Disorders of Carbohydrate Metabolism

Dixon, Marjorie

doi:10.1002/9780470690369.ch16

Cited by 3 publications

(3 citation statements)

References 60 publications

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“…Provision of exogenous glucose to GSD I patients has altered over the years [1,6,7,11,12,15,26,36,37,45,46]. Methods are frequent feedings, meals and snacks preferably with precooked cornstarch (PCCS), continuous nocturnal gastric drip feeding (CNGDF) and administration of uncooked cornstarch (UCSS).…”

Section: Recommendations For Dietary Treatmentmentioning

confidence: 99%

Guidelines for management of glycogen storage disease type I - European Study on Glycogen Storage Disease Type I (ESGSD I)

Rake

Visser

Labrune³

et al. 2002

European Journal of Pediatrics

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Section: Recommendations For Dietary Treatmentmentioning

confidence: 99%

Guidelines for management of glycogen storage disease type I - European Study on Glycogen Storage Disease Type I (ESGSD I)

Rake

Visser

Labrune³

et al. 2002

European Journal of Pediatrics

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“…The main aim in the treatment of hepatic GSD is the maintenance of normoglycaemia [4]. The management varies according to the age of the patient and the specific type of disorder.…”

Section: Introductionmentioning

confidence: 99%

Dental disease indices and caries‐related microflora in children with glycogen storage disease

Kidd

Rademeyer

Roberts

et al. 2002

Int J Paed Dentistry

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Objective. To establish the levels of dental caries, bacterial dental plaque, gingivitis and caries-related microflora in children with glycogen storage disease (GSD). Patients with GSD are treated with regular intakes of glucose polymer and uncooked cornstarch to prevent hypoglycaemia. Dental health data are scarce. Study Group. The study group comprised 21 children with GSD attending the Great Ormond Street Hospital for Children. Outcome Measures. These included the number of decayed, missing and filled teeth, and surfaces in both the primary and permanent dentitions, plaque and gingivitis scores. Both plaque and saliva were collected from each child and cultured for Mutans streptococci, Lactobacilli and Candida.Results. The study group included 13 boys and eight girls, aged from 2·7 to 15·5 years. Four of the 21 children had some caries experience. The mean dmft was 0·5 and the mean DMFT, 0·06. Mean plaque and gingivitis scores were 4·8 and 5·9, respectively, for plaque and gingivitis adjacent to the primary teeth, and 11·6 and 12 for those related to permanent teeth. Conclusions. Only a small proportion of the children had caries experience but most were found to have plaque associated with both primary and permanent teeth. Preventive care should be targeted to improve plaque control thus minimizing the risk of developing periodontal disease as adults.

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“…Galactosaemia is treated by a minimal galactose diet, which is essentially one free from milk and milk products, supplemented with a lactose-free milk substitute (Dixon, 2001). There is no consensus as to whether dietary galactosides need to be excluded in early years of life.…”

Section: Introductionmentioning

confidence: 99%

Dietary calcium in galactosaemia

Rutherford

Davidson

Matthai

2002

J Human Nutrition Diet

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The diets of 19 galactosaemic patients were assessed for calcium and phosphorous intake. Despite the use of infant soya formula or calcium-supplemented soya milk the reference nutrient intake (RNI) for calcium was only met in 26% of the group; all patients met > 100% of the RNI for phosphorous. The regular assessment of the diets of galactosaemia patients is recommended as the milk-free nature of the diet can lead to an inadequate calcium intake. Lactose-free calcium supplements should be prescribed if the diet alone is inadequate.

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Disorders of Carbohydrate Metabolism

Cited by 3 publications

References 60 publications

Guidelines for management of glycogen storage disease type I - European Study on Glycogen Storage Disease Type I (ESGSD I)

Guidelines for management of glycogen storage disease type I - European Study on Glycogen Storage Disease Type I (ESGSD I)

Dental disease indices and caries‐related microflora in children with glycogen storage disease

Dietary calcium in galactosaemia

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