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KMID : 1130320190620040144
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Korean Journal of Pediatrics
2019 Volume.62 No. 4 p.144 ~ p.147
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Reninoma: a rare cause of curable hypertension
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Kim Ji-Hye
Kim Ji-Hyun
Cho Myung-Hyun
Park Eu-Jin
Hyun Hye-Sun
Ahn Yo-Han
Kang Hee-Gyung
Moon Kyung-Chul
Ha Il-Soo
Cheong Hae-Il
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Abstract
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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.
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KEYWORD
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Renal hypertension, Renin, Juxtaglomerular apparatus, Hypokalemia
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