DIFFUSE AND FOCAL NESIDIOBLASTOSIS - A CLINICOPATHOLOGICAL STUDY OF 24 PATIENTS WITH PERSISTENT NEONATAL HYPERINSULINEMIC HYPOGLYCEMIA
DIFFUSE AND FOCAL NESIDIOBLASTOSIS - A CLINICOPATHOLOGICAL STUDY OF 24 PATIENTS WITH PERSISTENT NEONATAL HYPERINSULINEMIC HYPOGLYCEMIA
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DOI：
10.1097/00000478-198909000-00006
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发表时间：
1989-09-01
影响因子：
5.6
通讯作者：
KLOPPEL, G
中科院分区：
文献类型：
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作者：
GOOSSENS, A;GEPTS, W;KLOPPEL, G
作为持久性新生儿高胰岛素性低血糖症（PNHH）基础的内分泌胰腺形态异常，包括在“nesidioblastosis”的标题下，似乎是异质性的。我们对24例患者的胰腺标本进行了光镜和免疫细胞化学的研究，目的是对胰腺内分泌的变化进行分类，并将其与所采用的治疗方法联系起来。nesidioblastosis主要有两种类型：局灶性和弥漫性。这两种类型发生的频率相同。局灶性肾母细胞病的特征是胰岛样细胞团的结节性增生，包括管状岛复合体和巨核肥大的胰岛素细胞。在9例患者中，该病变是单灶性的（包括一个腺瘤样结节），而2例患者各有两个独立的灶，1例患者至少有三个灶。弥漫性肾母细胞病累及整个胰腺；它的特点是大小不规则的胰岛和导管-胰岛复合物，两者都含有明显肥大的胰岛素细胞。三种胰腺缺乏这些诊断特征，因此提出了困难的诊断问题。从随访数据中，我们得出结论，对于大多数局灶性肾母细胞病患者来说，切除病变病灶的部分胰腺切除术是治疗的选择，而弥漫性肾母细胞病则需要近全胰切除术。在两例分别在6岁和8岁时接受治疗的多灶性和弥漫性nesidioblastosis患者中，胰腺内分泌的变化与年轻年龄组相当，没有显示出任何成熟的迹象。
The morphological abnormalities of the endocrine pancreas that underlie persistent neonatal hyperinsulinemic hypoglycemia (PNHH) and are included under the heading "nesidioblastosis" appear to be heterogeneous. We studied pancreatic specimens of 24 patients by light microscopy and immunocytochemistry in order to classify the changes of the endocrine pancreas and relate them to the therapy applied. Two main forms of nesidioblastosis were recognized: a focal and a diffuse type. Both types occurred with equal frequency. Focal nesidioblastosis was characterized by nodular hyperplasia of islet-like cell clusters, including ductuloinsular complexes and hypertrophied insulin cells with giant nuclei. In nine patients, this lesion was unifocal (including an adenoma-like nodule), while two patients each had two separate foci, and one patient had at least three foci. Diffuse nesidioblastosis involved the entire pancreas; it was distinguished by irregularly sized islets and ductulo-insular complexes, both of which contained distinctly hypertrophied insulin cells. Three pancreases lacked these diagnostic features and thus posed difficult diagnostic problems. From the follow-up data, we conclude that partial pancreatectomy with excision of the diseased focus is the treatment of choice for most patients with focal nesidioblastosis whereas diffuse nesidioblastosis requires near-total pancreatectomy. In two patients who had multifocal and diffuse nesidioblastosis and were treated at the age of 6 and 8 years, respectively, the changes of the endocrine pancreas were comparable with those of the young age group and failed to reveal any signs of maturation.