Sheth K J, Tang T T, Blaedel M E, Good T A
J Pediatr. 1978 Jun;92(6):921-4. doi: 10.1016/s0022-3476(78)80361-8.
A 16-month-old black male infant had unusual thirst, polyuria, hyponatremia, and hypertension. His polyuria was unresponsive to vasopressin therapy, and his high blood pressure was not effectively controlled by antihypertensive drugs. Radiographic examinations revealed an occult Wilms tumor in the right kidney. After removal of the tumor, the signs and symptoms were relieved. The tumor had a renin activity about 280 times that of the adjacent renal cortex, and many intracytoplasmic secretory granules were found on electron microscopy. The pathogenesis of these clinical manifestations appears to be mediated through the physiologic pathways of renin-angiotensin II and renin-aldosterone.
一名16个月大的黑人男婴出现异常口渴、多尿、低钠血症和高血压。他的多尿对加压素治疗无反应,高血压也未被抗高血压药物有效控制。影像学检查发现右肾有一个隐匿性肾母细胞瘤。切除肿瘤后,症状和体征得到缓解。肿瘤的肾素活性约为相邻肾皮质的280倍,电子显微镜检查发现许多胞质内分泌颗粒。这些临床表现的发病机制似乎是通过肾素-血管紧张素II和肾素-醛固酮的生理途径介导的。