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  1. Effect of continuous glucose therapy begun in infancy on the long-term Clinical course of patients with type I glycogen storage disease.

    Article - En anglais


    To evaluate the effects of continuous glucose therapy on metabolic control, occurrence of severe hypoglycemia, physical growth and development, and complications of glycogen storage disease type I (GSD-I).


    Seventeen patients (11 males) with GSD-I were studied, mean age 14.6 ± 5.0 (SD) years, in whom continuous glucose therapy was begun at 0.8 ± 0.4 years.

    At the time of this study, subjects had received continuous glucose therapy for a total duration of 13.9 ± 5.0 years.

    Uncooked cornstarch was used as the method of providing glucose continuously for 10.2 ± 3.2 years.

    Subjects were admitted to the Clinical Research Center and followed their usual home dietary regimens, which included cornstarch supplements at 2-to 4-hour intervals during the day and at 4-to 8-hour intervals during the night.

    Plasma glucose, blood lactate, and glucoregulatory hormones were measured hourly for 24 hours.


    During a 24-hour period of biochemical monitoring, mean hourly plasma glucose concentrations for the group of 17 subjects ranged from 76<17 (SD) mg/dl (4.2 ± 0.9 mmol/l) to 108 ± 16 mg/dl (6.0<0.9 mmol/l), and blood lactate concentrations ranged from 2.1 ± 1.2 mmol/l to 3.8<2.8 mmol/l. Four subjects had transient plasma glucose levels of 50 mg/dl (2.8 mmol/l) or less in the interval between midnight and 8 : 00 AM.

    Mean blood lactate levels were highest (>=3 mmol/l) between 2 : 00 and 09 : 00 AM. (...)

    Mots-clés Pascal : Glycogène, Stockage, Evaluation, Glucose, Recommandation alimentaire, Long terme, Trouble métabolisme, Croissance, Incidence, Hypoglycémie, Développement sexuel, Complication, Tardif, Aliment cru, Amidon maïs, Adolescent, Homme, Nourrisson, Biologie moléculaire, Glucide, Métabolisme pathologie, Prévention

    Mots-clés Pascal anglais : Glycogen, Storage, Evaluation, Glucose, Dietary allowance, Long term, Metabolic disorder, Growth, Incidence, Hypoglycemia, Sexual development, Complication, Late, Uncooked food, Corn starch, Adolescent, Human, Infant, Molecular biology, Carbohydrate, Metabolic diseases, Prevention

    Logo du centre Notice produite par :
    Inist-CNRS - Institut de l'Information Scientifique et Technique

    Cote : 99-0424820

    Code Inist : 002B02N. Création : 22/03/2000.