Modern therapeutic aspects in two cases of congenital hyperinsulinism

Abstract

In this article we will present the therapeutic and clinical aspects found in the case of two pediatric patients diagnosed with congenital hyperinsulinism. Thus, we want to show the modern therapeutic principles used in the treatment of these patients. It is important to remember that congenital hyperinsulinism is the only curable metabolic congenital disease, as currently surgical therapy is only indicated in isolated cases. This is why quick diagnosis is essential, because the cases of hypoglycemia caused by this illness are severe. Furthermore, congenital hyperinsulinism blocks ketogenesis, so neither the brain nor the myocardium can use ketone bodies as an alternative energy substrate in case of hypoglycemia, and the risk of brain damage or cardiac arrest is very high in this disease.

Keywords

• hypoketotic hypoglycemia
• curable disease
• diazoxide