Individuals affected with two genetic syndromes identified in Ecuador have severe short stature and diminished insulin secretion, along with essentially different GH counterregulatory effects on insulin action, which leads to the appearance of opposing metabolic phenotypes. In the case of Laron syndrome, subjects have enhanced insulin sensitivity and diminished incidence of type 2 diabetes mellitus. In the other clinical entity, individuals have innate insulin resistance, a varying degree of carbohydrate metabolism disturbances, glucose intolerance, and eventually insulin-resistant diabetes mellitus. Since both groups have diminished insulin secretion, the standard homeostatic minimal models for assessment of insulin sensitivity and resistance were used to see if they could properly identify the metabolic status, especially considering that these methodologies are simple and non-invasive procedures.

Methods

Fasting insulin concentrations, fasting glucose/fasting insulin ratio and various minimal models were determined in individuals from the two syndromic cohorts, as well as in a control group made of first-degree normal relatives of the insulin-resistant phenotype subjects.

Results

The metabolic characteristics of enhanced insulin sensitivity in one of the syndromes and innate insulin resistance in the other could not be properly ascertained by the selected methodology. Furthermore, results were confusing and even discrepant with the clinical findings.

Conclusions

The standard homeostatic minimal models could not properly identify or discriminate insulin sensitivity and resistance in subjects with inherently diminished secretion. It is thereby suggested that these models should be used with caution in clinical situations where reduced secretion of the metabolic peptide is found or suspected.

中文翻译：

---

评估严重身材矮小综合征的胰岛素敏感性和抵抗力。

在厄瓜多尔发现的受两种遗传综合征影响的个体具有严重的身材矮小和胰岛素分泌减少，以及对胰岛素作用的 GH 反调节作用截然不同，这导致出现相反的代谢表型。在 Laron 综合征的情况下，受试者的胰岛素敏感性增强，2 型糖尿病的发病率降低。在另一个临床实体中，个体具有先天性胰岛素抵抗、不同程度的碳水化合物代谢紊乱、葡萄糖耐受不良和最终的胰岛素抵抗性糖尿病。由于两组都减少了胰岛素分泌，因此使用用于评估胰岛素敏感性和抵抗力的标准稳态最小模型来查看它们是否可以正确识别代谢状态，

方法

空腹胰岛素浓度、空腹血糖/空腹胰岛素比值和各种最小模型在来自两个综合征队列的个体以及由胰岛素抵抗表型受试者的一级正常亲属组成的对照组中确定。

结果

所选方法无法正确确定一种综合征中胰岛素敏感性增强和另一种先天性胰岛素抵抗的代谢特征。此外，结果令人困惑，甚至与临床发现不一致。

结论

标准稳态最小模型无法正确识别或区分固有分泌减少的受试者的胰岛素敏感性和抵抗力。因此建议在发现或怀疑代谢肽分泌减少的临床情况下应谨慎使用这些模型。