A case of leprechaunism with severe hyperinsulinemia
A. M. Rosenberg, J. C. Haworth, G. W. Degroot, C. L. Trevenen and M. M. Rechler
This report describes an infant with physical features typical of
leprechaunism, including a characteristic facies, hirsutism, and decreased
subcutaneous tissue and muscle mass. Intermittent hypoglycemia and severe
hyperinsulinemia were documented. The patient's insulin was normal in
molecular size and biological activity, but its binding to the patient's
cultured fibroblasts was profoundly decreased. Insulin antibodies were not
present. A literature review has been undertaken to clarify further the
clinical, metabolic, and pathological characteristics of this condition.