Pathirana N S W, Dissanayake P, Pathmanathan S, Sumanatilleke M R, Eranthaka M D U, Herath D A, Samarasinghe T M, Athukorala A D P
National Hospital of Sri Lanka, Colombo, Sri Lanka.
Faculty of Medicine, University of Moratuwa, Colombo, Sri Lanka.
BMC Endocr Disord. 2024 Dec 18;24(1):271. doi: 10.1186/s12902-024-01804-0.
Reninoma is a rare cause of secondary hypertension, which can be cured with surgery if identified early before any target organ damage occurs. It leads to hypokalaemia and hypertension and typically responds well to treatment with renin-angiotensin-aldosterone system blockers. However, confirmation of the diagnosis and the localisation of this rare culprit lesion can be challenging.
We describe a case of young-onset hypertension in a 19-year-old girl due to a reninoma. She had resistant hypertension with marked hypokalaemia, which required exceedingly high doses of potassium supplements. Biochemical Investigations revealed secondary hyperaldosteronism. Thus, she underwent a renal angiogram to exclude a renovascular cause for her hypertension. While the renal artery anatomy was normal, there was an exophytic renal lesion in the lower pole of the left kidney. Hence, the diagnosis of a reninoma was suspected. She underwent renal vein sampling to confirm the functionality of the detected tumour, but the results were inconclusive. After a multidisciplinary discussion, based on the clinical evidence, the renal lesion was thought to be a reninoma and a partial nephrectomy was done, removing the lesion. Immediately following resection, her blood pressure and potassium normalised without further drug treatment, and the resected lesion was later confirmed to be a reninoma by histopathological examination.
In young people with hypokalemic hypertension, reninoma should be considered when the more common causes are excluded since prompt treatment with excision of the culprit lesion can cure hypertension and prevent associated morbidity and mortality.
肾素瘤是继发性高血压的罕见病因,如果在任何靶器官损害发生之前早期确诊,可通过手术治愈。它导致低钾血症和高血压,通常对肾素 - 血管紧张素 - 醛固酮系统阻滞剂治疗反应良好。然而,确诊这种罕见的致病病变及其定位可能具有挑战性。
我们描述了一名19岁女孩因肾素瘤导致的青年期高血压病例。她患有顽固性高血压并伴有明显的低钾血症,需要极高剂量的钾补充剂。生化检查显示继发性醛固酮增多症。因此,她接受了肾血管造影以排除其高血压的肾血管病因。虽然肾动脉解剖结构正常,但左肾下极有一个外生性肾脏病变。因此,怀疑为肾素瘤。她接受了肾静脉采样以确认检测到的肿瘤的功能,但结果尚无定论。经过多学科讨论,根据临床证据,认为该肾脏病变为肾素瘤,并进行了部分肾切除术,切除了病变。切除后,她的血压和血钾立即恢复正常,无需进一步药物治疗,切除的病变后来经组织病理学检查证实为肾素瘤。
在排除更常见病因的低钾性高血压年轻人中,应考虑肾素瘤,因为及时切除致病病变可治愈高血压并预防相关的发病率和死亡率。