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Fluctuation of Insulin Resistance in a Leprechaun With a Primary Defect in Insulin Binding^*

Third Department of Medicine, Shiga University of Medical Science Ohtsu, Shiga
The Department of Endocrinology and Metabolism, Kobe Children' s Hospital (K.G.) Kobe, Japan

Address requests for reprints to: Masashi Kobayashi, M.D., Third Department of Medicine, Shiga University of Medicine, Ohtsu, Shiga 520-21, Japan.

A 3-month-old female leprechaun demonstrated extreme insulin resistance with hyperinsulinemia (330 µmol/L) and resistance to exogenous insulin. Insulin binding to erythrocytes, cultured lymphocytes, and fibroblasts from the patient were decreased to less than 20% of normal, whereas insulin-like growth factor I binding to fibroblasts was normal. Antiinsulin receptor antibody binding to cultured lymphocytes was also decreased to 20% of normal, indicating a decreased concentration of insulin receptors on the cell surface. The ability of insulin to stimulate D-[¹⁴C]glucose uptake was decreased to 35% of normal in the patient' s fibroblasts, and the dose-response curve was shifted to the right. With time, the insulin resistance fluctuated from near normal (fasting insulin, 244.0 pmol/L) to severe resistance (fasting insulin, 5740–9328 pmol/L), and an insulin tolerance test revealed amelioration of insulin resistance during remission. However, insulin binding to erythrocytes and adipocytes was decreased persistently to 20% of normal. These results indicate that the patient had a primary defect in her insulin receptors, i.e. decreased insulin receptor concentration. The variable degree of insulin resistance was possibly due to variable receptor function in the signal transmission process.

^* This work was supported in part by a research grant-in-aid from the Ministry of Education, Science, and Culture and the Research Grant for the Intractable Disease from the Ministry of Health and Welfare, Japan.

Received August 24, 1987.

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