ABSTRACT
Hyperinsulinism is characterized by inappropriately high insulin levels for the concentration of blood glucose. Most infants with hyperinsulinism present within the first few days after birth. The hyperinsulinism may be associated with a few well-defined clinical conditions such as Beckwith-Wiedemann syndrome (exomphalos, macroglossia, gigantism = EMG), the infant of a diabetic mother or the ‘stressed neonate’ following birth asphyxia and Rh incompatibility. The hypoglycemia associated with these conditions is transient and will not be discussed further. It is also important to exclude leucin sensitivity and other endocrine disorders such as cortisol deficiency, or inborn errors of metabolism, e.g. glycogen storage disease.
