A 4-year-old boy is brought to the physician for a follow-up appointment. Otoscopy shows a small amount of fluid behind the right tympanic membrane. He recovered from an episode of otitis media 3 weeks ago. This is his third episode of otitis media in the past 2 years. Vitals are normal. Physical examination shows hepatomegaly, coarse facial features, and joint contractures. The enzyme deficiency responsible for this patient’s condition is normally present at the organelle shown in the following electron micrograph.
Which of the following is most likely to be seen in this patient?
Avascular necrosis of the femoral head
Arylsulfatase A deficiency
Defective phosphorylation of the 6th position on mannose
Galactocerebroside and psychosine accumulation
Hexosaminidase A deficiency
Sphingomyelin accumulation
