04-02-2009, 03:28 PM
A 10 month old male baby dies, and on autopsy is found to have hepatomegaly with fatty infiltration, splenomegaly, and calcified adrenal cortices. He was ok after birth for about 3-4 months, after which he regressed neurologically, failed to thrive, became anemic and had steatorrhea. This rare AR lipid storage disease was caused by a deficiency of:
1. beta galactosidase
2. alpha iduronidase
3. acid lipase
4. arylsulfatase A
1. beta galactosidase
2. alpha iduronidase
3. acid lipase
4. arylsulfatase A