Abstract | OBJECTIVES: STUDY DESIGN: 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.
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Authors | R J Ferry Jr, A Kelly, A Grimberg, S Koo-McCoy, M J Shapiro, K E Fellows, B Glaser, L Aguilar-Bryan, D E Stafford, C A Stanley |
Journal | The Journal of pediatrics
(J Pediatr)
Vol. 137
Issue 2
Pg. 239-46
(Aug 2000)
ISSN: 0022-3476 [Print] United States |
PMID | 10931418
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- ABCC8 protein, human
- ATP-Binding Cassette Transporters
- Potassium Channels
- Potassium Channels, Inwardly Rectifying
- Receptors, Drug
- Sulfonylurea Compounds
- Sulfonylurea Receptors
- Calcium
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Topics |
- ATP-Binding Cassette Transporters
- Adolescent
- Adult
- Calcium
(blood)
- 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
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