Nesidioblastosis (2) In its most extreme form, gross interuterine hyperinsulinism is associated with obesity at birth and intractable symptomatic hypoglycaemia immediately thereafter. This can cause severe and permanent brain damage or death unless effective and continuous treatment is instituted at once. In the moderately severe case, however, the baby has a normal appearance at birth, and nothing untoward happens until the child is in its second or third month of life, when failure of mental development or the onset of fits brings it to medical attention. Some of the cases are familial, especially those in which leucine sensitivity is a feature. See also: • ‘Inappropriate’ Insulin Secretion • Neuroglycopenia • Diagnosis of Endogenous Hyperinsulinism • Treatment of Endogenous Hyperinsulinism