Abstract

Marking by peroxidase-conjugated anti-insulin antibodies, and electron microscopy reveal in these pancreases, besides normal islets of Langerhans in lobular exocrine tissue -a/numerous insulin cells inside acini, singly or in small groups, with dense crystalline insulin granules. Some acinar “mixed cells” contain both zymogen and insulin granules, a possible intermediate transformation stage. -b/large “extra-lobu-lar islets”, in contact with pancreatic ducts whose most epithelial cells contain insulin granules and give way to budding of insulin cells, showing growth of extra lobular endocrine islets from duct walls. In one child, the lobulate tissue is mainly composed of endocrine islets with small or irregular duct-like hollows and pseudo-acinar figures. In this syndrom of endocrine metaplasia related to “B-cell nesidioblastosis”, a defect in regulation of the differenciation and secretory mechanism of the primitive insulin cells could be attributed to a neoplasic process of the diffuse endocrine system cells.

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