Disorders of Carbohydrate Metabolism

Dixon, Marjorie; MacDonald, Anita

doi:10.1002/9780470692004.ch18

Clinical Paediatric Dietetics 2007

DOI: 10.1002/9780470692004.ch18

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Disorders of Carbohydrate Metabolism

Marjorie Dixon¹,

Anita MacDonald²

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Cited by 2 publications

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Dietary dilemmas in the management of glycogen storage disease type I

Bhattacharya

2011

J of Inher Metab Disea

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Over the last 50 years, understanding the biochemical bases of glycogen storage disease type I has led to vastly improved survival and health outcomes but the management still centres around an extremely intensive dietary regimen. Patients' metabolic profiles are really determined by the whole of the diet and it can be very difficult to adjust therapy accordingly. In an iso-energetic diet with reference total energy intake, high carbohydrate intake could compromise other macro- and micro-nutrients; if carbohydrates are not restricted then total energy intake is excessive. The quality of the macronutrient such as the glycemic index of carbohydrate, the type of sugar and the proportion of medium-chain triglyceride and essential fatty acids also has a bearing on an individual's long-term metabolic control with potential clinical correlates. These factors as well as the different requirements between individuals and within individuals as they get older mean that the management of glycogen storage disease type I is particularly fraught. Regular clinical and dietary review is imperative as patients grow, ensuring adequate but not excessive low glycaemic index carbohydrate intake, appropriate dynamic biochemical profiles and suitable age appropriate eating patterns. Without diligent management, and education that empowers the patient, these individuals can struggle in adult life.

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Dietary dilemmas in the management of glycogen storage disease type I

Bhattacharya

2011

J of Inher Metab Disea

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A pilot longitudinal study of the use of waxy maize heat modified starch in the treatment of adults with glycogen storage disease type I: a randomized double-blind cross-over study

Bhattacharya

Mundy

Lilburn

et al. 2015

Orphanet Journal of Rare Diseases

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BackgroundUncooked corn-starch (UCCS) has been the mainstay of therapy for the hepatic glycogen storage diseases (GSD) but is not always effective. A new starch (WMHMS) has demonstrated a more favourable short-term metabolic profile.ObjectiveTo determine efficacy and safety of a new uncooked starch (WMHMS) compared to UCCS over 16 weeks treatment with each.MethodA double-blind cross-over study of 10 adults (aged 16 – 38 years, six male) with GSD Ia and Ib. After an individualised fast, subjects were randomised to take a 50 g starch-load of either WMHMS or UCCS. Starch-loads terminated when blood glucose was < 3.0 mmol/L or the subject felt subjectively hypoglycaemic. Anonymous biochemical profiles were assessed by 2 investigators and a starch administration schedule recommended. Each starch was delivered in coded sachets and intake was monitored for the following 16 weeks. After a washout period, the protocol was repeated with the alternative product.Results4 subjects failed to establish therapy on the cross-over limb. Data from 7 paired starch load showed: longer median fasting duration with WMHMS (7.5 versus 5 hours; p = 0.023), slower decrease in the glucose curve (0.357 versus 0.632 mmol/hr p = 0.028) and less area under insulin curves for the first 4 hours (p = 0.03). Two of six subjects took 50% or less WMHMS compared to UCCS and one took more. Plasma triglycerides, cholesterol and uric acid were unchanged after each study phase.ConclusionWMHMS leads to significant reduction in insulin release and reduced starch use in some GSD patients.

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Disorders of Carbohydrate Metabolism

Cited by 2 publications

References 110 publications

Dietary dilemmas in the management of glycogen storage disease type I

Dietary dilemmas in the management of glycogen storage disease type I

A pilot longitudinal study of the use of waxy maize heat modified starch in the treatment of adults with glycogen storage disease type I: a randomized double-blind cross-over study

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