Department of Endocrinology and metabolism, Guangzhou Red Cross Hospital of Jinan University, No. 396, Tong Fu Zhong Rd, Guangzhou, 510220, China.
Department of Urology, Guangzhou Red Cross Hospital of Jinan University, No. 396, Tong Fu Zhong Rd, Guangzhou, 510220, China.
BMC Endocr Disord. 2024 Nov 14;24(1):244. doi: 10.1186/s12902-024-01770-7.
Adolescents with secondary hyperaldosteronism often present with severe and treatment-resistant hypertension, along with hypokalemia. Renovascular hypertension is frequently caused by renal artery stenosis, primarily due to atherosclerosis and fibromuscular dysplasia (FMD). The presence of an accessory renal artery (ARA) is a common anatomical variation that can contribute to secondary renal vascular hypertension. However, FMD occurring in the ARA is a rare cause of renal vascular hypertension. Juxtaglomerular cell tumor (JGCT) represents a rare etiology of renal hypertension. The co-occurrence of the pathogenic ARA with JGCT is infrequently reported in the existing literature.
This case study presents a young individual with a 12-year history of resistant hypertension, initially diagnosed with pathogenic ARA but later confirmed as JGCT 4 years later. Following surgery for JGCT, the patient experienced only temporary stabilization of blood pressure without anti-hypertensive medication. Stenosis of the ARA was definitively diagnosed one and a half years post-surgery, with FMD occurring on the ARA strongly suspected. The patient underwent balloon dilatation angioplasty 3 years later, leading to sustained blood pressure stability with the use of two medications.
The case study discussed herein involves a patient with resistant hypertension initially diagnosed with ARA but later determined to have late-onset JGCT and renal artery stenosis. It is imperative to consider atypical JGCT in young patients exhibiting resistant hypertension, hypokalemia, and hyperreninemia. Adequate management of renal artery stenosis is crucial in the management of hyperreninemic hypertension.
患有继发性醛固酮增多症的青少年常表现为严重且治疗抵抗性高血压,同时伴有低钾血症。肾血管性高血压常由肾动脉狭窄引起,主要由动脉粥样硬化和纤维肌性发育不良(fibromuscular dysplasia,FMD)引起。副肾动脉(accessory renal artery,ARA)的存在是导致继发性肾血管性高血压的常见解剖变异。然而,ARA 中的 FMD 是导致肾血管性高血压的罕见原因。肾小球旁细胞瘤(juxtaglomerular cell tumor,JGCT)是肾高血压的罕见病因。在现有的文献中,致病的 ARA 与 JGCT 同时存在的情况很少被报道。
本病例研究介绍了一名年轻患者,其有 12 年难治性高血压病史,最初被诊断为致病性 ARA,但 4 年后被确诊为 JGCT。在因 JGCT 进行手术后,患者的血压仅暂时得到稳定,未服用抗高血压药物。手术后 1 年半时,明确诊断为 ARA 狭窄,强烈怀疑 ARA 上存在 FMD。3 年后,患者接受了球囊扩张血管成形术,同时使用两种药物,血压持续稳定。
本文讨论的病例涉及一名患者,其最初被诊断为 ARA 所致难治性高血压,但后来被确定为迟发性 JGCT 和肾动脉狭窄。对于表现出难治性高血压、低钾血症和高肾素血症的年轻患者,需要考虑不典型 JGCT。充分管理肾动脉狭窄是治疗高肾素性高血压的关键。