Noninsulinoma pancreatogenous hypoglycemia

A novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes

F. John Service, Neena Natt, Geoffrey B. Thompson, Clive S. Grant, Jonathan A. Van Heerden, James C. Andrews, Eva Lorenz, Andre Terzic, Ricardo V. Lloyd

Research output: Contribution to journalArticle

170 Citations (Scopus)

Abstract

In adults, endogenous hyperinsulinemic hypoglycemia is almost invariably due to insulinoma. In these patients with insulinoma, neuroglycopenic episodes exclusively after meal ingestion and negative 72-h fasts are extraordinarily rare. We describe five adults with neuroglycopenic episodes from hyperinsulinemic hypoglycemia within 4 h of meal ingestion and negative 72-h fasts. Each had negative transabdominal ultrasonography, spiral computed tomographic scanning, and celiac axis angiography of the pancreas. However, all showed positive selective arterial calcium stimulation tests indicative of pancreatic β-cell hyperfunction. At pancreatic exploration, no insulinoma was detected by intraoperative ultrasonography and complete mobilization and palpation of the pancreas. Moreover, the resected pancreata showed islet hypertrophy and nesidioblastosis, but no insulinoma. No definite disease-causing mutation was detected in Kir6.2 and SUR1 genes, which encode the subunits of the pancreatic ATP-sensitive potassium channel responsible for glucose-induced insulin secretion. Four patients who underwent gradient-guided partial pancreatectomy have been free of hypoglycemic symptoms for up to 3 yr follow-up; the other, who underwent a limited distal pancreatectomy, has had brief recurrence of symptoms. The unique clinical features and responses to dynamic testing in these adults with hyperinsulinemic hypoglycemia in the absence of insulinoma may constitute a new syndrome of postprandial hypoglycemia from diffuse β-cell hyperfunction.

Original languageEnglish (US)
Pages (from-to)1582-1589
Number of pages8
JournalJournal of Clinical Endocrinology and Metabolism
Volume84
Issue number5
StatePublished - 1999

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Ultrasonography
Insulinoma
Hypoglycemia
Genes
KATP Channels
Mutation
Angiography
Pancreas
Hypoglycemic Agents
Pancreatectomy
Meals
Insulin
Calcium
Scanning
Glucose
Nesidioblastosis
Eating
Testing
Palpation
Abdomen

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Service, F. J., Natt, N., Thompson, G. B., Grant, C. S., Van Heerden, J. A., Andrews, J. C., ... Lloyd, R. V. (1999). Noninsulinoma pancreatogenous hypoglycemia: A novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes. Journal of Clinical Endocrinology and Metabolism, 84(5), 1582-1589.

Noninsulinoma pancreatogenous hypoglycemia : A novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes. / Service, F. John; Natt, Neena; Thompson, Geoffrey B.; Grant, Clive S.; Van Heerden, Jonathan A.; Andrews, James C.; Lorenz, Eva; Terzic, Andre; Lloyd, Ricardo V.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 84, No. 5, 1999, p. 1582-1589.

Research output: Contribution to journalArticle

Service, F. John ; Natt, Neena ; Thompson, Geoffrey B. ; Grant, Clive S. ; Van Heerden, Jonathan A. ; Andrews, James C. ; Lorenz, Eva ; Terzic, Andre ; Lloyd, Ricardo V. / Noninsulinoma pancreatogenous hypoglycemia : A novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes. In: Journal of Clinical Endocrinology and Metabolism. 1999 ; Vol. 84, No. 5. pp. 1582-1589.
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