Luciani J C, Baldet P, Dumas R, Jean R
Arch Fr Pediatr. 1979 Mar;36(3):240-9.
Two children who presented with Wilm's tumour and severe hypertension are described. The hypertension, which was secondary to excessive renin secretion, regressed after unilateral nephrectomy. In one child the total quantity of renin in the tumour was high and the peripheral plasma renin was also increased. The latter was unaffected by posture or a low salt diet and on angiography there was no compression of the renal arteries. Electron microscopy of the tumour cells showed numerous intra-cytoplasmic granules. In the other child the peripheral renin was only moderated elevated and could be stimulated by changes in posture or a low salt diet. Angiography demonstrated a large tumour that was compressing the renal artery on that side. No renin was detected in the tumour. In this child the inappropriate secretion of renin was probably due to renal parenchyma close to the tumour causing reno-vascular hypertension. The anti-hypertensive effect of propranolol given pre-operatively was excellent.
本文描述了两名患有威尔姆斯瘤(肾母细胞瘤)并伴有严重高血压的儿童。这种高血压继发于肾素分泌过多,单侧肾切除术后血压恢复正常。其中一名儿童肿瘤中的肾素总量较高,外周血浆肾素也增加。外周血浆肾素不受体位或低盐饮食影响,血管造影显示肾动脉无受压情况。肿瘤细胞的电子显微镜检查显示有大量胞浆内颗粒。另一名儿童外周肾素仅中度升高,可因体位改变或低盐饮食而受到刺激。血管造影显示有一个大肿瘤压迫患侧肾动脉。肿瘤中未检测到肾素。在该儿童中,肾素的不适当分泌可能是由于靠近肿瘤的肾实质导致肾血管性高血压。术前给予普萘洛尔的降压效果极佳。