[Persistent hyperinsulinemic hypoglycemia in infancy: differentiated approach to a heterogeneous syndrome]

Abstract

Persistent hyperinsulinemic hypoglycaemia in infancy (PHHI) presents a diagnostic and therapeutic challenge for the treating physician: increased glucose requirements, detectable insulin levels at the point of hypoglycaemia, inappropriately low blood levels of free fatty acids and ketone bodies are characteristic of this condition. Despite recent developments in understanding its pathophysiology, treatment remains difficult and there are many long-term complications. Adequate treatment strategies are needed to prevent severe neurological damage. As there is a high possibility that hyperinsulinism may only be transient, aggressive pharmacological treatment is necessary for 4 to 6 weeks before moving on to surgical intervention. In the light of recent knowledge, routine subtotal pancreatectomy in children is no longer justifiable. An attempt should first be made to differentiate between focal and diffuse hypersecretion of insulin by using interventional radiology techniques, notably pancreatic venous sampling. This then enables targeted partial pancreatectomy.