Abstract:we present an accurate non-linear regression model for EGP as a function of age. With standard dextrose-based saline solutions infused at fluid maintenance rate, only approximately 50% or less of EGP is provided. With prolonged infusion of these solutions, the deficit between exogenous glucose supplementation and EGP may induce a catabolic state and may ultimately lead to hypoglycaemia, especially in younger children.
“…The mechanism is not understood, theoretically, residual in vivo glucose-6-phosphatase-α activity next to alternative glycogenolysis by (muscle) glucose-6-phosphatase-β and/or the α-glucosidase pathway may contribute to whole body EGP in GSD Ia patients.Fig. 1Two regression models to calculate endogenous glucose production, based on body weight ( grey , by Bier et al 1977) and age ( black , by Huidekoper et al 2014). The grey line represents the EGP regression line according to Bier.…”
Section: The Role Of Dietary Carbohydrate Fat and Proteinmentioning
confidence: 99%
“…Two regression models to calculate endogenous glucose production, based on body weight ( grey , by Bier et al 1977) and age ( black , by Huidekoper et al 2014). The grey line represents the EGP regression line according to Bier.…”
Section: The Role Of Dietary Carbohydrate Fat and Proteinmentioning
confidence: 99%
“…Experimental data from stable isotope studies demonstrate that EGP is age dependent and decreases relatively with body weight (Bier et al 1977) and age (Huidekoper et al 2014). Because in GSD I patients both glycogenolysis and GNG are impaired, defective EGP and fasting intolerance are more pronounced and lifelong, compared to the ketotic GSD types like GSD III.…”
Section: Pathophysiology Of Lipid Metabolism In Hepatic Gsdmentioning
confidence: 99%
“…Several case reports describe the effects of medium chain triglycerides (MCT) in GSD I and III (Fernandes and Pikaar 1969;Cuttino et al 1970 Two regression models to calculate endogenous glucose production, based on body weight (grey,b yB i e re ta l1977) and age (black, by Huidekoper et al 2014). The grey line represents the EGP regression line according to Bier.…”
Section: The Role Of Dietary Carbohydrate Fat and Proteinmentioning
Hepatic glycogen storage diseases (GSD) underscore the intimate relationship between carbohydrate and lipid metabolism. The hyperlipidemias in hepatic GSD reflect perturbed intracellular metabolism, providing biomarkers in blood to monitor dietary management. In different types of GSD, hyperlipidemias are of a different origin. Hypertriglyceridemia is most prominent in GSD type Ia and associated with long-term outcome morbidity, like pancreatitis and hepatic adenomas. In the ketotic subtypes of GSD, hypertriglyceridemia reflects the age-dependent fasting intolerance, secondary lipolysis and increased mitochondrial fatty acid oxidation. The role of high protein diets is established for ketotic types of GSD, but non-traditional dietary interventions (like medium-chain triglycerides and the ketogenic diet) in hepatic GSD are still controversial and necessitate further studies. Patients with these rare inherited disorders of carbohydrate metabolism meet several criteria of the metabolic syndrome, therefore close monitoring for cardiovascular diseases in ageing GSD patients may be justified.
