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| Ionic control of beta cell function in nesidioblastosis. A possible therapeutic role for calcium channel blockade |
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| Author(s): Lindley KJ, Dunne MJ, Kane C, Shepherd RM, Squires PE, James RFL, Johnson PRV, Eckhardt S, Wakeling E, Dattani M, Milla PJ, AynsleyGreen A |
| Source: ARCHIVES OF DISEASE IN CHILDHOOD Volume: 74 Issue: 5 Pages: 373-378 Published: MAY 1996 |
| Times Cited: 56 References: 29 |
| Abstract: A preterm female infant presented with intractable hypoglycaemia within 10 minutes of delivery. Normoglycaemia could be maintained only by the intravenous infusion of glucose at a rate of 20-22 mg/kg/min. Persistent hyperinsulinaemic hypoglycaemia of infancy was diagnosed from an inappropriately raised plasma insulin concentration (33 mU/l) at the time of hypoglycaemia (blood glucose <0.5 mmol/l). Medical treatment with glucagon, somatostatin, and diazoxide led to only a modest reduction in the intravenous glucose requirement; a 95% pancreatectomy was performed and histological 'nesidioblastosis' confirmed. In vitro electrophysiological studies using patch clamp techniques on isolated pancreatic beta cells characterised the ionic basis for insulin secretion in nesidioblastosis. The beta cells were depolarised in low ambient glucose concentrations with persistently firing action potentials; these were blocked reversibly by the calcium channel blocking agent verapamil. Persistent postoperative hyperinsulinaemic hypoglycaemia was treated with oral nifedipine. This increased median blood glucose concentrations from 3.5 to 4.8 mmol/l and increased in duration the child's tolerance to fasting from 3 to 10.5 hours. These data allude to an abnormality in the ionic control of insulin release in nesidioblastosis and offer a new logical approach to treatment which requires further evaluation. |
| Document Type: Article |
| Language: English |
| Reprint Address: Lindley, KJ (reprint author), INST CHILD HLTH, 30 GUILFORD ST, LONDON WC1N 1EH, ENGLAND |
Addresses:
1. GREAT ORMOND ST HOSP CHILDREN, NHS TRUST, LONDON, ENGLAND
2. UNIV SHEFFIELD, DEPT BIOMED SCI, SHEFFIELD S10 2TN, S YORKSHIRE ENGLAND
3. UNIV LEICESTER, DEPT SURG, LEICESTER LE1 7RH, LEICS ENGLAND
4. GEN HOSP, ST HELIER, JERSEY ENGLAND |
| Publisher: BRITISH MED JOURNAL PUBL GROUP, BRITISH MED ASSOC HOUSE, TAVISTOCK SQUARE, LONDON, ENGLAND WC1H 9JR |
| Subject Category: Pediatrics |
| IDS Number: UL736 |
| ISSN: 0003-9888 |
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