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PS 14-27 EXTRARENAL MANIFESTATIONS AND OUTCOMES IN CHILDREN WITH RENOVASCULAR HYPERTENSION

Objective: Prevalence of hypertension in children is estimated to be between 1% and 2%, and children with secondary hypertension usually are younger and have higher blood pressure (BP). Renovascular hypertension accounts for 5–10% of children with hypertension, and could be associated with underlying disease involving other organs. This study reviewed the clinical characteristics of children with renovascular hypertension due to renal artery stenosis. Design and Method: The medical records of 25 Korean children who were newly diagnosed with renovascular hypertension from January 2000 to December 2015 in Samsung Medical Center were retrospectively analyzed. Results: The male-to-female ratio of the patients was 1.1:1. The median age at diagnosis was 12 years. Sixteen patients (64%) had underlying disease including Moyamoya disease (n = 9), vasculitis (n = 3), Klippel-Trenaunay-Weber syndrome (n = 1), neurofibromatosis (n = 1), vesicoureteral reflux (n = 1), and multicystic dysplastic kidney (n = 1). Two patients with Moyamoya disease were siblings. Among 9 patients with isolated renal artery stenosis, 4 patients were diagnosed with fibromuscular dysplasia. All patients showed the stage 2 hypertension, and received the medication at the initial diagnosis. Angioplasty was tried in 21 patients, but failed in 4 patients. One patient with Takayasu's arteritis received the nephrectomy, and showed normal BP after operation without medication. One patient with neurofibromatosis received coil-embolization. During follow-up, BP was well-controlled in 12 patients, but only 4 patients stopped the medication. Eight patients showed the target-organ damage including posterior reversible encephalopathy syndrome (n = 3), heart failure (n = 2), and retinopathy (n = 3). Five patients (20 %) progressed to chronic kidney disease. Conclusions: Most common underlying disease in Korean children with renovascular hypertension is Moyamoya disease, and special concern for development of hypertension is necessary in children with underlying disease associated with renovascular disease. It is difficult for angioplasty alone to control BP effectively, and combined treatment is necessary to prevent target organ damage.