Document Detail

Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism.
MedLine Citation:
PMID:  10931418     Owner:  NLM     Status:  MEDLINE    
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.
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
Related Documents :
193448 - Immunological disorders and malignancies in five young brothers.
9171068 - The risk of birth defects among children of persian gulf war veterans.
11096168 - Folic acid antagonists during pregnancy and the risk of birth defects.
18655098 - The embryology of body wall closure: relevance to gastroschisis and other ventral body ...
23332848 - Short communication: radio frequency dielectric heating of nonfat dry milk affects solu...
16837118 - Meconium-stained amniotic fluid--perinatal outcome and obstetrical management in a low-...
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:  2011-05-05    
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    
Division of Endocrinology & Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4318, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
ATP-Binding Cassette Transporters*
Calcium / blood,  diagnostic use*
Case-Control Studies
Child, Preschool
Diagnosis, Differential
Diagnostic Techniques, Endocrine
Hyperinsulinism / blood,  congenital*,  diagnosis*
Injections, Intravenous
Potassium Channels* / genetics
Potassium Channels, Inwardly Rectifying*
Receptors, Drug* / genetics
Sulfonylurea Compounds / metabolism*
Grant Support
Reg. No./Substance:
0/ATP-Binding Cassette Transporters; 0/Potassium Channels; 0/Potassium Channels, Inwardly Rectifying; 0/Receptors, Drug; 0/Sulfonylurea Compounds; 0/sulfonylurea receptor; 7440-70-2/Calcium

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

Previous Document:  Alterations in sleep physiology in young children with insulin-dependent diabetes mellitus: relation...
Next Document:  Kawasaki disease in older children and adolescents.