Effect of continuous glucose therapy begun in infancy on the long-term Clinical course of patients with type I glycogen storage disease.
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
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0424820
Code Inist : 002B02N. Création : 22/03/2000.