An Unusual Cause of Pediatric Hypertension

Hypertension and hypokalemia in a 15-year-old boy: answer

“…The most common presenting symptom was headache (48% of the patients), as in our patient [5]. Hypokalemia was detected in 85% of previously reported patients, with a mean serum K level of 2.9 mEq/l [1]. PRA was markedly elevated, in which the mean PRA was 12±11 -fold higher than the upper limit of normal [5].…”

“…Other renin-secreting tumors, such as Wilm's tumor or rhabdoid tumor of the kidney, should be considered in the differential diagnosis [1]. Approximately 90 cases of reninoma have been reported to date, and the mean age at diagnosis was 27 years (range 6-69 years) [5].…”

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AnswersDuring any evaluation of patients with hypertension and hypokalemic metabolic alkalosis, hormones, including plasma renin activity (PRA), serum aldosterone, and serum cortisol concentration, should be checked [1]. The results of these hormone analyses can indicate the specific area requiring a more specific evaluation.

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“…These results allowed us to rule out true mineralocorticoid excess, such as primary aldosteronism or glucocorticoid-remediable aldosteronism. We also ruled out non-aldosterone mineralocorticoid excess, which is observed in patients with congenital adrenal hyperplasia or in apparent mineralocorticoid excess, because the suppression of both PRA and serum aldosterone concentration is expected in these diseases [1]. The results steered us towards a diagnosis of secondary aldosteronism with excess renin secretion, a condition that can occur in renal parenchymal disease associated with inflammation or scarring, renovascular diseases, or aortic coarctation [2].…”

Reninoma presenting as cardiac syncope

“…Appropriate detection and diagnosis of this tumor is imperative because it is a surgically curable cause of severe hypertension, which can be associated with major complications including intestinal ischemia, cerebrovascular accidents, heart disease and death. [9]…”