Some aspects of carbohydrate metabolism in Prader-Willi syndrome.
Three patients with Prader-Willi syndrome are reported. The subjects presented chemical diabetes mellitus. All patients were hypersentsiive to exogenous insulin and they showed poor adrenal medullary response to hypoglycaemics. It was postulated that the poor adrenal medullary response to the insulin injection may be a contributing factor in the excessive hypoglycaemic response, and that the diabetes mellitus observed in the patients could be due to inactivation of endogenous insulin.
D000293:Adolescent; D000313:Adrenal Medulla / Q000503:physiopathology; D000328:Adult; D001786:Blood Glucose / Q000032:analysis*; D003920:Diabetes Mellitus / Q000378:metabolism*; D004837:Epinephrine / Q000378:metabolism; D005260:Female; D005951:Glucose Tolerance Test; D006801:Humans; D007003:Hypoglycemia / Q000139:chemically induced; D007328:Insulin / Q000378:metabolism*; D008607:Intellectual Disability / Q000378:metabolism*; D008297:Male; D009638:Norepinephrine / Q000378:metabolism; D009765:Obesity / Q000378:metabolism*; D011236:Prediabetic State / Q000378:metabolism / Q000503:physiopathology; D013577:Syndrome; D014044:Tolbutamide
D001786:Blood Glucose; D007328:Insulin; D014044:Tolbutamide; D009638:Norepinephrine; D004837:Epinephrine