Classical hyperinsulinism is a relatively uncommon disease of the islets of Langerhans, characterized by hypersecretion of insulin and, sometimes, proinsulin. Neuropsychiatric symptoms and obesity predominate. All 13 of our patients had benign or malignant tumors or hyperplasia, confirmed by operation or biopsy. One of nine adults and one of four infants died. The latter, if they fail to respond to glucose, require emergency surgery.
(JAMA 230:1538-1543, 1974)