Mitchell J D, Baxter T J, Blair-West J R, McCredie D A
Arch Dis Child. 1970 Jun;45(241):376-84. doi: 10.1136/adc.45.241.376.
The case history is reported of a child aged 1 year 10 months with malignant hypertension in association with Wilms' tumour. In addition there was a marked electrolyte disturbance, with hyponatraemia and hypokalaemia, and a blood picture of microangiopathic haemolytic anaemia. All these features resolved after removal of the kidney and tumour. Pre-operative plasma renin concentration was extremely high and had returned to normal 3 months after operation. The renal tumour showed a marked degree of differentiation, with glomerulus-like structures attached to tubules, and significant quantities of renin were found on assay. No renin was detected in the renal cortex of the affected kidney. In two other normotensive patients with Wilms' tumour no renin could be detected in the tumour tissue. We consider that the evidence suggests that this patient had a renin-secreting nephroblastoma.
报告了一名1岁10个月大儿童的病例,该患儿患有与肾母细胞瘤相关的恶性高血压。此外,还存在明显的电解质紊乱,伴有低钠血症和低钾血症,以及微血管病性溶血性贫血的血象。切除肾脏和肿瘤后,所有这些特征均消失。术前血浆肾素浓度极高,术后3个月恢复正常。肾肿瘤显示出明显的分化程度,有类似肾小球的结构附着于肾小管,检测发现有大量肾素。在患侧肾脏的肾皮质中未检测到肾素。在另外两名血压正常的肾母细胞瘤患者的肿瘤组织中也未检测到肾素。我们认为,证据表明该患者患有分泌肾素的肾母细胞瘤。