1. Glucose-6-phosphatase activity in homogenates of the livers of 2 infants with v. Gierke disease was found to be extremely low when compared to values obtained in other liver diseases or in normal livers.
2. In 1 of the 2 cases and in 4 controls the liver cells were counted. When the activity of the phosphatase was based on cell count it was found to be 12 times lower than the lowest and 37 times lower than the highest control value.
3. Phosphoglucomutase activity in the storage disease livers was within the range found in the controls whether based on weight or cell count.
4. The cell content of the liver of a 4-month fetus was found to be very high. The glucose-6-phosphatase activity per unit of weight was 9 times higher while per cell it was as low as in the v. Gierke liver. Phosphoglucomutase activity per unit of weight was within the normal range, per cell it was very low.
- Received July 14, 1954.
- Copyright © 1954 by the American Academy of Pediatrics