Familial islet-cell adenomatosis.
Familial multiple adenoma of pancreatic beta-cells is described for the first time. The occurrence of diabetes mellitus in different members of the family raises the possibility of a common genetic origin for the multiple islet-cell adenomas and the diabetic trait. The evidence suggests that this gene is autosomal and dominant, that it is not linked with the HLA antigens, and that is causes an abnormal sensitivity of the beta-cells, which become hyperplastic or hypofunctional.
D007516:Adenoma, Islet Cell / Q000175:diagnosis / Q000209:etiology; D000328:Adult; D001786:Blood Glucose / Q000032:analysis; D048909:Diabetes Complications; D005260:Female; D005934:Glucagon; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D007328:Insulin / Q000097:blood; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis / Q000209:etiology; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000209:etiology; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000209:etiology; D010375:Pedigree; D014044:Tolbutamide
D001786:Blood Glucose; D006680:HLA Antigens; D007328:Insulin; D005934:Glucagon; D014044:Tolbutamide