Humans ; Renal Artery ; Constriction, Pathologic ; Hypertension, Renovascular/therapy* ; Vascular Diseases ; Renal Artery Obstruction/therapy* ; Embolization, Therapeutic

Infant, Newborn ; Humans ; Fibromuscular Dysplasia/complications* ; Renal Artery ; Hypertension, Renovascular/etiology* ; Renal Artery Obstruction/complications* ; Hypertension/complications*

Fibromuscular Dysplasia/diagnostic imaging* ; Humans ; Hypertension/complications* ; Hypertension, Renovascular/etiology* ; Kidney ; Renal Artery/diagnostic imaging*

PURPOSE: This study aimed to determine the diagnostic value of the relative filtration fraction (RFF) assessed by dynamic ⁹⁹mTc-diethylenetriaminepentaacetic acid (⁹⁹mTc-DTPA) renal scintigraphy after angiotensin-converting enzyme (ACE) inhibition for renovascular hypertension (RVHT) diagnosis.METHODS: ⁹⁹mTc-DTPA captopril renal scintigraphy performed in adolescents or adults (≥ 10 years) with suspected RVHT was retrospectively reviewed. The RFF of the affected kidney was qualitatively assessed as the relative glomerular filtration rate during the 2 to 3-min period compared with the relative perfusion during the first 60 s (qualitative RFF) and scored from 1 (definitely same) to 5 (definitely decreased). The quantitative RFF of the affected kidney was obtained by dividing the percentage of glomerular filtration rate by the percentage of renal perfusion.RESULTS: Overall, 173 patients (high probability, n = 15; and low probability, n = 158) were included based on conventional captopril renal scintigraphic criteria. An abnormal qualitative RFF was observed in 12 patients with high probability, and the diagnostic sensitivity was 80.0% (95% CI, 51.9–95.7). The RFF was normal in 152 patients with low probability, and the diagnostic specificity was 96.2% (95% CI, 91.9–98.6). The RFF was lower in patients with high probability than in those with low probability (0.79 ± 0.15 vs. 1.02 ± 0.11, P < 0.0001).CONCLUSIONS: The RFF assessed by dynamic ⁹⁹mTc-DTPA renal scintigraphy after ACE inhibition can detect patients with high probability for RVHT. The RFF after ACE inhibition might be a useful diagnostic criterion especially when baseline scintigraphy is not available for evaluating ACE inhibition-induced changes.
Adolescent ; Adult ; Captopril ; Diagnosis ; Filtration ; Glomerular Filtration Rate ; Humans ; Hypertension, Renovascular ; Kidney ; Perfusion ; Radionuclide Imaging ; Retrospective Studies ; Sensitivity and Specificity

BACKGROUND: Renal artery stenosis (RAS) is always associated with abnormalities in renal microvascular perfusion (RMP). However, few imaging methods can simultaneously evaluate the degree of luminal stenosis and RMP. Thus, this study will aim to evaluate the feasibility of using contrast-enhanced ultrasound (CEUS) for assessing both RAS and RMP to achieve a one-stop assessment of patients with suspected renovascular hypertension. METHODS: This will be a single-center diagnostic study with a sample size of 440. Patients with chronic kidney disease (CKD) and suspected of having resistant hypertension will be eligible. Patients with Stages 1-3 CKD will undergo CEUS and computed tomography (CT) angiography (CTA). Values obtained by CEUS and CTA for diagnosing low-grade (lumen reduced by <60%) and high-grade (lumen reduced by ≥60%) RAS will be compared. Moreover, all patients will also undergo radionuclide imaging. The diagnostic value for RAS will be assessed by the receiver operating characteristic curve, including the accuracy, sensitivity, specificity, positive predictive values, negative predictive values, and area under the ROC. Pearson correlation analysis will be performed to assess the association between CEUS findings for RMP and glomerular filtration rate measured by a radionuclide imaging method. CONCLUSION: The data gathered from this study will be used to evaluate the feasibility of expanding clinical applications of CEUS for evaluation of patients with suspected renovascular hypertension. TRIAL REGISTRATION Chinese Clinical Trial Registry, ChiCTR1800016252; https://www.chictr.org.cn.
Contrast Media ; Glomerular Filtration Rate ; physiology ; Humans ; Hypertension, Renovascular ; physiopathology ; ROC Curve ; Renal Artery ; physiopathology ; Renal Artery Obstruction ; physiopathology

Concurrent involvement of bilateral renal and cerebral arteries, usually incurred as stenosis, is rare in childhood-onset Takayasu arteritis (c-TA). We report the case of a 14-year-old girl, with c-TA, presenting with transient ischemic attack after endovascular revascularization for renal artery stenosis and cerebrovascular stroke after surgical revascularization for cerebral artery stenosis associated with childhood-onset moyamoya syndrome. We deem that decrease of blood pressure by endovascular revascularization and improvement of cerebral perfusion by surgical revascularization may have jeopardized the cerebral deep watershed zone to cerebral ischemia followed by cerebral hyperperfusion syndrome and caused transient ischemic attack and cerebrovascular stroke in our patient. Revascularization could be a double-edge sword for c-TA patients presenting with concomitant renal artery stenosis and cerebral artery stenosis, and should be performed with caution. Quantitative analysis of cerebral blood flow by brain magnetic resonance imaging and angiography should be performed within 48 hours after surgical revascularization in c-TA.
Adolescent* ; Angiography ; Blood Pressure ; Brain ; Brain Ischemia ; Cerebral Arteries* ; Cerebrovascular Circulation ; Constriction, Pathologic* ; Female* ; Humans ; Hypertension, Renovascular ; Ischemic Attack, Transient ; Magnetic Resonance Imaging ; Moyamoya Disease* ; Perfusion ; Renal Artery Obstruction ; Stroke ; Takayasu Arteritis*

