Abstract
Background: Regular carbohydrate intake to avoid hypoglycemia is the mainstay of dietary treatment in glycogen storage disease type I (GSDI). The aim of this study was to evaluate the quality of dietary treatment and glycemic control in a cohort of GSDI patients, in relation to the presence of typical long-term complications. Methods: Data of 25 patients (22 GSD subtype Ia and 3 GSDIb, median age 20y) from the Swiss hepatic glycogen storage disease registry was analyzed cross-sectionally. Frequency and type of hypoglycemia symptoms were assessed prospectively using a structured questionnaire. Continuous glucose monitoring (CGMS) was performed as part of usual clinical care to assess glycemic control in 14 patients. Results: Although maintenance of euglycemia is the primary goal of dietary treatment, few patients (n=3, 13%) performed capillary blood glucose measurements regularly. Symptoms of hypoglycemia were present in 13 patients (57 %), but CGMS revealed periods of low glucose (<4mmol/l) in all patients, irrespective of the presence of symptoms. GSDIa patients with liver adenomas (n=9, 41%) showed a higher frequency and area under the curve (AUC) of low blood glucose than patients without adenomas (frequency 2.7±0.8 vs. 1.5±0.7 per day, AUC 0.11±0.08 vs. 0.03±0.02 mmol/l/d, p<0.05). The presence of microalbuminuria was also related to the frequency of low blood glucose. Z-Scores of bone density correlated negatively with lactate levels. Conclusion: The quality of glucose control is related to the presence of typical long-term complications in GSDI. Many patients experience episodes of asymptomatic low blood glucose. Regular assessment of glucose control is an essential element to evaluate the quality of treatment, and increasing the frequency of glucose self-monitoring remains an important goal of patient education and motivation. CGMS devices may support patients to optimize dietary therapy in everyday life.
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