Persistent hyperinsulinemic hypoglycemia of infancy in 38 children

To describe the clinical, biochemical, radiological and electrophysiological features of 38 Saudi children with persistent hyperinsulinemic hypoglycemia of infancy that have been followed since 1983. Data from 38 patients followed at King Faisal Specialist Hospital and Research Centre, Riyadh, Kingdom of Saudi Arabia from 1983 through to 2002 was retrospectively analyzed. Persistent hyperinsulinemic hypoglycemia of infancy was diagnosed on the basis of high intravenous glucose requirement, high insulin to glucose ratio, negative urinary ketones and normal tandem mass spectrometry. The patients were assessed radiologically by brain magnetic resonance imaging, computed tomography, or both and electrophysiologically by brain stem auditory evoked potential, visual evoked response and electroencephalogram. The patients who failed medical therapy had subtotal pancreatectomy. The patients were severely hypoglycemic and intolerant to fast. Hypoglycemic convulsion was the most commonly presenting complaint. Eighteen patients were developmentally delayed and 14 of them had brain atrophy. All patients, except nine, did not respond to medical treatment and had surgery. Four pancreatectomized patients developed diabetes and 2 had malabsorption. One patient was treated medically during childhood and developed diabetes and weight gain during adolescence. Persistent hyperinsulinemic hypoglycemia of infancy is a relatively common and serious disease among Saudi children. Early medical intervention is necessary to avoid neurological damage in our patients who are severely hypoglycemic and medical therapy unresponsive. Surgically and probably medically treated patients are at high risk of developing diabetes that could be the natural outcome of this disease.