Posterior reversible encephalopathy syndrome (PRES, or posterior leukoencephalopahty syndrome) is a neurological condition caused by reversible cortical/subcortical vasogenic brain edema secondary to hypertension, cytotoxic drugs, immunosuppressants, autoimmune diseases, renal disease, eclampsia or pre-eclampsia. It is characterized by acute neurological symptoms such as headache, seizures, visual disturbances, and impaired levels of consciousness. Brain imaging usually reveals bilateral, cortical/subcortical vasogenic edema. Completely unilateral PRES constituted only 2.6% of the cases in a previous study. Here we report the case of a pediatric patient with completely unilateral PRES. A 13-year-old boy was admitted with acute gastroenteritis. On the fourth day of hospitalization, he started to complain of headache and vomiting. He then developed generalized tonic-clonic seizure 3 times. His blood pressure was 180/121 mmHg during the first seizure, 188/112 mmHg during the second seizure and 152/92 mmHg during the third seizure. T2-weighted imaging with fluid attenuation by inversion recovery (T2 FLAIR) demonstrated high-signal intensity in the cortical gyri of the left frontal, parietal, and occipital lobes. Follow-up magnetic resonance imaging (MRI) was performed 2 weeks after the seizure onset, which indicated a significant improvement in the patient's condition. Abdominal pelvic computed tomography (CT) and renal CT angiography showed abnormal narrowing of the left renal artery. In summary, we present a case report of unilateral PRES secondary to renovascular hypertension due to left renal arterial obstruction.
Adolescent ; Angiography ; Autoimmune Diseases ; Blood Pressure ; Brain Edema ; Consciousness ; Eclampsia ; Edema ; Female ; Follow-Up Studies ; Gastroenteritis ; Headache ; Hospitalization ; Humans ; Hypertension ; Hypertension, Renovascular ; Immunosuppressive Agents ; Magnetic Resonance Imaging ; Male ; Neuroimaging ; Occipital Lobe ; Posterior Leukoencephalopathy Syndrome* ; Pre-Eclampsia ; Pregnancy ; Rabeprazole ; Renal Artery Obstruction* ; Renal Artery* ; Seizures ; Vomiting

Pediatric vascular disease is rare, and remains a big challenge to vascular surgeons. In contrast to adults, surgery for pediatric vascular disease is complicated by issues related to small size, future growth, and availability of suitable vascular conduit. During the last 30 years, 131 major vascular operations were performed in a tertiary referral center, Seoul National University Hospital, including aortoiliac aneurysm, acute or chronic arterial occlusion, renovascular hypertension, portal venous hypertension, trauma, tumor invasion to major abdominal vessels, and others. Herein we review on the important pediatric vascular diseases and share our clinical experiences on these rare diseases.
Adult ; Aneurysm ; Aortic Aneurysm ; Child ; Humans ; Hypertension ; Hypertension, Portal ; Hypertension, Renovascular ; Rare Diseases ; Seoul ; Surgeons ; Tertiary Care Centers* ; Thrombectomy ; Transplantation, Autologous ; Vascular Diseases

Moyamoya disease (MMD) is a steno-occlusive disease of the cerebral artery around the circle of Willis. It was first described in 1957 in Japan and named because the characteristic appearance of the basal collaterals in cerebral angiography looks like “a puff of smoke” (moyamoya in Japanese). MMD is one of the major causes of stroke in children worldwide, however most common in Korea, Japan and China. In 2011 the ring finger protein 213 gene (RNF213) was identified as a susceptibility gene for MMD. The RNF213 R4810K variant is an Asian founder mutation common to above nations with carrier rates of 0.5-2% of the general population but a 1/150 penetrance of clinical MMD. MMD patients in Korea and Japan harbors RNF213 R4810K variant in 70-90%. In MMD arterial stenosis was found to occur systematically, not only in the intracranial cerebral arteries but also in renal, coronary, pulmonary arteries, suggesting that MMD is a systemic vasculopathy. These extracranial vasculopathy (ECV) is rare but important as a cause of renovascular hypertension, ischemic heart disease, and pulmonary hypertension especially in children with MMD or family members of MMD. Clinical features of ECV will be reviewed in this article.
Asian Continental Ancestry Group ; Cerebral Angiography ; Cerebral Arteries ; Child ; China ; Circle of Willis ; Constriction, Pathologic ; Coronary Vessels ; Fingers ; Humans ; Hypertension, Pulmonary ; Hypertension, Renovascular ; Japan ; Korea ; Moyamoya Disease* ; Myocardial Ischemia ; Penetrance ; Pulmonary Artery ; Renal Artery ; Stroke

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.
Antihypertensive Agents ; Aorta, Abdominal ; Aortic Coarctation ; Blood Pressure ; Child ; Child, Preschool* ; Constriction, Pathologic ; Diagnosis ; Early Diagnosis ; Humans ; Hypertension ; Hypertension, Malignant* ; Hypertension, Renovascular ; Hypertrophy, Left Ventricular ; Korea ; Lower Extremity ; Male* ; Physical Examination ; Renal Artery Obstruction ; Upper Extremity ; Vascular Diseases