Clinical Features and Causes of Endogenous Hyperinsulinemic Hypoglycemia in Korea
Open accessAbstract
BACKGROUND: Endogenous hyperinsulinemic hypoglycemia (EHH) is characterized by an inappropriately high plasma insulin level, despite a low plasma glucose level. Most of the EHH cases are caused by insulinoma, whereas nesidioblastosis and insulin autoimmune syndrome (IAS) are relatively rare. METHODS: To evaluate the relative frequencies of various causes of EHH in Korea, we retrospectively analyzed 84 patients who were diagnosed with EHH from 1998 to 2012 in a university hospital. RESULTS: Among the 84 EHH patients, 74 patients (88%), five (6%), and five (6%) were diagnosed with insulinoma, nesidioblastosis or IAS, respectively. The most common clinical manifestation of EHH was neuroglycopenic symptoms. Symptom duration before diagnosis was 14.5 months (range, 1 to 120 months) for insulinoma, 1.0 months (range, 6 days to 7 months) for nesidioblastosis, and 2.0 months (range, 1 to 12 months) for IAS. One patient, who was diagnosed with nesidioblastosis in 2006, underwent distal pancreatectomy but was later determined to be positive for insulin autoantibodies. Except for one patient who was diagnosed in 2007, the remaining three patients with nesidioblastosis demonstrated severe hyperinsulinemia (157 to 2,719 µIU/mL), which suggests that these patients might have had IAS, rather than nesidioblastosis. CONCLUSION: The results of this study suggest that the prevalence of IAS may be higher in Korea than previously thought. Therefore, measurement of insulin autoantibody levels is warranted for EHH patients, especially in patients with very high plasma insulin levels.
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It is estimated that about 1 in 10,000,000 people are diagnosed with idiopathic/sporadic adult-onset nesidioblastosis annually.
“It is estimated that about 1 in 10,000,000 people are diagnosed with this disease annually [45-48].”
From An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS) (2023) · cites this paper as [48]
The Discussion (page not available) says adult nesidioblastosis is very rare, accounting for approximately 0.5% of endogenous hyperinsulinemic hypoglycemia cases. It does not give an annual population diagnosis rate of 1 in 10,000,000 or an estimate specific to idiopathic or sporadic adult-onset disease. (LLM, conf=0.84)
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ResultHirata's disease (insulin autoimmune syndrome) following envenomation by a common krait.
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“In a seemingly well individual, the dif- ferential diagnosis of hypoglycemia narrows to 2 major categories: 1) accidental, surreptitious, or intentional hypoglycemia, or 2) endogenous hyperinsulinism (EHH).”
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- Authors
- Woo, C.-Y., Jeong, J. Y., Jang, J. E., Leem, J., Jung, C. H., Koh, E. H., Lee, W. J., Kim, M.-S., Park, J.-Y., Lee, J. B., & Lee, K.-U.
- Affiliations
- University of Ulsan (KR)
- Corresponding author
- Lee, Ki-Up
- ORCID
- Venue
- Diabetes & Metabolism Journal
- Publisher
- Korean Diabetes Association
- Published
- 2015
- Volume
- 39
- Issue
- 2
- Pages
- 126
- ISSN
- 2233-6079 (ISSN-L)
- 2233-6087
- PMC
- PMC4411543
- OpenAlex
- W2016544936
- Cited by
- 32
- References
- 27
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 3 · 2023: 4 · 2022: 4 · 2021: 3 · 2020: 7 · 2019: 2 · 2018: 2 · 2016: 3
- FWCI
- 1.68
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Diabetes & Metabolism Journal (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Topics
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Diabetes Management and Research
- Keywords
- endogenous hyperinsulinemic hypoglycemia, insulinoma, nesidioblastosis, insulin autoimmune syndrome, insulin autoantibodies, hyperinsulinemia, neuroglycopenic symptoms, distal pancreatectomy
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