Fukushima N, Nohara Y, Fujita H, Endo M, Abe K, Matsuura N, Kosaka S, Satomi S
Nihon Naibunpi Gakkai Zasshi. 1982 Jun 20;58(6):807-16. doi: 10.1507/endocrine1927.58.6_807.
We described a 12 7/12-year-old boy presenting the syndrome of insulin resistance and acanthosis nigricans Type A. Insulin levels at fasting state and after glucose loading were 149 +/- 63 microunits/ml (mean +/- SD) and over 1000 microunits/ml, respectively, while fasting levels of blood glucose were 77.7 +/- 8.9 mg/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 were 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 microunits/ml, however insulin binding kinetics were not affected. This might suggesting that the receptor decrease was not secondary to hyperinsulinemia. These findings indicate that a decreased number of receptors was one of the causes for insulin resistance in the patient.
我们描述了一名12又7/12岁的男孩,其患有A型胰岛素抵抗综合征和黑棘皮症。空腹状态及葡萄糖负荷后胰岛素水平分别为149±63微单位/毫升(均值±标准差)和超过1000微单位/毫升,而空腹血糖水平为77.7±8.9毫克/分升(均值±标准差)。观察到对外源性胰岛素有明显抵抗。胰岛素拮抗剂如生长激素、皮质醇和胰高血糖素的循环水平在正常范围内。胰岛素原占总放射免疫可测定胰岛素的比例小于5%。未检测到胰岛素抗体和抗受体抗体。胰岛素与单核细胞的结合降至对照组的约50%。膜受体分析显示平均亲和力、解离动力学正常且具有负协同性,但受体数量减少。禁食两天后,血浆IRI水平降至27微单位/毫升,然而胰岛素结合动力学未受影响。这可能表明受体减少并非继发于高胰岛素血症。这些发现表明受体数量减少是该患者胰岛素抵抗的原因之一。