title: Complex carbohydrates in the dietary management of patients with glycogenosis caused by glucose-6-phosphatase deficiency.
abstract: Carbohydrates with digestion characteristics between those of lente uncooked starches and rapidly digestible oligosaccharides were administered in a dose of 1.5 g/kg body weight to five patients with glycogenosis from glucose-6-phosphatase deficiency. Postprandial duration of normoglycemia and concentrations of blood insulin and lactate were determined. Uncooked barley groats in water, or incorporated in a meal turned out to behave as lente carbohydrates. Uncooked couscous in water, couscous incorporated in a meal, and partially cooked macaroni given as a meal behaved as semilente carbohydrates as compared with uncooked cornstarch and glucose. The in vitro determination of the digestibility index along with the in vivo tolerance test enables us to choose and incorporate semilente carbohydrates in the day-time treatment of patients.
authors.affiliation: Department of Pediatrics, University of Groningen, The Netherlands.
authors.forename: G P
authors.initials: GP
authors.lastname: Smit
authors.forename: M T
authors.initials: MT
authors.lastname: Ververs
authors.forename: B
authors.initials: B
authors.lastname: Belderok
authors.forename: M
authors.initials: M
authors.lastname: Van Rijn
authors.forename: R
authors.initials: R
authors.lastname: Berger
authors.forename: J
authors.initials: J
authors.lastname: Fernandes
mesh_terms: D000293:Adolescent; D001786:Blood Glucose / Q000032:analysis; D002648:Child; D004040:Dietary Carbohydrates / Q000145:classification / Q000627:therapeutic use*; D006008:Glycogen Storage Disease / Q000097:blood / Q000178:diet therapy* / Q000209:etiology; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006801:Humans; D007328:Insulin / Q000097:blood; D007773:Lactates / Q000097:blood; D019344:Lactic Acid
chemical_list: D001786:Blood Glucose; D004040:Dietary Carbohydrates; D007328:Insulin; D007773:Lactates; D019344:Lactic Acid