Tags

Type your tag names separated by a space and hit enter

The focal form of persistent hyperinsulinemic hypoglycemia of infancy: morphological and molecular studies show structural and functional differences with insulinoma.
Diabetes. 2003 Mar; 52(3):784-94.D

Abstract

Paternal mutation of ATP-sensitive K(+) (K(ATP)) channel genes and loss of heterozygosity (LOH) of the 11p15 region including the maternal alleles of ABCC8, IGF2, and CDKN1C characterize the focal form of persistent hyperinsulinemic hypoglycemia of infancy (FoPHHI). We aimed to understand the actual nature of FoPHHI in comparison with insulinoma. In FoPHHI, the lesion consists in clusters of beta-cells surrounded by non-beta-cells. Compared with adjacent islets, proinsulin mRNA is similar and proinsulin production higher (P < or = 0.02), indicating regulation at a translational level, with slightly lower insulin stock and lower ABCC8 peptide labeling (P<0.05). Insulinomas, composed of beta-cell nests or cords, have similar proinsulin mRNA compared with adjacent islets, highly variable proinsulin production, lower insulin stock (P < or = 0.02), and higher ABCC8 peptide labeling (P<0.05). Proinsulin mRNA is lower than in FoPHHI (P<0.001). Islets adjacent to FoPHHI appear to be resting, in contrast to those adjacent to insulinomas, evidencing intrapancreatic regulation of islet beta-cell activity. IGF2 peptide is present inside and outside both lesions, but IGF2 mRNA is restricted to the lesions. The 11p15 LOH and absence of CDKN1C peptide staining are demonstrated in all FoPHHI but also in three of eight insulinomas. Despite some molecular similarities, FoPHHI is thus fundamentally different from insulinoma.

Authors+Show Affiliations

Department of Pathology (ANPS 1712), Cliniques Universitaires St-Luc, University Hospital, U.C.L. Avenue Hippocrate 10, B-1200 Brussels, Belgium. christine.sempoux@anps.ucl.ac.beNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

12606521

Citation

Sempoux, Christine, et al. "The Focal Form of Persistent Hyperinsulinemic Hypoglycemia of Infancy: Morphological and Molecular Studies Show Structural and Functional Differences With Insulinoma." Diabetes, vol. 52, no. 3, 2003, pp. 784-94.
Sempoux C, Guiot Y, Dahan K, et al. The focal form of persistent hyperinsulinemic hypoglycemia of infancy: morphological and molecular studies show structural and functional differences with insulinoma. Diabetes. 2003;52(3):784-94.
Sempoux, C., Guiot, Y., Dahan, K., Moulin, P., Stevens, M., Lambot, V., de Lonlay, P., Fournet, J. C., Junien, C., Jaubert, F., Nihoul-Fekete, C., Saudubray, J. M., & Rahier, J. (2003). The focal form of persistent hyperinsulinemic hypoglycemia of infancy: morphological and molecular studies show structural and functional differences with insulinoma. Diabetes, 52(3), 784-94.
Sempoux C, et al. The Focal Form of Persistent Hyperinsulinemic Hypoglycemia of Infancy: Morphological and Molecular Studies Show Structural and Functional Differences With Insulinoma. Diabetes. 2003;52(3):784-94. PubMed PMID: 12606521.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The focal form of persistent hyperinsulinemic hypoglycemia of infancy: morphological and molecular studies show structural and functional differences with insulinoma. AU - Sempoux,Christine, AU - Guiot,Yves, AU - Dahan,Karin, AU - Moulin,Pierre, AU - Stevens,Martine, AU - Lambot,Virginie, AU - de Lonlay,Pascale, AU - Fournet,Jean-Christophe, AU - Junien,Claudine, AU - Jaubert,Francis, AU - Nihoul-Fekete,Claire, AU - Saudubray,Jean-Marie, AU - Rahier,Jacques, PY - 2003/2/28/pubmed PY - 2003/5/13/medline PY - 2003/2/28/entrez SP - 784 EP - 94 JF - Diabetes JO - Diabetes VL - 52 IS - 3 N2 - Paternal mutation of ATP-sensitive K(+) (K(ATP)) channel genes and loss of heterozygosity (LOH) of the 11p15 region including the maternal alleles of ABCC8, IGF2, and CDKN1C characterize the focal form of persistent hyperinsulinemic hypoglycemia of infancy (FoPHHI). We aimed to understand the actual nature of FoPHHI in comparison with insulinoma. In FoPHHI, the lesion consists in clusters of beta-cells surrounded by non-beta-cells. Compared with adjacent islets, proinsulin mRNA is similar and proinsulin production higher (P < or = 0.02), indicating regulation at a translational level, with slightly lower insulin stock and lower ABCC8 peptide labeling (P<0.05). Insulinomas, composed of beta-cell nests or cords, have similar proinsulin mRNA compared with adjacent islets, highly variable proinsulin production, lower insulin stock (P < or = 0.02), and higher ABCC8 peptide labeling (P<0.05). Proinsulin mRNA is lower than in FoPHHI (P<0.001). Islets adjacent to FoPHHI appear to be resting, in contrast to those adjacent to insulinomas, evidencing intrapancreatic regulation of islet beta-cell activity. IGF2 peptide is present inside and outside both lesions, but IGF2 mRNA is restricted to the lesions. The 11p15 LOH and absence of CDKN1C peptide staining are demonstrated in all FoPHHI but also in three of eight insulinomas. Despite some molecular similarities, FoPHHI is thus fundamentally different from insulinoma. SN - 0012-1797 UR - https://www.unboundmedicine.com/medline/citation/12606521/The_focal_form_of_persistent_hyperinsulinemic_hypoglycemia_of_infancy:_morphological_and_molecular_studies_show_structural_and_functional_differences_with_insulinoma_ L2 - https://diabetes.diabetesjournals.org/lookup/pmidlookup?view=long&amp;pmid=12606521 DB - PRIME DP - Unbound Medicine ER -