Practical management of hyperinsulinism in infancy

Hyyperinsulinism in infancy is one of the most difficult problems to manage in contemporary paediatric endocrinology. Although the diagnosis can usual ly be achieved without difficulty, it presents the paediatrician with formi dable day to day management problems. Despite recent advances in understand ing the pathophysiology of hyperinsulinism, the neurological outcome remain s poor, and there is often a choice of unsatisfactory treatments, with life long sequelae for the child and his or her family. This paper presents a s tate of the art overview on management derived from a consensus workshop he ld by the European network for research into hyperinsulinism (ENRHI). The c onsensus is presented as an educational aid for paediatricians and children 's nurses. It offers a practical guide to management based on the most up t o date knowledge. It presents a proposed management cascade and focuses on the clinical recognition of the disease, the immediate steps that should be taken to stabilise the infant during diagnostic investigations, and the pr inciples of definitive treatment.