Arch Dis Child Fetal Neonatal Ed 2000;82:F98-F107 ( March )

Article

Practical management of hyperinsulinism in infancy A Aynsley-Green^a, K Hussain^a, J Hall^b, J M Saudubray^c, C Nihoul-Fékété^c, P De Lonlay-Debeney^c, F Brunelle^c, T Otonkoski^d, P Thornton^e, K J Lindley^a

^a The London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH and the Institute of Child Health, University College, London WC1N 1EH, UK, ^b The Institute of Molecular Physiology and Department of Biomedical Science, University of Sheffield, Sheffield S10 2TN, UK, ^c The Metabolic Unit of Pediatrics and Departments of Radiology and Surgery, Hospital Necker Enfants Malades, 75015 Paris, France, ^d Hospital for Children and Adolescents and the Hartman Institute Transplantation Laboratory, 000140 University of Helsinki, Finland, ^e The National Centre for Inherited Metabolic Disorders, The Children's Hospital, Temple Street, Dublin 1, Ireland, ^f On behalf of the European Network for Research into Hyperinsulinism (ENRHI)

Correspondence to: Professor A Aynsley-Green, The Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK

Hyperinsulinism in infancy is one of the most difficult problems to manage in contemporary paediatric endocrinology. Although the diagnosis can usually be achieved without difficulty, it presents the paediatrician with formidable day to day management problems. Despite recent advances in understanding the pathophysiology of hyperinsulinism, the neurological outcome remains 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 state of the art overview on management derived from a consensus workshop held by the European network for research into hyperinsulinism (ENRHI). The consensus is presented as an educational aid for paediatricians and children's nurses. It offers a practical guide to management based on the most up to 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 principles of definitive treatment.


Keywords: hyperinsulinism; treatment; hypoglycaemia; neurological damage; cation channels

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© 2000 by Archives of Disease in Childhood
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