背景:成人非肿瘤性高胰岛素血症性低血糖(ANHH),也被称为成人发病的痣母细胞病,是成人内源性高胰岛素血症性低血糖的罕见原因。这种疾病的特征是胰腺内分泌细胞的弥漫性增生,并通过病理检查诊断。虽然已经提出了这种疾病的诊断标准,我们建立了更多的定量标准来评估胰岛形态。
方法:我们测量了数字,最大直径,总面积,使用NIS-Elements软件程序,ANHH(n=4)和对照病例(n=5)的代表性部分中包含的胰岛的圆形(代表胰岛与完美球体的接近程度)。我们还测量了平均细胞大小,细胞核增大的细胞百分比,以及三个代表性胰岛中每一个具有可识别核仁的细胞百分比。我们还评估了五名经验丰富的病理学家和七名经验不足的病理学家之间的ANHH观察者之间的诊断一致性。
结果:数量没有显着差异,最大直径,或两组之间的胰岛总面积,即使在每单位面积校正这些参数。然而,ANHH标本中每个胰腺实质总面积中圆形度低(<0.71)的胰岛数量明显多于对照组.我们还发现,ANHH组具有可识别核仁的细胞百分比明显高于对照组。各组之间的平均细胞大小或细胞核增大的细胞数量没有显着差异。有经验的病理学家盲检正确诊断率为47.5%±6.12%,经验较少的病理学家为50.0%±8.63%,没有显著差异。
结论:低圆度,这表明一个不规则的胰岛形状,在以前的报告中被称为“胰岛的不规则形状和偶尔的扩大”和“分叶状胰岛结构”,是诊断ANHH的有用标记。可识别的核仁百分比增加,对应于β细胞中的大核,\"具有潜在的诊断价值。
BACKGROUND: Adult non-neoplastic hyperinsulinemic hypoglycemia (ANHH), also known as adult-onset
nesidioblastosis, is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. This disease is characterized by diffuse hyperplasia of pancreatic endocrine cells and is diagnosed by a pathological examination. While diagnostic criteria for this disease have already been proposed, we established more quantitative criteria for evaluating islet morphology.
METHODS: We measured the number, maximum diameter, total area, and circularity (representing how closely islets resemble perfect spheres) of islets contained in representative sections of ANHH (n = 4) and control cases (n = 5) using the NIS-Elements software program. We also measured the average cell size, percentage of cells with enlarged nuclei, and percentage of cells with recognizable nucleoli for each of three representative islets. We also assessed the interobserver diagnostic concordance of ANHH between five experienced and seven less-experienced pathologists.
RESULTS: There was no significant difference in the number, maximum diameter, or total area of islets between the two groups, even after correcting for these parameters per unit area. However, the number of islets with low circularity (< 0.71) per total area of the pancreatic parenchyma was significantly larger in ANHH specimens than in controls. We also found that the percentage of cells with recognizable nucleoli was significantly higher in the ANHH group than in the controls. There were no significant differences in the average cell size or the number of cells with enlarged nuclei between the groups. The correct diagnosis rate with the blind test was 47.5% ± 6.12% for experienced pathologists and 50.0% ± 8.63% for less-experienced pathologists, with no significant differences noted.
CONCLUSIONS: Low circularity, which indicates an irregular islet shape, referred to as \"irregular shape and occasional enlargement of islets\" and \"lobulated islet structure\" in a previous report, is a useful marker for diagnosing ANHH. An increased percentage of recognizable nucleoli, corresponding to \"macronucleoli in β-cells,\" has potential diagnostic value.