Enhanced Islet Cell Nucleomegaly Defines Diffuse Congenital Hyperinsulinism in Infancy but Not Other Forms of the Disease. Issue 6 (22nd June 2016)

• Record Type: Journal Article
• Authors: Han, Bing; Newbould, Melanie; Batra, Gauri; Cheesman, Edmund; Craigie, Ross J.; Mohamed, Zainab; Rigby, Lindsey; Padidela, Raja; Skae, Mars; Mironov, Aleksandr; Starborg, Tobias; Kadler, Karl E.; Cosgrove, Karen E.; Banerjee, Indraneel; Dunne, Mark J.
• Is Part Of: American journal of clinical pathology. Volume 145:Issue 6(2016)
• Page Start: 757
• Page End: 768
• Publication Date: 2016-06-22
• Subjects: Islet -- Congenital hyperinsulinism -- Insulin -- Hypoglycemia -- Nucleomegaly -- Serial block-face scanning electron microscopy -- Pancreas -- β cell -- Hyperplasia; Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqw075
• Languages: English
• ISSNs: 0002-9173

Abstract

Abstract : Objectives: To quantify islet cell nucleomegaly in controls and tissues obtained from patients with congenital hyperinsulinism in infancy (CHI) and to examine the association of nucleomegaly with proliferation. Methods: High-content analysis of histologic sections and serial block-face scanning electron microscopy were used to quantify nucleomegaly. Results: Enlarged islet cell nuclear areas were 4.3-fold larger than unaffected nuclei, and the mean nuclear volume increased to approximately threefold. Nucleomegaly was a normal feature of pediatric islets and detected in the normal regions of the pancreas from patients with focal CHI. The incidence of nucleomegaly was highest in diffuse CHI (CHI-D), with more than 45% of islets containing two or more affected cells. While in CHI-D nucleomegaly was negatively correlated with cell proliferation, in all other cases, there was a positive correlation. Conclusions: Increased incidence of nucleomegaly is pathognomonic for CHI-D, but these cells are nonproliferative, suggesting a novel role in the pathobiology of this condition.