Studies in Glycogen Storage Diseases. I. Intestinal Glucose-6-Phosphatase Activity in Patients with Von Gierke's Disease and Their Parents*

These studies also emphasize the importance of measuring nonspecific phosphatase in biopsy specimens and correcting the apparent glucose-6-phosphatase activity for this before concluding that a deficiency of glucose-6-phosphatase does not ex- ist. The failure to make this correction can probably explain those cases in the literature that clini- cally were typical of Von Gierke's disease but had significant amounts of hepatic glucose-6-phosphatase activity (3-5). Methods Clinical material. The case histories of the two pa-' ~/0 _ //~~~~~~X ~~0 /tag 5~~/ ~~~~~~_ /~~~~~~, _ _~~~~~z --eof ,-I/