Nesidioblastosis和持久性新生儿高胰岛素血症。

Diabete & metabolisme Pub Date : 1995-12-01
C Sempoux, F Poggi, F Brunelle, J M Saudubray, C Fekete, J Rahier
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引用次数: 0

摘要

新生儿高胰岛素血症的特点是严重的低血糖,可引起严重的神经系统影响。胰腺形态学异常包括局灶性或弥漫性病变。前者可以通过切除治愈,而后者由于发病机制不确定,通常需要行胰腺次全切除术。我们研究了各种假说,试图解释后一种形式的高胰岛素血症的起源。我们确定nesidioblastosis,长期以来被认为是弥漫性高胰岛素血症的基本结构病变,不是特异性的,也不对应于内分泌细胞的持续增殖。我们发现可以排除β细胞质量的增加,因为高胰岛素血症婴儿的β细胞体积密度并不比对照组高。D细胞减少的假设很有吸引力,但应谨慎考虑,因为在低血糖婴儿中观察到的D细胞体积密度下降是不稳定的。最后,β细胞功能异常的概念似乎是最可能的解释,因为在高尔基区通过特异性抗体检测到较高数量的胰岛素原,并且在一些细胞中观察到具有丰富细胞质的异常细胞核。术中形态学检查可观察到这些组织学异常。功能活性也可通过研究胰岛素原的信使RNA来评价。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Nesidioblastosis and persistent neonatal hyperinsulinism.

Neonatal hyperinsulinism is characterized by severe hypoglycaemia which can cause serious neurologic effects. Pancreatic morphological abnormalities involve either focal or diffuse lesions. The former can be cured by resection, whereas the latter, of uncertain pathogenesis, often require subtotal pancreatectomy. We investigated various hypotheses in an effort to explain the origin of this latter form of hyperinsulinism. We determined that nesidioblastosis, long considered to be the basic structural lesion of the diffuse form of hyperinsulinism, is not specific and does not correspond to a continuous proliferation of endocrine cells. We found that an increase in beta-cell mass can be excluded since the volume density of beta cells is not systematically higher in hyperinsulinemic infants than in controls. The hypothesis of a decrease in D cells is attractive but should be considered with due caution since the decrease of the D-cell volume density observed in hypoglycaemic infants is inconstant. Finally, the notion of beta-cell functional abnormality seems the most likely explanation since a higher quantity of proinsulin was detected within the Golgi area by a specific antibody and abnormal nuclei with abundant cytoplasm were observed in some cells. These histological abnormalities can be observed during intraoperative morphological examination. Functional activity might also be evaluated by studying the messenger RNA of proinsulin.

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