Focal and diffuse forms of congenital hyperinsulinism: the keys for differential diagnosis.

Details

Serval ID
serval:BIB_4103679BAE0D
Type
Article: article from journal or magazin.
Collection
Publications
Title
Focal and diffuse forms of congenital hyperinsulinism: the keys for differential diagnosis.
Journal
Endocrine Pathology
Author(s)
Sempoux C., Guiot Y., Jaubert F., Rahier J.
ISSN
1046-3976 (Print)
ISSN-L
1046-3976
Publication state
Published
Issued date
2004
Peer-reviewed
Oui
Volume
15
Number
3
Pages
241-246
Language
english
Notes
Publication types: Journal Article ; Review Publication Status: ppublish
Abstract
Congenital hyperinsulinism is clinically characterized by an inappropriate insulin secretion resulting in recurrent severe hypoglycemia. Nesidioblastosis, the proliferation of islets cells budding off from pancreatic ducts, has been considered for years as the histological lesion responsible for the syndrome. In our morphological studies, we demonstrated that nesidioblastosis is not specific for the disease, which is actually not a single entity. Indeed, we recognized the existence of two different forms--a diffuse form and a focal form--and demonstrated that they can be differentiated on the basis of morphological criteria, even on frozen sections during surgery. This histological distinction directs the therapeutic approach because the patients suffering from the focal form of the syndrome can be completely cured by a very limited pancreatectomy. Molecular findings confirmed the reliability of this histological distinction, showing a specific genetic background for each form.
Keywords
Cell Nucleus/pathology, Congenital Hyperinsulinism/classification, Congenital Hyperinsulinism/genetics, Diagnosis, Differential, Humans, Islets of Langerhans/pathology, Nesidioblastosis/genetics, Nesidioblastosis/pathology, Pancreatectomy, Treatment Outcome
Pubmed
Web of science
Create date
20/10/2016 16:21
Last modification date
20/08/2019 13:40
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