Dietary Management of the Ketogenic Glycogen Storage Diseases

Bhattacharya, Kaustuv; Pontin, Jennifer; Thompson, Sandra A.

doi:10.1177/2326409816661359

Cited by 8 publications

(3 citation statements)

References 45 publications

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“…Renewable energy sources are relatively inexpensive, which encourages companies to employ them. Bhattacharya et al noted that the only approach to reduce environmental consequences is to implement alternative energy solutions, which falls under the sphere of green regulations (Bhattacharya et al, 2016).…”

Section: Literature Review and Hypothesesmentioning

confidence: 99%

Dynamic association between socio-economic, environmental and logistic operations: Evidence from SSA BRI host countries

Aden

Zheng²,

Almoshaogeh³

et al. 2022

Front. Environ. Sci.

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This study examines the connection among green logistic operations, countries-level economic, environmental, and social indicators in Sub-Saharan Africa (SSA) Belt and Road Countries. Using the system generalized method of moments (S-GMM) estimator, this study analyses annual data from 2008 to 2018 and offers three key findings. First, economic indicators China’s foreign direct investment (FDI), trade openness and economic output) are positively associated with green logistic operations. Second, logistics are positively correlated with renewable energy while inversely correlated with carbon emissions. Third, social indicators are also directly associated with green logistic operations measured through health expenditure and institutional quality. Lastly, information communication technology also spurs green logistic operations. Manifestly, Chinese outbound FDI in SSA substantially improved the quality of their logistics in terms of infrastructure, cost, time, customs services, tracking, and the consistency of international shipments. These findings show that green logistics provide adequate infrastructure, and supply chain partners share information more frequently, increasing trade volume, growth potential, and environmental sustainability. Similar results are also endorsed using a feasible generalized least square (FGLS) estimator and suggest that SSA should take effective measures to improve their logistics operation.

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Section: Literature Review and Hypothesesmentioning

confidence: 99%

Dynamic association between socio-economic, environmental and logistic operations: Evidence from SSA BRI host countries

Aden

Zheng²,

Almoshaogeh³

et al. 2022

Front. Environ. Sci.

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“…(), Activates or leads to; (), suppresses. (Adapted from Bhattacharya et al ., 2 with permission. )…”

Section: What Is Already Known On This Topicmentioning

confidence: 99%

Investigating paediatric hypoglycaemia: Dynamic studies at a tertiary paediatric hospital

Graves

Stewart

Ambler

et al. 2021

J Paediatrics Child Health

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Aim Paediatric hypoglycaemia often requires specific investigations to determine aetiology. Samples from the time of hypoglycaemia may not be available and a diagnostic fasting test may be required. Additionally, fasting studies can determine safe fasting intervals and prolonged oral glucose challenges can assess hypoglycaemia due to abnormal post‐prandial glucose handling. This audit reviewed the current utility and yield of fasting studies, prolonged oral glucose challenges and starch loads. Methods Retrospective audit of clinical record to determine purpose and outcome of tests performed at a Tertiary Paediatric Endocrine/Metabolic Testing Unit in Sydney, Australia, from 2013 to 2018 inclusive. Results One hundred and thirty‐eight children (aged 3 weeks–17 years) underwent 170 tests: 122 fasting studies, 20 five‐hour OGTTs, 22 uncooked corn starch loads and six modified waxy maize starch (Glycosade) loads. The majority were for diagnostic purposes (n = 113, 66%), with 57 (34%) to guide management in patients with known diagnoses. Following diagnostic studies, 35 (31%) patients received a pathological diagnosis, the most common of which (n = 19, 17%) was accelerated starvation. Hypoglycaemia developed in n = 15/113 (13%) during the diagnostic studies. Management studies helped determine length of safe fast, adjustment of medication or diet and document resolution of pathology. Conclusion Fasting studies remain a safe and effective method to assist with diagnoses, confirm or exclude pathological causes of childhood hypoglycaemia and to guide management of known diagnoses in the paediatric population.

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“…Although carbohydrates, particularly glucose, are not ordinarily required in the diet, they may be required exogenously in certain populations who do not synthesise them in adequate amounts due to a genetic defect or pathological state. For example, glycogen storage disease type I (von Gierke disease) results from a genetic defect in glucose-6-phosphatase, thereby preventing the liver from releasing glucose from gluconeogenesis and glycogenolysis into the blood stream (92,93) . Therefore, although ketosis can lower the requirement for glucose, the body may still be unable to produce and utilise a sufficient amount of glucose without exogenous intake of carbohydrate depending on the severity of the glucose-6-phosphatase enzyme defect.…”

Section: Populations Where Dietary Carbohydrates May Be Conditionallymentioning

confidence: 99%

Application of nutrient essentiality criteria to dietary carbohydrates

2020

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The purpose of the present review is to describe how human physiology at very low carbohydrate intakes relates to the criteria for nutritional essentiality. Although we did not limit ourselves to one particular type or function of carbohydrates, we did primarily focus on glucose utilisation as that function was used to determine the recommended daily allowance. In the general population, the human body is able to endogenously synthesise carbohydrates, and does not show signs of deficiency in the absence of dietary carbohydrates. However, in certain genetic defects, such as glycogen storage disease type I, absence of dietary carbohydrates causes abnormalities that are resolved with dietary supplementation of carbohydrates. Therefore, dietary carbohydrates may be defined as conditionally essential nutrients because they are nutrients that are not required in the diet for the general population but are required for specific subpopulations. Ketosis may be considered a physiological normal state due to its occurrence in infants in addition to at very low carbohydrate intakes. Although sources of dietary carbohydrates can provide beneficial micronutrients, no signs of micronutrient deficiencies have been reported in clinical trials of low-carbohydrate ketogenic diets. Nonetheless, more research is needed on how micronutrient requirements can change depending on the dietary and metabolic context. More research is also needed on the role of dietary fibre during a low-carbohydrate ketogenic diet as the beneficial effects of dietary fibre were determined on a standard diet and several studies have shown beneficial effects of decreasing non-digestible carbohydrates.

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Dietary Management of the Ketogenic Glycogen Storage Diseases

Cited by 8 publications

References 45 publications

Dynamic association between socio-economic, environmental and logistic operations: Evidence from SSA BRI host countries

Dynamic association between socio-economic, environmental and logistic operations: Evidence from SSA BRI host countries

Investigating paediatric hypoglycaemia: Dynamic studies at a tertiary paediatric hospital

Application of nutrient essentiality criteria to dietary carbohydrates

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