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Abstract: TH-PO493

Clinical Characteristics of Korean Pediatric Renovascular Hypertension According to Underlying Diseases

Session Information

  • Pediatric Nephrology - I
    November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology


  • Joung, Jinwoon, Samsung Medical Center, Gangnam-gu, Seoul, Korea (the Republic of)
  • Cho, Heeyeon, Samsung Medical Center, Gangnam-gu, Seoul, Korea (the Republic of)

Previously, Moyamoya disease (MMD) has been suggested as the most common cause of renovascular hypertension (RVH) in Korean children. The ring finger protein 213 (RNF213) has also been reported to be a causative gene of systemic vasculopathy in East Asia which results in MMD and RVH. This study aimed to evaluate the clinical characteristics of RVH and to compare the outcomes according to underlying diseases in Korean children.


A retrospective study was performed in patients who were diagnosed RVH at age under 18 years in Samsung Medical Center from 2010 to 2022. Medical records including sex, age, gene study, location of vascular lesion, treatment modalities and response to treatment were reviewed. RNF213-related vasculopathy designates systemic vasculopathy involving intracranial, renal, and pulmonary arteries with defined RNF213 mutation. According to underlying diseases, patients were grouped as RNF213-related vasculopathy (group 1, n=17), atypical vasculopathy (group 2, n=14), and others including congenital anomalies of kidney and urinary tract (CAKUT) and solid tumor (group 3, n=10).


A total of 41 pediatric patients (19 males and 22 females, mean age of 10.1±4.8 years) were included. The mean value of initial blood pressure (BP) was 162/96 mmHg. Twenty five children were incidentally detected hypertension without symptoms while 15 patients showed neurological symptoms. Five patients were diagnosed RVH prior to MMD. Twenty patients underwent percutaneous transluminal angioplasty (PTA), 3 patients received bypass surgery, and 3 patients were done nephrectomy. Target organ damage was found in 15 patients (36.6%). Ostial vascular involvement was notably prevalent in group 1. For response to treatment, BP and stenotic lesions were poorly controlled in group 1 and 2 while overall outcome was relatively favorable in group 3. However, no statistical differences were revealed for the location and level of stenosis, treatment modalities, and outcomes between 3 groups.


The RNF213-related vasculopathy including MMD is the most common cause of RVH in Korean children in association with ostial vascular lesions. PTA and bypass surgery are mostly performed for patients with RNF213-related or atypical vasculopathy and clinical outcomes are relatively poor in these patients compared to other underlying diseases such as CAKUT or solid tumor.