Document Detail


Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism.
MedLine Citation:
PMID:  10931418     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: To identify infants with hyperinsulinism caused by defects of the beta-cell adenosine triphosphate-dependent potassium channel complex and to distinguish focal and diffuse forms of hyperinsulinism caused by these mutations.
STUDY DESIGN: The acute insulin response to intravenous calcium stimulation (CaAIR) was determined in 9 patients <20 years with diffuse hyperinsulinism caused by defective beta-cell sulfonylurea receptor (SUR1(-/-)), 3 patients with focal congenital hyperinsulinism (6 weeks to 18 months), a 10-year-old with insulinoma, 5 with hyperinsulinism/hyperammonemia syndrome caused by defective glutamate dehydrogenase (6 months to 28 years), 4 SUR1(+/-) heterozygotes with no symptoms, and 9 normal adults. Three infants with congenital focal disease, 1 with diffuse hyperinsulinism, and the child with insulinoma underwent selective pancreatic intra-arterial calcium stimulation with hepatic venous sampling.
RESULTS: Children with diffuse SUR1(-/-) disease and infants with congenital focal hyperinsulinism responded to CaAIR, whereas the normal control group, patients with hyperinsulinism/hyperammonemia syndrome, and SUR1(+/-) carriers did not. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling revealed selective, significant step-ups in insulin secretion that correlated anatomically with the location of solitary lesions confirmed surgically in 2 of 3 infants with congenital focal disease and in the child with insulinoma. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling demonstrated markedly elevated baseline insulin levels throughout the pancreas of the infant with diffuse hyperinsulinism.
CONCLUSIONS: The intravenous CaAIR is a safe and simple test for identifying infants with diffuse SUR1(-/-) hyperinsulinism or with focal congenital hyperinsulinism. Preoperative selective arterial calcium stimulation of the pancreas with hepatic venous sampling can localize focal lesions causing hyperinsulinism in children. The combination of these calcium stimulation tests may help distinguish focal lesions suitable for cure by local surgical resection.
Authors:
R J Ferry; A Kelly; A Grimberg; S Koo-McCoy; M J Shapiro; K E Fellows; B Glaser; L Aguilar-Bryan; D E Stafford; C A Stanley
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  137     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  2000 Aug 
Date Detail:
Created Date:  2000-09-19     Completed Date:  2000-09-19     Revised Date:  2014-08-04    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  239-46     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
ATP-Binding Cassette Transporters*
Adolescent
Adult
Calcium / blood,  diagnostic use*
Case-Control Studies
Child
Child, Preschool
Diagnosis, Differential
Diagnostic Techniques, Endocrine
Female
Humans
Hyperinsulinism / blood,  congenital*,  diagnosis*
Infant
Injections, Intravenous
Male
Potassium Channels* / genetics
Potassium Channels, Inwardly Rectifying*
Receptors, Drug* / genetics
Sulfonylurea Compounds / metabolism*
Sulfonylurea Receptors
Grant Support
ID/Acronym/Agency:
F32 DK009985-01/DK/NIDDK NIH HHS; R01 DK053012/DK/NIDDK NIH HHS; R01 DK056268/DK/NIDDK NIH HHS; R01 DK53012/DK/NIDDK NIH HHS; R01 DK56268/DK/NIDDK NIH HHS; T32 DK07314/DK/NIDDK NIH HHS
Chemical
Reg. No./Substance:
0/ABCC8 protein, human; 0/Potassium Channels; 0/Potassium Channels, Inwardly Rectifying; 0/Receptors, Drug; 0/Sulfonylurea Compounds; 0/Sulfonylurea Receptors; SY7Q814VUP/Calcium

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


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