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Noninsulinoma pancreatogenous hypoglycemia syndrome: an update in 10 surgically treated patients.

AbstractBACKGROUND:
Neuroglycopenia from endogenous hyperinsulinism usually is caused by insulinomas in adults. We recently reported a novel hypoglycemic disorder in 5 patients (patients 1 to 5) with postprandial neuroglycopenia, negative 72-hour fasts, negative perioperative imaging studies, but positive calcium stimulation tests and islet hypertrophy and nesidioblastosis in the gradient-guided resected pancreata.
METHODS:
In this report we compare our experience with 5 additional patients (patients 6 to 10) with this syndrome to that in the original report.
RESULTS:
The clinical features of patients 6 to 10 were similar to those of patients 1 to 5. Each had positive calcium stimulation testing that guided the extent of the distal pancreatectomy and histologic evidence of islet cell hypertrophy or nesidioblastosis. All 10 patients are alive from 9 to 50 months after operation, 1 of whom had no amelioration of neuroglycopenia. Minor perioperative complications occurred in 3 patients. One patient has experienced repeated bouts of acute pancreatitis, pseudocyst formation, and exocrine insufficiency.
CONCLUSIONS:
We have identified adult patients with severe, postprandial hyperinsulinemic hypoglycemia from diffuse islet cell disease, 80% of whom have been well palliated with surgery. The results in 7 men have been better than those in the 3 women for reasons that are not obvious.
AuthorsG B Thompson, F J Service, J C Andrews, R V Lloyd, N Natt, J A van Heerden, C S Grant
JournalSurgery (Surgery) Vol. 128 Issue 6 Pg. 937-44;discussion 944-5 (Dec 2000) ISSN: 0039-6060 [Print] United States
PMID11114627 (Publication Type: Journal Article)
Chemical References
  • Calcium
Topics
  • Adolescent
  • Adult
  • Aged
  • Calcium (pharmacology)
  • Female
  • Humans
  • Hypoglycemia (pathology, surgery)
  • Male
  • Middle Aged
  • Pancreatectomy
  • Postoperative Complications
  • Syndrome

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