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Review Question - QID 106256

Lysosome Other Lysosomal Disorders I (inclusion)-cell disease QID: 106256 (M1.BC.14.1)
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QID 106256 (Type "106256" in App Search)
An 8-month-old female infant from a first-degree consanguinous couple was brought to the physican because the mother noticed abnormalities in the growth of her child as well as the different lengths of her child's legs. The infant had gingival hyperplasia, restricted movement in both shoulders, a prominent, pointed forehead, and enophthalmos with a slight opacity in both corneas. A blood test revealed 10 fold higher than normal levels of the following enzymes: N-acetyl-ß-glucosaminidase, ß-glucuronidase, ß-hexosaminidase A, and alkaline phosphatase. Which of the following is most likely deficient in this patient?
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