PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY - EXPERIENCE WITH28 CASES

Twenty-eight infants with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) were seen during a 10-year period. There were 13 males and 15 females. Their age at time of presentation ranged from a few ho urs to 6 months. Consanguinity was reported in 20 cases (71.4%). One f amily had two affected siblings and two affected cousins, another had three affected siblings and one affected cousin, and three others had lost siblings because of hypoglycemia and seizures. The primary clinic al presentation was jitters and seizures in association with hypoglyce mia. The diagnosis was suspected when the therapeutic glucose requirem ent was found to be more than 12 mg/kg/min and also when there was a g ood response to glucagon after exclusion of metabolic and storage dise ases. A high insulin-to glucose ratio was noted for all patients. Twen ty-two had near-total (90%) pancreatectomy; the result was excellent i n all but four, who required supplemental medical therapy. Five patien ts were treated medically, and one patient's family refused treatment. Twelve patients sustained moderate to severe brain injury before refe rral. There were no deaths, and only one patient had evidence of malab sorption after the pancreatectomy. PHHI correlates well with consangui nity and family history, Clinical awareness is essential to permit ear ly diagnosis and prompt medical and supportive therapy. Early surgery is recommended in the majority of cases if permanent brain damage is t o be avoided. Near-total pancreatectomy provides the best surgical out come, with little morbidity and no mortality. Copyright (C) 1995 by W. B. Saunders Company