Cited by 121Pancreatic function and diabetesNeuroendocrine Tumor Research AdvancesPancreatitis Pathology and Treatment

Noninsulinoma pancreatogenous hypoglycemia syndrome: An update in 10 surgically treated patients

Thompson, Geoffrey B., Service, F.John, Andrews, James C., Lloyd, Ricardo V., Natt, Neena, van Heerden, Jon A., Grant, Clive S.·2000·Surgery

Abstract

Background 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.

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Registry

Crossref · 10.1067/msy.2000.110243

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23 of 23 references verified0 citation errors · 0 not checkable

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  • NIPHS is characterized by spells of neuroglycopenia due to endogenous hyperinsulinemic hypoglycemia typically, but not invariably, after a meal.

    “The noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is characterized by spells of neuroglycopenia due to endogenous hyperinsulinemic hypoglycemia typically, but not invariably, after a meal (42)(43)(44)(45).”

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Authors
Thompson, G. B., Service, F. J., Andrews, J. C., Lloyd, R. V., Natt, N., van Heerden, J. A., & Grant, C. S.
Affiliations
Mayo Clinic (US)
Venue
Surgery
Publisher
Elsevier BV
Published
2000-12
Volume
128
Issue
6
Pages
937-945
ISSN
0039-6060 (ISSN-L)
OpenAlex
W2087465249
Cited by
121
References
23
Citations by year
2026: 3 · 2025: 3 · 2024: 5 · 2023: 3 · 2022: 5 · 2021: 5 · 2020: 2 · 2019: 2 · 2018: 3 · 2017: 3 · 2016: 2 · 2015: 6 · 2014: 4 · 2013: 3 · 2012: 8
FWCI
1.80
Type
journal article
Language
en
Open access
closed
Locations
  • Surgery (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Surgery › Pancreatitis Pathology and Treatment
Keywords
hyperinsulinemic hypoglycemia, nesidioblastosis, calcium stimulation test, distal pancreatectomy, surgical outcomes, sex differences in outcomes
MeSH terms
Adolescent, Adult, Aged, Calcium, Female, Humans, Hypoglycemia, Male, Middle Aged, Pancreatectomy, Postoperative Complications, Syndrome

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