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儿童肾肿瘤中原发性和继发性肾素增多症的表现。

Demonstration of both primary and secondary reninism in renal tumors in children.

作者信息

Yokomori K, Hori T, Takemura T, Tsuchida Y

机构信息

Department of Pediatric Surgery, Japanese Red Cross Medical Center, Tokyo.

出版信息

J Pediatr Surg. 1988 May;23(5):403-9. doi: 10.1016/s0022-3468(88)80435-4.

Abstract

Two infants with renal tumors and associated hypertension are presented. By using an antibody to purified human renal renin, the sites of renin production were localized immunohistochemically in each tumor. The first case was a 9-month-old girl with Beckwith-Wiedemann syndrome. She presented with bilateral renal masses and hypertension (140/90 mm Hg). Following a left nephrectomy and chemotherapy and radiotherapy, her BP returned to normal. Her tumor was a Wilms' tumor of favorable histology, composed predominantly of glomeruloid structures. Renin was localized within a part of these neoplastic glomeruloid bodies. We therefore designated this as a Wilms' tumor with glomeruloid differentiation having primary reninism. The second case was a 24-day-old girl with hypertension (140/70 mm Hg). A renal tumor was found and successfully removed. Her BP returned to normal. The tumor was histologically confirmed as a congenital mesoblastic nephroma. By indirect immunoperoxidase staining, renin was localized only in the hypertrophied juxtaglomerular cells adjacent to the residual glomeruli entrapped by the tumor. None was seen in the tumor cells. We concluded that this was a case of secondary reninism--a case of hypertension secondary to the local ischemia at the entrapped glomeruli.

摘要

本文报告了两名患有肾肿瘤并伴有高血压的婴儿。通过使用针对纯化人肾素的抗体,免疫组织化学定位了每个肿瘤中肾素的产生部位。第一例是一名9个月大患有贝克威思-维德曼综合征的女孩。她表现为双侧肾肿块和高血压(140/90毫米汞柱)。在进行左肾切除、化疗和放疗后,她的血压恢复正常。她的肿瘤是组织学表现良好的肾母细胞瘤,主要由肾小球样结构组成。肾素定位于这些肿瘤性肾小球样体的一部分内。因此,我们将其命名为具有肾小球样分化并伴有原发性肾素增多症的肾母细胞瘤。第二例是一名24天大患有高血压(140/70毫米汞柱)的女孩。发现并成功切除了一个肾肿瘤。她的血压恢复正常。肿瘤经组织学确诊为先天性中胚叶肾瘤。通过间接免疫过氧化物酶染色,肾素仅定位于与肿瘤包绕的残余肾小球相邻的肥大球旁细胞中。肿瘤细胞中未发现肾素。我们得出结论,这是一例继发性肾素增多症——一例由包绕的肾小球局部缺血继发的高血压病例。

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