Endogenous glucose production in type I glycogen storage disease
Autor: | Ira K. Brandt, Richard C. Powell, Samuel M. Wentworth |
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Rok vydání: | 1981 |
Předmět: |
Adult
Blood Glucose Male medicine.medical_specialty Hydrocortisone Fasting Hypoglycemia Endocrinology Diabetes and Metabolism Phosphatase Endogeny Fructose Glycogen Storage Disease Type I Biology Glucagon chemistry.chemical_compound Endocrinology Internal medicine medicine Humans Lactic Acid Starvation Ethanol Fasting Lactic acid Kinetics chemistry Lactates Female medicine.symptom medicine.drug |
Zdroj: | Metabolism. 30:443-450 |
ISSN: | 0026-0495 |
DOI: | 10.1016/0026-0495(81)90178-5 |
Popis: | The adaptive mechanisms that protect some patients with Type I glycogen storage disease from fasting hypoglycemia were examined in two young adults. Both maintained low normal fasting plasma glucose concentrations even during 3 day fasts; blood lactate concentrations increased during the first 12 hr and then decreased to normal during the second and third days. Acute hyperglycemic responses to glucagon nearly doubled after three days of starvation when compared with responses following 12 hr fasts. Enhanced glucagon-induced hyperglycemic changes also were observed following the administration of alcohol or glucocorticoids. However, fructose infusions failed to demonstrate hyperglycemic responses after a 3 day fast, alcohol or glucocorticoids. The present studies demonstrate endogenous glucose production in our patients despite an absence of the enzyme glucose-6-phosphatase. These findings could explain why some patients with Type I glycogen storage disease are protected from fasting hypoglycemia. |
Databáze: | OpenAIRE |
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