title: Glucose metabolism in 105 children and adolescents after pancreatectomy for congenital hyperinsulinism.
abstract: OBJECTIVE
  To describe the long-term metabolic outcome of children with congenital hyperinsulinism after near-total or partial elective pancreatectomy.
  
  
  METHODS
  Patients (n = 105: 58 diffuse and 47 focal congenital hyperinsulinism) received operations between 1984 and 2006. Follow-up consisted of periodic measurements of pre- and postprandial plasma glucose over 24 h, OGTT, and IVGTT. Cumulative incidence of hypo- or hyperglycemia/insulin treatment was estimated by Kaplan-Meier analysis.
  
  
  RESULTS
  After near-total pancreatectomy, 59% of children with diffuse congenital hyperinsulinism still presented mild or asymptomatic hypoglycemia that responded to medical treatments and disappeared within 5 years. One-third of the patients had both preprandial hypoglycemia and postprandial hyperglycemia. Hyperglycemia was found in 53% of the patients immediately after surgery; its incidence increased regularly to 100% at 13 years. The cumulative incidence of insulin-treated patients was 42% at 8 years and reached 91% at 14 years, but the progression to insulin dependence was very variable among the patients. Plasma insulin responses to IVGTT and OGTT correlated well with glycemic alterations. In focal congenital hyperinsulinism, hypoglycemia or hyperglycemia were rare, mild, and transient.
  
  
  CONCLUSIONS
  Patients with focal congenital hyperinsulinism are cured of hypoglycemia after limited surgery, while the outcome of diffuse congenital hyperinsulinism is very variable after near-total pancreatectomy. The incidence of insulin-dependent diabetes is very high in early adolescence.
authors.affiliation: Pediatric Endocrinology and Diabetes, Hôpital Necker-Enfants Malades, Université Paris Descartes Sorbonne Paris cité, Paris, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Beltrand
authors.forename: Marylène
authors.initials: M
authors.lastname: Caquard
authors.forename: Jean-Baptiste
authors.initials: JB
authors.lastname: Arnoux
authors.forename: Kathleen
authors.initials: K
authors.lastname: Laborde
authors.forename: Gilberto
authors.initials: G
authors.lastname: Velho
authors.forename: Virginie
authors.initials: V
authors.lastname: Verkarre
authors.forename: Jacques
authors.initials: J
authors.lastname: Rahier
authors.forename: Francis
authors.initials: F
authors.lastname: Brunelle
authors.forename: Claire
authors.initials: C
authors.lastname: Nihoul-Fékété
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Saudubray
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Robert
authors.forename: Pascale
authors.initials: P
authors.lastname: de Lonlay
mesh_terms: D000293:Adolescent; D001786:Blood Glucose / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D044903:Congenital Hyperinsulinism / Q000097:blood* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007328:Insulin / Q000097:blood; D053208:Kaplan-Meier Estimate; D008297:Male; D010180:Pancreatectomy; D016896:Treatment Outcome
chemical_list: D001786:Blood Glucose; D007328:Insulin