelated 4-Year-Olds (Lester and Lucy) Have Mucopolysaccharidosis

question 7

Multiple Choice

A pair of unrelated 4-year-olds (Lester and Lucy) have mucopolysaccharidosis 1 (MPS1) . Lester has severe dysmorphic features and many skeletal anomalies. Lucyhas onlymildly coarse features and slight developmental delay. What is the best explanation for these differences?

• A)Skewed X inactivation allowed more paternal X expression for Lucy and more maternal X expression for Lester.
• B)Lucy's mother had better prenatal care, including good diet, exercise, and vitamins, than Lester's mother.
• C)It is likely that Lucy has been misdiagnosed and really has MPS2.
• D)The disorder has wide variability in expression of severity.

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