アカントージスニグリカンスを伴ったインスリン抵抗性糖尿病:内分泌学的病態解析

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We described a 12 7/12-year-old boy presenting the syndrome of insulin resistance and acanthosis nigricans Type A..<BR>Insulin levels at fasting state and after glucose loading were 149 ± 63/μU/ml (mean ± SD) and over 1000W/ml, respectively, while fasting levels of blood glucose were 77.7 ± 8.9mg/dl (mean ± SD). A marked resistance to exogenous insulin was observed. Circulating levels of insulin antagonists such as growth hormone, cortisol and glucagon, were within the normal range. Proinsulin was less than 5% of the total radioimmunoassayable insulin. Insulin antibody and anti-receptor antibody were not detected. Insulin binding to mononuclear cells was decreased to about 50% of controls. Analysis of membrane receptors demonstrated the normal average affinity, dissociation kinetics and negative cooperativity with a decreased number of receptors. After two days fasting, plasma IRI levels decreased to 27/μU/ml, however insulin binding kinetics were not affected. This might suggesting that the receptor decrease was not secondary to hyperinsulinemia.<BR>These findings indicate that a decreased number of receptors was one of the causes for insulin resistance in the patient.