Kim Ji Hye, Kim Ji Hyun, Cho Myung Hyun, Park Eujin, Hyun Hye Sun, Ahn Yo Han, Kang Hee Gyung, Moon Kyung Chul, Ha Il-Soo, Cheong Hae Il
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Department of Pediatrics, Hallym University Kangnam Sacred Heart Hospital, Seoul, Korea.
Korean J Pediatr. 2019 Apr;62(4):144-147. doi: 10.3345/kjp.2018.06926. Epub 2018 Oct 29.
The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.
儿童中最常见的难治性高血压类型是继发性高血压，这是一种潜在可治愈的疾病。肾素瘤是一种分泌肾素的球旁细胞瘤，是严重高血压的罕见病因，通常在青少年和年轻成年人中被诊断出来。手术切除肿瘤可完全治愈肾素瘤患者的高血压。肾素瘤的典型临床表现包括低钾血症、代谢性碱中毒以及肾素 - 血管紧张素系统激活增加继发的特征，且无肾动脉狭窄。我们报告一例具有典型临床表现的女性青少年肾素瘤病例，其中手术切除肿瘤完全治愈了高血压。我们在此讨论用于确立肾素瘤诊断及病情管理的肿瘤临床特征、影像学检查和免疫组化检查。
