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Glycogen storage disease type Ia (GSDIa) is caused by defective glucose-6-phosphatase, a key enzyme in carbohydrate metabolism. Affected individuals cannot release glucose during fasting and accumulate excess glycogen and fat in the liver and kidney, putting them at risk of severe hypoglycaemia and secondary metabolic perturbations. Good glycaemic/metabolic control through strict dietary treatment and regular doses of uncooked cornstarch (UCCS) is essential for preventing hypoglycaemia and long-term complications. Dietary treatment has improved the prognosis for patients with GSDIa; however, the disease itself, its management and monitoring have significant physical, psychological and psychosocial burden on individuals and parents/caregivers. Hypoglycaemia risk persists if a single dose of UCCS is delayed/missed or in cases of gastrointestinal intolerance. UCCS therapy is imprecise, does not treat the cause of disease, may trigger secondary metabolic manifestations and may not prevent long-term complications. We review the importance of and challenges associated with achieving good glycaemic/metabolic control in individuals with GSDIa and how this should be balanced with age-specific psychosocial development towards independence, management of anxiety and preservation of quality of life (QoL). The unmet need for treatment strategies that address the cause of disease, restore glucose homeostasis, reduce the risk of hypoglycaemia/secondary metabolic perturbations and improve QoL is also discussed.

Hypoglycemia is a pathological condition characterized by a low plasma glucose concentration associated with typical autonomic and/or neuroglycopenic symptoms, and resolution of these symptoms with carbohydrate consumption. Hypoglycemia is quite common in clinical practice, particularly in insulin-treated patients with diabetes and in other inherited or acquired conditions involving the regulation of glucose metabolism. Beyond symptoms that might strongly affect the quality of life, hypoglycemia can lead to short- and long-term detrimental consequences for health. Hypoglycemia can be prevented by appropriate changes in dietary habits or by relevant modifications of the drug treatment. Several dietary approaches based on the intake of various carbohydrate foods have been tested for hypoglycemia prevention; among them uncooked cornstarch (UCS) has demonstrated a great efficacy. In this narrative review, we have summarized the current evidence on the UCS usefulness in some conditions characterized by high hypoglycemic risk, focusing on some inherited diseases −i.e. glycogen storage diseases and other rare disorders − and acquired conditions such as type 1 diabetes, postprandial hypoglycemia consequent to esophageal-gastric or bariatric surgery, and insulin autoimmune syndrome. We also considered the possible role of UCS during endurance exercise performance. Lastly, we have discussed the dose requirement, the side effects, the limitations of UCS use, and the plausible mechanisms by which UCS could prevent hypoglycemia.

Introduction: Among persons living with type 1-diabetes hypoglycemia and fear of hypoglycemia remain limiting barriers for achieving optimal glucose control and a good quality of life. Fear of hypoglycemia has been found stable over time if not treated. Uncooked cornstarch has been found to reduce the risk of hypoglycemia but has not been studied in relation to fear of hypogly-cemia. The aims of this study were to through clinical data, self-reported measures and clinical interviews explore subjects’ experience of using un-cooked cornstarch before bedtime and their beliefs and experiences of fear of hypoglycemia. Methods: Mixed methods with both quantitative and qualita-tive data were used. Self-reported measures of hypoglycemia and fear of hy-poglycemia were compared to subjects’ responses during a clinical interview. The interviews were analyzed with a functional behavior analytical approach. Results: A total of five subjects took part in the study. One subject perceived the uncooked cornstarch helpful in reducing hypoglycemia. Several subjects could recall frightening hypoglycemic episodes triggering their fear. Three out of the five subjects reported avoidance behaviors such as excessive self-monitoring of blood glucose or overeating related to fear of hypoglyce-mia. Conclusions: The uncooked cornstarch was found appetizing but was not perceived as having an effect on BG or hypoglycemia frequency. The clinical interviews confirmed previous research regarding experience of hy-poglycemia and fear of hypoglycemia.