It was found in the early phases of this study that oral uncooked cornstarch could sustain a normal blood glucose in patients with type I GSD whose liver glucose production was inadequate. The associated findings of GSD, e.g., growth failure, hypoglycemia, liver adenomata, could be corrected or improved with this regimen. A number of different types of starch and starch regimens were tried but almost without exception uncooked cornstarch provided the best results. As more patients lived longer, more associated disorders began to appear. It became apparent that focal glomerular sclerosis was very frequent in older individuals and it led to death with renal failure. Additional associated conditions which have since been added to the list include renal stones, generalized demineralization, anemia, abnormal granulocyte function, central ovarian failure, primary pulmonary hypertension, and amyloid deposition. This facet of the disease became much more compelling and it was felt that the cornstarch treatment phase was completed with worldwide acceptance as the treatment of choice. Hence, the protocol was discontinued.
