LONG-TERM TREATMENT OF PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY WITH DIAZOXIDE - A RETROSPECTIVE REVIEW OF 77 CASES AND ANALYSISOF EFFICACY-PREDICTING CRITERIA

Primary persistent hyperinsulinaemic hypoglycaemia of infancy is rare. Diazoxide treatment remains the mainstay of medical therapy in long-t erm management. We reviewed 77 cases of primary persistent hyperinsuli nism in neonates and infants who were treated with diazoxide and studi ed criteria predictive of therapeutic efficacy. The only criterion ide ntified was age at manifestation. All but 1 of the 31 neonatal cases w ere unresponsive to diazoxide. Responsiveness increased with age: 12 o f 39 early-infantile cases, and all seven late-infantile cases were di azoxide-responsive. In responders, a diazoxide dose of 10-15 mg/kg per day was always effective, suggesting an ''all or none'' response. Dia zoxide-resistant hyperinsulinism is characterized by its severity with higher plasma insulin levels. The analysis of 46 surgically treated p atients showed that the efficacy of diazoxide is not related to the ae tiology of the pancreatic lesions. In six cases, after many years of m anagement, diazoxide treatment was stopped without recurrence of hypog lycaemia.