Dietary treatment of glycogen storage disease type Ia: Uncooked cornstarch and/or continuous nocturnal gastric drip-feeding?

Increased plasma ACs and abnormal UOA profile suggest mitochondrial impairment in GSDIa. Correlation data suggest a possible connection between mitochondrial impairment and IR. We hypothesized that mitochondrial overload might generate by-products potentially affecting the insulin signaling pathway, leading to IR. On the basis of the available data, the possible pathomechanism for IR in GSDIa is proposed.

Section: Dietary Considerationsmentioning

confidence: 99%

mentioning

confidence: 99%

Insulin‐resistance in glycogen storage disease type Ia: linking carbohydrates and mitochondria?

Rossi

Ruoppolo

Formisano

et al. 2018

“…Although UCCS may be introduced at 6 months of age, the tolerance may be reduced as a consequence of lower pancreatic amylase activity until 1 year of age (Hayde and Widhalm 1990). Both CNGDF and UCCS have advantages and disadvantages and differ in many ways, like the optimal age of introduction, duration of normoglycemia, (no) need to wake up during the night, caloric content, glycemic index, risk of technical failure with subsequent metabolic risks, difficulty for parents and/or patients, invasiveness and costs (Derks et al 2013). Dietary management in GSD III patients follows similar principles of exogenous carbohydrates, but the additional role of increasing dietary protein is recognized.…”

Section: Introductionmentioning

confidence: 99%

Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions

Derks

Rijn

2015

Self Cite

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.

“…In the 1980s, uncooked cornstarch (UCCS) was introduced as an alternative to extend the fasting period during the night (Chen et al 1984). We have previously discussed the advantages and disadvantages of both nocturnal treatments (Derks et al 2013). Recently, a modified starch was developed, and the first clinical studies report a longer maintenance of normoglycemia with the modified starch compared with UCCS (Bhattacharya et al 2007;Correia et al 2008).…”

Section: Introductionmentioning

confidence: 99%

In vitro digestion of starches in a dynamic gastrointestinal model: an innovative study to optimize dietary management of patients with hepatic glycogen storage diseases

Nalin

Venema

Weinstein

et al. 2014

Self Cite

Uncooked cornstarch (UCCS) is a widely used treatment strategy for patients with hepatic glycogen storage disease (GSD). It has been observed that GSD-patients display different metabolic responses to different cornstarches. The objective was to characterize starch fractions and analyze the digestion of different starches in a dynamic gastrointestinal in vitro model. The following brands of UCCS were studied: Argo and Great Value from the United States of America; Brazilian Maizena Duryea and Yoki from Brazil; Dutch Maizena Duryea from the Netherlands. Glycosade, a modified starch, and sweet polvilho, a Brazilian starch extracted from cassava, were also studied. The starch fractions were analyzed by glycemic TNO index method and digestion analyses were determined by the TIM-1 system, a dynamic, computer-controlled, in vitro gastrointestinal model, which simulates the stomach and small intestine. The final digested amounts were between 84 and 86% for the UCCS and Glycosade, but was 75.5% for sweet povilho. At 180 min of the experiment, an important time-point for GSD patients, the digested amount of the starches corresponded to 67.9-71.5 for the UCCS and Glycosade, while it was 55.5% for sweet povilho. In an experiment with a mixture of sweet polvilho and Brazilian Maizena Duryea, a final digested amount of 78.4% was found, while the value at 180 min was 61.7%. Sweet polvilho seems to have a slower and extended release of glucose and looks like an interesting product to be further studied as it might lead to extended normoglycemia in GSD-patients.