BIOCHEMICAL-EVIDENCE FOR THE REQUIREMENT OF CONTINUOUS GLUCOSE THERAPY IN YOUNG-ADULTS WITH TYPE-1 GLYCOGEN-STORAGE-DISEASE

To determine whether patients with GSD-1 need nocturnal glucose therap y after completing physical growth and development, studies were perfo rmed on two consecutive nights. On the first night uncooked cornstarch (UCS) was given at the calculated glucose production rate at 21:00 h and 02:00 h. On the second night UCS was given at 21:00 h but omitted at 02:00 h. Six GSD-1 patients, aged 17.2-20.9 years, previously treat ed with continuous glucose therapy were studied. Measurements were mad e of plasma glucose (PG), serum insulin, growth hormone, cortisol, pla sma glucagon (n = 4), and blood lactate at 30-60-min intervals. Serum uric acid, cholesterol, and triglycerides were measured at 21:00 h and 07:00 h, and serum FFA at 21:00 h, 02:00 h and 07:00 h on the first n ight and immediately before treatment for hypoglycaemia on the second night. For five hours after UCS at 21:00h, mean PG, serum insulin and blood lactate concentrations were similar on the two nights. With UCS at 02:00h, mean PG concentrations were greater than or equal to 4.1mmo l/L from 02:00 to 07:00h. Without UCS at 02:00h, in all subjects PG co ncentrations fell to < 2.5 mmol/L after 6.5-8.5 h and mean blood lacta te concentration increased to 7.4 +/- 3.0 mmol/L. Young adults with GS D-1 developed hypoglycaemia and hyperlactataemia after a relatively br ief period without exogenous glucose and, therefore, need to continue nocturnal glucose therapy to prevent fasting hypoglycaemia.