Insulin effect on serum potassium and auto‐inhibition of insulin secretion is intact in a patient with leprechaunism despite severe impairment of substrates metabolism

The effect of insulin on glucose, protein metabolism, circulating fatty acids (FFA), potassium (K+) and C‐peptide concentrations were investigated in a 12‐year‐old girl with leprechaunism. The mutations do not affect the insulin‐receptor binding affinity and insulin‐stimulated auto‐phosphorylation of the receptor.

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