Document Detail


Clinical features of 52 neonates with hyperinsulinism.
MedLine Citation:
PMID:  10202168     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Neonatal hyperinsulinemic hypoglycemia is often resistant to medical therapy and is often treated with near-total pancreatectomy. However, the pancreatic lesions may be focal and treatable by partial pancreatic resection.
METHODS: We studied 52 neonates with hyperinsulinism who were treated surgically. The type and location of the pancreatic lesions were determined by preoperative pancreatic catheterization and intraoperative histologic studies. Partial pancreatectomy was performed in infants with focal lesions, and near-total pancreatectomy was performed in those with diffuse lesions. The postoperative outcome was determined by measurements of plasma glucose and glycosylated hemoglobin and by oral glucose-tolerance tests.
RESULTS: Thirty neonates had diffuse beta-cell hyperfunction, and 22 had focal adenomatous islet-cell hyperplasia. Among the latter, the lesions were in the head of the pancreas in nine, the isthmus in three, the body in eight, and the tail in two. The clinical manifestations were similar in both groups. The infants with focal lesions had no symptoms of hypoglycemia and had normal preprandial and postprandial plasma glucose and glycosylated hemoglobin values and normal results on oral glucose-tolerance tests after partial pancreatectomy (performed in 19 of 22 neonates). By contrast, after near-total pancreatectomy, 13 of the patients with diffuse lesions had persistent hypoglycemia, type 1 diabetes mellitus developed in 8, and hyperglycemia developed in another 7; overall, only 2 patients with diffuse lesions had normal plasma glucose concentrations in the first year after surgery.
CONCLUSIONS: Among neonates with hyperinsulinism, about half may have focal islet-cell hyperplasia that can be treated with partial pancreatectomy. These neonates can be identified through pancreatic catheterization and intraoperative histologic studies.
Authors:
P de Lonlay-Debeney; F Poggi-Travert; J C Fournet; C Sempoux; C Dionisi Vici; F Brunelle; G Touati; J Rahier; C Junien; C Nihoul-Fékété; J J Robert; J M Saudubray
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The New England journal of medicine     Volume:  340     ISSN:  0028-4793     ISO Abbreviation:  N. Engl. J. Med.     Publication Date:  1999 Apr 
Date Detail:
Created Date:  1999-04-15     Completed Date:  1999-04-15     Revised Date:  2014-07-08    
Medline Journal Info:
Nlm Unique ID:  0255562     Medline TA:  N Engl J Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1169-75     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
ATP-Binding Cassette Transporters*
Blood Glucose / analysis
Glucose Tolerance Test
Hemoglobin A, Glycosylated / analysis
Humans
Hyperinsulinism / complications,  congenital*,  pathology,  surgery
Hyperplasia / complications,  genetics,  surgery
Hypoglycemia / etiology
Infant, Newborn
Insulin / blood
Islets of Langerhans / pathology*,  physiopathology
Mutation
Pancreatectomy* / methods
Potassium Channels / genetics
Potassium Channels, Inwardly Rectifying*
Receptors, Drug / genetics
Sulfonylurea Receptors
Chemical
Reg. No./Substance:
0/Blood Glucose; 0/Hemoglobin A, Glycosylated; 0/Insulin; 0/Potassium Channels; 0/Potassium Channels, Inwardly Rectifying; 0/Receptors, Drug; 0/Sulfonylurea Receptors
Comments/Corrections
Comment In:
N Engl J Med. 1999 Apr 15;340(15):1200-1   [PMID:  10202173 ]
N Engl J Med. 1999 Aug 26;341(9):701-2   [PMID:  10475832 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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