Contemporary strategies in the diagnosis and management of neonatal hyperinsulinaemic hypoglycaemia.
Congenital hyperinsulinism (CHI) is a genetically and phenotypically diverse syndrome. Key management issues involve early diagnosis by ensuring that appropriate samples are taken at the point of hypoglycaemia, prevention of recurrent hypoglycaemia, and detailed characterisation of the clinical, biochemical, and genetic features of each case. Infants with persistent diazoxide resistant CHI require evaluation at specialist referral centres equipped to differentiate those with focal (fo-HI) and diffuse (di-HI) pancreatic disease. Fo-HI is treated with selective pancreatic resection but di-HI is treated by surgery only if intensive medical management regimes are not efficacious.
Lindley
Keith J
KJ
London Centre for Pancreatic Disease in Childhood, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK. K.Lindley@ich.ucl.ac.uk
Dunne
Mark J
MJ
D001774:Blood Chemical Analysis; D044903:Congenital Hyperinsulinism / Q000175:diagnosis* / Q000378:metabolism* / Q000601:surgery / Q000628:therapy*; D005947:Glucose / Q000378:metabolism; D006801:Humans; D007231:Infant, Newborn; D007328:Insulin / Q000378:metabolism; D000078790:Insulin Secretion; D010179:Pancreas / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D015221:Potassium Channels / Q000378:metabolism*
D007328:Insulin; D015221:Potassium Channels; D005947:Glucose