Neonatal hyperinsulinemic hypoglycemia: heterogeneity of the syndrome and keys for differential diagnosis.

Détails

ID Serval
serval:BIB_F2430029808C
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Neonatal hyperinsulinemic hypoglycemia: heterogeneity of the syndrome and keys for differential diagnosis.
Périodique
Journal of Clinical Endocrinology and Metabolism
Auteur(s)
Sempoux C., Guiot Y., Lefevre A., Nihoul-Fékété C., Jaubert F., Saudubray J.M., Rahier J.
ISSN
0021-972X (Print)
ISSN-L
0021-972X
Statut éditorial
Publié
Date de publication
1998
Peer-reviewed
Oui
Volume
83
Numéro
5
Pages
1455-1461
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov'tPublication Status: ppublish
Résumé
The two major forms of infantile persistent hyperinsulinemic hypoglycemia require different treatments, but are difficult to differentiate during surgery. Indeed, one is characterized by focal adenomatous hyperplasia often macroscopically invisible, whereas the other consists of a diffuse, but discreet, beta-cell abnormality. We evaluated, in a large series of persistent hyperinsulinemic hypoglycemia, the reliability of two criteria in differentiating these two forms: the mean beta-cell nuclear radius (MNR) and the beta-cell nuclear crowding, i.e. the number of nuclei per 1000 micron 2 beta-cell (BCNC). The values of the largest MNR and of BCNC in cases bearing a focal lesion (respectively, 3.27 microns +/- 0.25 and 14.62 +/- 1.78) were significantly different from those in the diffuse pathology (4.25 microns +/- 0.43 and 10.00 +/- 1.55). Setting the threshold value of MNR at 3.70 microns and that of BCNC at 12.00 enabled correct classification of 90.9% of the diffuse and 100% of the focal forms. beta-Cell nuclear analysis can thus contribute to a subclassification of the syndrome, not allowed by clinical or biological data. If performed during surgery it could help in determining the extent of pancreatectomy necessary to cure the patient, as the diffuse form, with abnormal nuclei in the whole pancreas, requires subtotal to near-total pancreatectomy, whereas the focal form, devoid of abnormal insular beta-cell nuclei, can be cured by partial pancreatectomy.
Mots-clé
Cell Nucleus/pathology, Diagnosis, Differential, Female, Humans, Hyperinsulinism/complications, Hyperplasia, Hypoglycemia/diagnosis, Hypoglycemia/etiology, Infant, Newborn, Islets of Langerhans/abnormalities, Islets of Langerhans/pathology, Male, Pancreatectomy, Pancreatic Diseases/complications, Pancreatic Diseases/diagnosis, Retrospective Studies, Syndrome
Pubmed
Web of science
Open Access
Oui
Création de la notice
20/10/2016 17:38
Dernière modification de la notice
09/05/2019 3:21
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