• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.84-87
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• 2016

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.

• The Korean Journal of Physiology and Pharmacology
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• v.4 no.2
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• pp.143-147
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• 2000

The present study was aimed at investigating whether there are changes in the expression of nitric oxide synthase (NOS) in relation with the unclipping-induced fall of blood pressure in two-kidney, one clip (2K1C) hypertension. Male Sprague-Dawley rats were made 2K1C hypertensive by clipping the left renal artery for four weeks. Sham-clipped rats served as control. The expression of endothelial constitutive (ec) NOS proteins and tissue levels of NO metabolites were determined in the kidney. Systolic blood pressure was significantly increased in clipped rats compared with that in the control. The development of hypertension was associated with decreases in the expression of ecNOS proteins and tissue levels of NO metabolites in the clipped kidney. The blood pressure at twenty-four hours after removal of the renal arterial clip fell to the control level. Accordingly, in the unclipped kidney, the expression of ecNOS proteins and tissue contents of NO metabolites were increased to the control level. The contralateral kidney was not affected by the development or reversal of hypertension. It is suggested that an enhanced expression of ecNOS in the unclipped kidney is an important component in the reversal of renovascular hypertension.

• Journal of Chest Surgery
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• v.20 no.1
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• pp.171-176
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• 1987

Takayasu`s arteritis is one of chronic inflammatory disease characteristically involving the aorta and its major branches. Symptoms and signs of the disease are various depending on the involved area. We experienced a surgical case of Takayasu`s arteritis mainly involving both renal arteries with renovascular hypertension in a 13 year old girl. Hypertension was not controlled by medical treatment including diuretics and captopril [160/140 mmHg]. Aortogram showed severely stenosed right renal artery, nearly obstructed left renal artery and not visulalized superior mesenteric artery. Angioplasty was performed for the right renal artery but aorta-renal bypass graft with greater saphenous vein was inevitable for the left renal artery. Blood pressure was controlled sufficiently with some adjunct of captopril postoperatively [130/90 mmHg]. While the patient was discharged with much improvement, she was lost follow up and died of not identified definitive cause 3 months later.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.51-55
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• 2014

Primary renal artery aneurysm has been estimated to account for an incidence of 0.015-1% with associated morbidities including renovascular hypertension and rupture. Renovascular hypertension associated renal artery aneurysms in children is not a common disease. In patients with complicated renal vascular disease, renal autotransplantation has been used as an alternative to percutaneous transluminal angioplasty, which may be hazardous in these situations. We report a case of a renal artery aneurysm in a 13-year-old Korean child presenting hypertension detected during school health examination. Preoperative workup demonstrated a $2.8{\times}2.1{\times}1.9$ cm saccular aneurysm in the right renal hilum that was not amendable to endovascular repair. A surgical strategy including extracorporeal renal artery reconstruction with autotransplantation was applied in order to restore renal artery anatomy and to treat renovascular hypertension. Immediately he complained of severe right flank pain and postoperative doppler sonography revealed lack of perfusion. On the 5th day after autotransplantation, the patient underwent a transplant nephrectomy. He was well postoperatively and was found to have a normal kidney function and stable blood pressure control without antihypertensive medication. This is the first pediatric case of renal artery aneurysm in Korea who underwent extracorporeal repair followed by autotransplantation failure. More pediatric cases with renal artery aneurysm should be reported to identify therapeutic outcome and long term prognosis.

• Journal of Physiology & Pathology in Korean Medicine
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• v.30 no.2
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• pp.95-100
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• 2016

The aim of the present study is to investigate the hypotensive effect of Mantidis ootheca (WMO), Rosa laevigata (WIC), and Imperata cylindrica (WRL) in renovascular hypertension rats. Experimental hypertension model is 2-kidney and 1-clip (2K1C) induced rats. 2K1C rats were treated with WMO, WIC, and WRL at dose of 100 mg/kg/day orally for 3 weeks, respectively. Treatment groups with WMO, WIC, and WRL significantly lowered blood pressure. Interestingly, WMO, WIC, and WRL ameliorated endothelium-dependent and independent vascular relaxation in the phenylephrine-precontracted thoracic aorta in hypertension models. In addition, 2K1C-induced hypertension model increased plasma renin activity, however, WMO, WIC, and WRL attenuated those activities. These results suggest that WMO, WIC, and WRL ameliorates vascular dysfunction in 2K1C-induced hypertension models via the regulation of nitric oxide and renin-angiotensin-aldosterone system.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.223-229
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• 2010

