Unravelling a mystery of hypokalemic hypertension- a rare case report of a reninoma.

IF 2.8 3区 医学 Q3 ENDOCRINOLOGY & METABOLISM BMC Endocrine Disorders Pub Date : 2024-12-18 DOI:10.1186/s12902-024-01804-0
N S W Pathirana, P Dissanayake, S Pathmanathan, M R Sumanatilleke, M D U Eranthaka, D A Herath, T M Samarasinghe, A D P Athukorala
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Abstract

Background: 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.

Case presentation: 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.

Conclusion: 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.

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揭开低钾性高血压的神秘面纱——一个罕见的肾鞘瘤病例报告。
背景:肾鞘瘤是一种罕见的继发性高血压的病因,如果在任何靶器官损害发生之前及早发现,可以通过手术治愈。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,确认诊断和定位这种罕见的罪魁祸首病变是具有挑战性的。病例介绍:我们描述了一个19岁女孩因肾鞘瘤引起的年轻高血压病例。她患有顽固性高血压,伴有明显的低钾血症,这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高血压的肾血管原因。虽然肾动脉解剖正常,但左肾下极有外生性肾病变。因此,我们怀疑诊断为肾鞘瘤。她接受了肾静脉取样以确认检测到的肿瘤的功能,但结果不确定。经过多学科的讨论,基于临床证据,认为肾脏病变为肾腺瘤,并行部分肾切除术,切除病变。切除后立即血压和钾恢复正常,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鞘瘤。结论:年轻低钾性高血压患者在排除常见病因的情况下,应考虑肾膜瘤,因为及时治疗并切除罪魁祸首病变可治愈高血压并预防相关的发病率和死亡率。
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来源期刊
BMC Endocrine Disorders
BMC Endocrine Disorders ENDOCRINOLOGY & METABOLISM-
CiteScore
4.40
自引率
0.00%
发文量
280
审稿时长
>12 weeks
期刊介绍: BMC Endocrine Disorders is an open access, peer-reviewed journal that considers articles on all aspects of the prevention, diagnosis and management of endocrine disorders, as well as related molecular genetics, pathophysiology, and epidemiology.
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