“…The mechanism is not understood, theoretically, residual in vivo glucose-6-phosphatase-α activity next to alternative glycogenolysis by (muscle) glucose-6-phosphatase-β and/or the α-glucosidase pathway may contribute to whole body EGP in GSD Ia patients.Fig. 1Two regression models to calculate endogenous glucose production, based on body weight ( grey , by Bier et al 1977) and age ( black , by Huidekoper et al 2014). The grey line represents the EGP regression line according to Bier.…”
Section: The Role Of Dietary Carbohydrate Fat and Proteinmentioning
confidence: 99%
“…Two regression models to calculate endogenous glucose production, based on body weight ( grey , by Bier et al 1977) and age ( black , by Huidekoper et al 2014). The grey line represents the EGP regression line according to Bier.…”
Section: The Role Of Dietary Carbohydrate Fat and Proteinmentioning
confidence: 99%
“…Experimental data from stable isotope studies demonstrate that EGP is age dependent and decreases relatively with body weight (Bier et al 1977) and age (Huidekoper et al 2014). Because in GSD I patients both glycogenolysis and GNG are impaired, defective EGP and fasting intolerance are more pronounced and lifelong, compared to the ketotic GSD types like GSD III.…”
Section: Pathophysiology Of Lipid Metabolism In Hepatic Gsdmentioning
confidence: 99%
“…Several case reports describe the effects of medium chain triglycerides (MCT) in GSD I and III (Fernandes and Pikaar 1969;Cuttino et al 1970 Two regression models to calculate endogenous glucose production, based on body weight (grey,b yB i e re ta l1977) and age (black, by Huidekoper et al 2014). The grey line represents the EGP regression line according to Bier.…”
Section: The Role Of Dietary Carbohydrate Fat and Proteinmentioning
Hepatic glycogen storage diseases (GSD) underscore the intimate relationship between carbohydrate and lipid metabolism. The hyperlipidemias in hepatic GSD reflect perturbed intracellular metabolism, providing biomarkers in blood to monitor dietary management. In different types of GSD, hyperlipidemias are of a different origin. Hypertriglyceridemia is most prominent in GSD type Ia and associated with long-term outcome morbidity, like pancreatitis and hepatic adenomas. In the ketotic subtypes of GSD, hypertriglyceridemia reflects the age-dependent fasting intolerance, secondary lipolysis and increased mitochondrial fatty acid oxidation. The role of high protein diets is established for ketotic types of GSD, but non-traditional dietary interventions (like medium-chain triglycerides and the ketogenic diet) in hepatic GSD are still controversial and necessitate further studies. Patients with these rare inherited disorders of carbohydrate metabolism meet several criteria of the metabolic syndrome, therefore close monitoring for cardiovascular diseases in ageing GSD patients may be justified.
“…Thus, recommendations vary widely and for adults there is no clear consensus, which reflects the paucity of data in this patient group. There is however a clear relation between increasing endogenous glucose production (and thus need) during fasting, ranging from above 8 mg/kg/min in the newborn to 2 mg/kg/min in the adult human (Huidekoper et al 2014). In our view, the easiest way to prescribe glucose for the adult patient is to use a fixed 10% solution and give 2 mg/kg/min.…”
Section: Glucose Infusion and Laboratory Measurementsmentioning
Surgery and anesthesia pose a threat to patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), because prolonged fasting, stress, and pain are known risk factors for the induction of metabolic derangement. The optimal perioperative management in these patients is unknown and the use of volatile agents and agents dissolved in fatty acids has been related to postoperative metabolic complications. However, the occurrence of metabolic derangement is multifactorial and depends, amongst others, on the severity of the mutation and residual enzyme activity. Current guidelines suggest avoiding both volatile anesthetics as well as propofol, which seriously limits the options for providing safe anesthesia. Therefore, we reviewed the available literature on the perioperative management of patients with VLCADD. We concluded that the use of some medications, such as volatile anesthetics, in patients with VLCADD might be wrongfully avoided and could in fact prevent metabolic derangement by the adequate suppression of pain and stress during surgery. We will illustrate this with a case report of an adult VLCADD patient undergoing minor surgery. Besides the use of remifentanil, anesthesia was uneventfully maintained with the use of sevoflurane, a volatile agent, and continuous glucose infusion. The patient was monitored with a continuous glucose meter and creatinine kinase measurements.
Continuous glucose monitoring (CGM) systems have great potential for real‐time assessment of glycemic variation in patients with hepatic glycogen storage disease (GSD). However, detailed descriptions and in‐depth analysis of CGM data from hepatic GSD patients during interventions are scarce. This is a retrospective in‐depth analysis of CGM parameters, acquired in a continuous, real‐time fashion describing glucose management in 15 individual GSD patients. CGM subsets are obtained both in‐hospital and at home, upon nocturnal dietary intervention (n = 1), starch loads (n = 11) and treatment of GSD Ib patients with empagliflozin (n = 3). Descriptive CGM parameters, and parameters reflecting glycemic variation and glycemic control are considered useful CGM outcome parameters. Furthermore, the combination of first and second order derivatives, cumulative sum and Fourier analysis identified both subtle and sudden changes in glucose management; hence, aiding assessment of dietary and medical interventions. CGM data interpolation for nocturnal intervals reduced confounding by physical activity and diet. Based on these analyses, we conclude that in‐depth CGM analysis can be a powerful tool to assess glucose management and optimize treatment in individual hepatic GSD patients.
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