Percutaneous transluminal renal angioplasty (PTRA) is the current treatment of choice for renal artery revascularization, but renal autotransplantation has been an alternative treatment for complex cases. Here we report a 14-year-old boy with severe hypertension successfully treated with PTRA and renal autotransplantation. Doppler ultrasonography and computed tomography (CT) angiography revealed slight narrowing in the right renal artery ostium and complete obstruction in the left renal artery ostium with multiple collaterals. PTRA with stent insertion was performed for the treatment of the right renal artery, but it was impossible for the left renal artery due to the total obstruction. Therefore, left nephrectomy for autotransplantation was done with the peritoneal approach and the left kidney was autotransplanted to the ipsilateral iliac fossa. Postoperatively, Doppler ultrasonography and mercapto-acetyl-triglycine (MAG-3) renogram were performed, which showed normal renal artery blood flow and kidney function. Blood pressure was normalized and anti-hypertensive drugs were gradually tapered. Fibromuscular dysplasia was suspected to be responsible for the renal artery stenosis based on clinical aspects. In conclusion, renal autotransplantation is also a good treatment option for children with severe renovascular hypertension when endovascular treatment has failed or is not possible.

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.142-146
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• 2021

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

• Journal of Chest Surgery
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• v.25 no.2
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• pp.200-204
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• 1992

A 63 year old male had suffered from hypertension and angina pectoris for 4 years, On physical examination, blood pressure was 150/110 mmHg with medication of antihypertensive drugs. Aortogram showed the stenosis of the left renal artery, the complete occlusion of the right renal artery, and atherosclerotic change of abdominal aorta. Blood urea nitrogen was 25 mg/dl, serum creatinine was 1.2 mg/dl, and renin activity in peripheral blood was 8.7 ng /ml /hour, The stenosis of left renal artery and the complete occlusion of right renal artery should have produced the renovascular hypertension Bilateral aorto-renal bypasses with saphenous grafts were done for treatment of ren-ovascular hypertension Postoperatively, blood pressure was normalized with only small dosage of antihypertensive drugs.

• The Korean Journal of Nuclear Medicine
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• v.38 no.6
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• pp.498-505
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• 2004

Purpose: We compared captopril renal scintigraphic criteria for the diagnosis of renovascular hypertension by unilateral renal artery stenosis. Materials and Methods: The study group consisted of 24 patients (m/f : 16/8, age: $39{\pm}18$ years) with unilateral renal artery stenosis who underwent renal artery revascularization and captopril renal scintigraphy with $^{99m}Tc$-diethylenetriaminepentaacetic acid between May 1995 and April 2004. The blood pressure response was classified as cure/improvement or failure. We evaluated captopril-induced changes in relative function (BCfun) and renogram grade (0 to 5: 0=normal, and 5=renal failure pattern without measurable uptake) (CBren) and the difference of renograms between the normal and stenotic kidney on captopril scan (CNren). Results: light of 24 patients were cured and 11 improved and 5 patients were classified as failed revascularization. Significant predictors of a cure or improvement of blood pressure were younger age, stenosis by fibromuscular dysplasia or arteritis, BCfun, CBren and CNren. Areas under the receiver operating characteristic curve of age, BCfun, CBren and CNren were not significantly different. Positive and negative predictive values of predictors were 100% and 42% (age ${\leq}38$): 92% and 50% (BCfun ${\geq}1%$): 92% and 75% (CBren ${\geq}1$), and 90% and 60% (CNren ${\geq}1$), respectively. Conclusion: Captopril induced changes in renal function and renogram can reliably predict hypertension response to revascularization. Renogram pattern on captopril scan can diagnose renovascular hypertension without baseline data in patients with unilateral renal artery stenosis.

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.109-114
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• 1994

We studied the incidence and results of treatment of secondary hypertension in Yeungnam University Hospital. Ntneteen hundred fifty patients with hypertension were included from march 1990 to march 1994. We analysed the prevalence of secondary hypertension and results of treatment. The incidence of secondary hypertension in Yeungnam University Hospital was six percent. The most common underlying causes of secondary hypertension was renal parenchymal disease. Patients with three forms of potentially reversible secondary hypertension, namely, renovascular hypertension, endocrine disease exogenous hormone, were assed to determine whether surgery or withdrawal of the exogenous hormone had led to an improvement in blood pressure control. The incidence of secondary hypertension in Yeungnam University Hospital was low(6%), but some of these are curable. Thus it is very inportant that evaluate the secondary hypertension.