Christ, Emanuel; Iacovazzo, Donato; Korbonits, Marta; Perren, Aurel (2023). Insulinomatosis - new aspects. Endocrine-related cancer, 30(6) BioScientifica 10.1530/ERC-22-0327
Full text not available from this repository. (Request a copy)Endogenous hyperinsulinemic hypoglycemia (EHH) is a rare condition with an incidence of approximately 4-6 per million person-years and comprises a group of disorders causing hyperinsulinemic hypoglycemia without exogenous administration of insulin or its secretagogues. In adults, most cases (approximately 90%) are secondary to a single insulinoma. Other causes include insulinoma in the context of multiple endocrine neoplasia type 1 (approximately 5% of cases) and non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS), which is estimated to account for 0.5-5% of all cases. Recently, an entity called insulinomatosis has been described as a novel cause of EHH in adults. The characteristic feature of insulinomatosis is the synchronous or metachronous occurrence of multiple pancreatic neuroendocrine micro-tumors expressing exclusively insulin. While most cases arise sporadically, there is recent evidence that autosomal dominant inheritance of mutations in the v-maf avian musculoaponeurotic fibrosarcoma oncogene homolog A (MAFA) gene can cause a familial form of insulinomatosis. In these families EHH is paradoxically associated with the occurrence of diabetes mellitus within the same family. This review summarizes the current clinical, biochemical, imaging and genetic knowledge of this disease.
Item Type: |
Journal Article (Review Article) |
|---|---|
Division/Institute: |
04 Faculty of Medicine > Service Sector > Institute of Pathology > Clinical Pathology 04 Faculty of Medicine > Service Sector > Institute of Pathology |
UniBE Contributor: |
Perren, Aurel |
Subjects: |
500 Science > 570 Life sciences; biology 600 Technology > 610 Medicine & health |
ISSN: |
1479-6821 |
Publisher: |
BioScientifica |
Language: |
English |
Submitter: |
Pubmed Import |
Date Deposited: |
24 Mar 2023 14:46 |
Last Modified: |
18 May 2023 00:14 |
Publisher DOI: |
10.1530/ERC-22-0327 |
PubMed ID: |
36952647 |
URI: |
https://boris.unibe.ch/id/eprint/180606 |
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