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Glucose Metabolism in 105 Children and Adolescents After Pancreatectomy for Congenital Hyperinsulinism.
MedLine Citation:
PMID:  22190679     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
OBJECTIVETo describe the long-term metabolic outcome of children with congenital hyperinsulinism after near-total or partial elective pancreatectomy.RESEARCH DESIGN AND METHODSPatients (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.RESULTSAfter 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.CONCLUSIONSPatients 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:
Jacques Beltrand; Marylène Caquard; Jean-Baptiste Arnoux; Kathleen Laborde; Gilberto Velho; Virginie Verkarre; Jacques Rahier; Francis Brunelle; Claire Nihoul-Fékété; Jean-Marie Saudubray; Jean-Jacques Robert; Pascale de Lonlay
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-12-21
Journal Detail:
Title:  Diabetes care     Volume:  -     ISSN:  1935-5548     ISO Abbreviation:  -     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-12-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7805975     Medline TA:  Diabetes Care     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Pediatric Endocrinology and Diabetes, Hôpital Necker-Enfants Malades, Université Paris Descartes Sorbonne Paris cité, Paris, France.
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