|
|
||||||||||||||
|
|
|||||||||||||||
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.
Related Article
This article has been cited by other articles:
![]() |
P. Venkataramani and P. A. Ganesan Index of Suspicion in the Nursery NeoReviews, August 1, 2007; 8(8): e354 - e356. [Full Text] [PDF] |
||||
![]() |
K. Hussain, O. Blankenstein, P. De Lonlay, and H. T Christesen Hyperinsulinaemic hypoglycaemia: biochemical basis and the importance of maintaining normoglycaemia during management Arch. Dis. Child., July 1, 2007; 92(7): 568 - 570. [Full Text] [PDF] |
||||
![]() |
I Giurgea, D Sanlaville, J-C Fournet, C Sempoux, C Bellanne-Chantelot, G Touati, L Hubert, M-S Groos, F Brunelle, J Rahier, et al. Congenital hyperinsulinism and mosaic abnormalities of the ploidy J. Med. Genet., March 1, 2006; 43(3): 248 - 254. [Abstract] [Full Text] [PDF] |
||||
![]() |
K. Hussain, J. Bryan, H. T. Christesen, K. Brusgaard, and L. Aguilar-Bryan Serum Glucagon Counterregulatory Hormonal Response to Hypoglycemia Is Blunted in Congenital Hyperinsulinism Diabetes, October 1, 2005; 54(10): 2946 - 2951. [Abstract] [Full Text] [PDF] |
||||
![]() |
B. Raju and P. E. Cryer Mechanism, temporal patterns, and magnitudes of the metabolic responses to the KATP channel agonist diazoxide Am J Physiol Endocrinol Metab, January 1, 2005; 288(1): E80 - E85. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. De Vroede, N.M.A. Bax, K. Brusgaard, M. J. Dunne, and F. Groenendaal Laparoscopic Diagnosis and Cure of Hyperinsulinism in Two Cases of Focal Adenomatous Hyperplasia in Infancy Pediatrics, October 1, 2004; 114(4): e520 - e522. [Abstract] [Full Text] [PDF] |
||||
![]() |
D. Muller, M. Zimmering, and C. C. Roehr Should nifedipine be used to counter low blood sugar levels in children with persistent hyperinsulinaemic hypoglycaemia? Arch. Dis. Child., January 1, 2004; 89(1): 83 - 85. [Full Text] [PDF] |
||||
![]() |
K Hussain and A Aynsley-Green Hyperinsulinaemic hypoglycaemia in preterm neonates Arch. Dis. Child. Fetal Neonatal Ed., January 1, 2004; 89(1): F65 - F67. [Abstract] [Full Text] [PDF] |
||||
![]() |
A. Kelly, C. Li, Z. Gao, C. A. Stanley, and F. M. Matschinsky Glutaminolysis and Insulin Secretion: From Bedside to Bench and Back Diabetes, December 1, 2002; 51(90003): S421 - 426. [Abstract] [Full Text] [PDF] |
||||
![]() |
H. Huopio, S.-L. Shyng, T. Otonkoski, and C. G. Nichols KATP channels and insulin secretion disorders Am J Physiol Endocrinol Metab, August 1, 2002; 283(2): E207 - E216. [Abstract] [Full Text] [PDF] |
||||
![]() |
N. MEHTA, J. STONE, and A. WHITELAW Practical management of hyperinsulinism in infancy Arch. Dis. Child. Fetal Neonatal Ed., May 1, 2001; 84(3): 218F - 218. [Full Text] |
||||
![]() |
C F J Munns and J A Batch Hyperinsulinism and Beckwith-Wiedemann syndrome Arch. Dis. Child. Fetal Neonatal Ed., January 1, 2001; 84(1): 67F - 69. [Full Text] |
||||
Read all eLetters
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS | REGISTER |
| ARCH DIS CHILD | FETAL NEONATAL ED | ED PRACTICE |