Aim: Description of a series of children and adolescents with Primary Aldosteronism (PA) diagnosed and treated in our Outpatient Hypertension Clinic from 1994 to 2018.
Methods: Review of the clinical records and of the relevant literature. Values are given as mean +s.d.
Results: 16 of a total of 49 patients younger than 20 years of age referred to our Hypertension Clinic from 1994 to 2018 were diagnosed of PA (33%), while 27 (55%) were diagnosed of essential hypertension and the other 6 (12%) of miscellaneous causes. Two of the patients with PA (13%) already had shown transient cerebrovascular complications. Their mean age was 15.2+3.1 yr (range 819). Eleven (69%) were female. Their untreated blood pressure (ABPM): 164+23/96+18 mmHg, heart rate 79+13 bpm (awaken), and 158+22/89+18 mmHg, 75+14 lpm (sleeping). 13 (81%) had confirmed hypertension by ABPM. eGFR (CKD/EPI) 98+27 ml/min/1.73 m2, low in 3 patients (19%). Kalemia: 3.2+0.7 mEq/l, low in 9 (56%). Plasma aldosterone: 58.7+36.2 ng/dl; plasma renin activity (PRA): 0.52+0.37 ng/mL per h, avoiding pharmacological interferences. Confirmation test was not performed in patients with obvious hypokalemia. Eleven (69%) underwent a captopril test, and 3 (19%) a saline infusion test. 15 had abdominal CT scan, with unilateral adenoma found in 6 (37%) and bilateral adenomas in 1(6%). Adrenal venous sampling (AVS) was performed in this patient, but aldosterone was symmetrical, and surgery was not considered. All 6 patients with unilateral adenomas underwent surgery and PA was biochemically resolved in all of them, but two of them still needed antihypertensive treatment. One patient was diagnosed of type 1 familial aldosteronism and is treated with low-dose dexametasone. The other 9 patients are receiving treatment based on mineralocorticoid receptor antagonists, and 8 of them (89%) maintain normal blood pressure, normokalemia and unsuppressed PRA (>1 ng/ml per h), although most maintain high plasma aldosterone. The final diagnoses were: idiopathic PA in 6 patients (37%), Lityński-Conn adenomas in 7 patients (44%), type II familial aldosteronism in 2 patients (13%) and type 1 familial aldosteronism in one patient (6%).
Conclusions: PA was the main cause of secondary hypertension, and is complicated with organ damage in 1/3 of the patients. Half had hypokalemia. Idiopathic PA and functioning adenomas had similar prevalence. Confirmation test and CT is required in most patients, but rarely AVS. Patients with unilateral adenomas achieve biochemical cure. MCRA control blood pressure, potassium and PRA in most of the rest.
18 - 21 May 2019
European Society of Endocrinology