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一名儿童患佐林格-埃利森综合征并伴有肾胃泌素瘤。

Zollinger-Ellison syndrome associated with a renal gastrinoma in a child.

作者信息

Nord K S, Joshi V, Hanna M, Khademi M, Saad S, Marquis J, Pelzman H, Verner E

出版信息

J Pediatr Gastroenterol Nutr. 1986 Nov-Dec;5(6):980-6. doi: 10.1097/00005176-198611000-00030.

DOI:10.1097/00005176-198611000-00030
PMID:2878987
Abstract

Renal gastrinoma has not been previously reported. A 12-year-old boy with Zollinger-Ellison syndrome was found to have a renal tumor. No other tumor was detectable by imaging techniques, and selective venous sampling for gastrin showed a significant renal vein to vena cava gradient. Nephrectomy was performed, and examination of the tumor showed typical histologic features of an endocrine tumor. G cells were apparent by electron microscopy, and immunoperoxidase staining for gastrin, neuron-specific enolase, and chromogranin were positive. The gastrin content was unusually low for gastrinomas: 128 pg/g. Following nephrectomy, fasting gastrin and secretin stimulation testing were normal. Basal acidity was reduced by 60% but remained elevated at 39 mmol H +/h (hydrogen ion per hour). We speculate that renal gastrinoma may be characterized by uniquely poor gastrin storage and that curative resection of all gastrinoma tissue may not necessarily be associated with immediate complete suppression of hyperacidity.

摘要

肾胃泌素瘤此前未见报道。一名患有卓-艾综合征的12岁男孩被发现患有肾肿瘤。影像学检查未发现其他肿瘤,胃泌素选择性静脉采样显示肾静脉至腔静脉存在显著梯度。进行了肾切除术,肿瘤检查显示具有内分泌肿瘤的典型组织学特征。电子显微镜下可见G细胞,胃泌素、神经元特异性烯醇化酶和嗜铬粒蛋白的免疫过氧化物酶染色呈阳性。该胃泌素瘤的胃泌素含量异常低:128 pg/g。肾切除术后,空腹胃泌素和促胰液素刺激试验正常。基础酸度降低了60%,但仍升高至39 mmol H⁺/h(每小时氢离子)。我们推测肾胃泌素瘤的特征可能是胃泌素储存特别差,并且所有胃泌素瘤组织的根治性切除不一定与胃酸过多的立即完全抑制相关。

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