Nesidioblastosis associated with hyperinsulinemic hypoglycemia in adults: review of the literature
Abstract
Nesidioblastosis is a term that was first introduced by Laidlaw in 1938 to define the diffuse proliferation of pancreatic islet cells budding from ductal epithelium. First described in neonates, it is widely recognized to be the primary cause of persistent hyperinsulinemic hypoglycemia in infants. In adults, insulinoma accounts for most cases of hyperinsulinemic hypoglycemia. Adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia is a very rare entity, representing 0.5-5% of cases of organic hyperinsulinemia. Since 1981, when Harness et al. reported the first series of adult nesidioblastosis causing hypoglycemia, only 71 cases have been published. This article provides a guide for the diagnosis of organic hyperinsulinism and indicates when nesidioblastosis should be suspected. New diagnostic methods and treatment options for this rare disease are proposed. We also present a review of all reported cases of adult nesidioblastosis in the last 22 years.
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This symptom finally led to the correct diagnosis of a functional β-cell disorder, also known as diffuse adult-onset nesidioblastosis or NIPHS.
“This symptom finally led to the correct diagnosis of a functional β -cell disorder, also known as diffuse adult-onset nesidioblastosis or non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) [4-6].”
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“[2,8] Moreover, radiological localization studies, such as CT scan, endoscopic ultrasound, octreotide scan, and selective arterial calcium stimulation test (SACST) with hepatic venous sampling, can be performed to distinguish between a focal abnormality (insulinoma) and a diffuse process (nesidioblastosis).”
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- Authors
- Jabri, A. L., & Bayard, C.
- Affiliations
- Hôpital de Sion (CH)
- Corresponding author
- Jabri, A.L.
- Bayard, C.
- Venue
- European Journal of Internal Medicine
- Publisher
- Elsevier BV
- Published
- 2004-11
- Volume
- 15
- Issue
- 7
- Pages
- 407-410
- ISSN
- 0953-6205 (ISSN-L)
- OpenAlex
- W2060328028
- Cited by
- 56
- References
- 2
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 2 · 2023: 7 · 2022: 2 · 2021: 2 · 2020: 3 · 2019: 2 · 2018: 1 · 2017: 1 · 2016: 4 · 2015: 4 · 2014: 1 · 2013: 2 · 2012: 2
- FWCI
- 2.27
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- European Journal of Internal Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
- Keywords
- nesidioblastosis, hyperinsulinemic hypoglycemia, organic hyperinsulinism, insulinoma, diagnosis, treatment, rare diseases
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