The DOI matches its Crossref record; 23 of the 23 references it cites were found in a registry or an index.
Noninsulinoma pancreatogenous hypoglycemia syndrome: An update in 10 surgically treated patients
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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“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).”
From Evaluation and Management of Adult Hypoglycemic Disorders: An Endocrine Society Clinical Practice Guideline (2009) · cites this paper as [44]
Citations
Cites
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Metadata
- 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 › Endocrinology, Diabetes and Metabolism › Pancreatic function and diabetes
- Health Sciences › Medicine › Oncology › 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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