• Childhood Kidney Diseases
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• v.17 no.2
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• pp.143-148
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• 2013

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.

• The Korean Journal of Nuclear Medicine
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• v.12 no.1
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• pp.9-16
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• 1978

It has been suggested that plasma renin activity (PRA) and its response to volume depletion may be abnormal in that it shows little or exaggerated change in patients with chronic renal failure and hypertension. Intravenous furosemide stimulation test was performed in 46 control subjects and 51 patients with chronic renal failure and/or malignant hypertension in order to evaluate PRA response. In contrast to the consistent increase in PRA in control subjects (from $2.5{\pm}1.95\;to\;4.5{\pm}2.51ng/ml/hr$), no consistent increase was observed in patients with chronic renal failure, especially in those who showed favorable response to antihypertensive therapy (from $2.5{\pm}2.21\;to\;2.9{\pm}2.46ng/ml/hr$). But poor responder to antihypertensive treatment showed considerably higher PRA before and after furosemide stimulation (from $4.9{\pm}1.96\;to\;6.4{\pm}1.71ng/ml/hr$) than the responder group did. Moreover, this group seemed to retain the ability to increase PRA in response to intravenous furosemide stimulation. Thus it became apparent that responder group was unable to increase PRA normally in response to furosemide as well as volume depletion, while poor responder seemed to retain that ability. Thus intravenous furosemode may serve as a convenient way to differenfiate those who might be benefited by conservative antihypertensive measures from those who would require more drastic measures such as bilateral nephrectomy for their optimal blood pressure control.

• Journal of Yeungnam Medical Science
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• v.32 no.2
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• pp.143-145
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• 2015

Page kidney refers to the phenomenon of hypertension secondary to long-standing compression of renal parenchyma caused by renal subcapsular collection. The most common cause of renal subcapsular collection is a hematoma which usually occurs after a history of blunt trauma. A 42-year-old female patient who received botulinum toxin injection in her back during chiropractic care was admitted to the emergency room with sudden bilateral flank pain and hypertension. The computed tomography (CT) images demonstrated the presence of bilateral subcapsular renal hematoma. The patient was treated conservatively and recovered well. The follow up CT images showed markedly resolved bilateral hematoma.

• Clinical and Experimental Pediatrics
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• v.62 no.4
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• pp.144-147
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• 2019

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.

• Childhood Kidney Diseases
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• v.20 no.1
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• pp.33-36
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• 2016

There are two pathogenic models of renovascular hypertension (RVH) originating from renal artery stenosis. We noted a case of a boy who had severe hypertension with atrophic left kidney, hypertrophic right kidney, a segmental stricture of the abdominal aorta, and total occlusion of the right renal artery. Due to the atrophic change of the contralateral, unclipped left kidney, this patient presented with various clinical manifestations related to both models of pathogenesis of RVH occurring at the same time. We conclude that this patient presented with the middle stage of the two RVH pathogenetic models, so called the 'one-clip, one and half-kidney model.'

• 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.

• Childhood Kidney Diseases
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• v.27 no.2
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• pp.121-126
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• 2023

The administration of angiotensin type 2 receptor blockers (ARBs) during pregnancy is known to cause ARB fetopathy, including renal insufficiency. We aimed to analyze the outcomes of two patients who survived ARB fetopathy and perform an accompanying literature review. Case 1 was exposed antenatally from a gestational age of 30 weeks to valsartan because of maternal pregnancy-induced hypertension. The patient presented with oliguria immediately after birth, and renal replacement therapy was administered for 24 days. Seven years after birth, renal function was indicative of stage 2 chronic kidney disease (CKD) with impaired urinary concentration. Case 2 had a maternal history of hypertension and transient ischemic attack and was treated with olmesartan until 30 weeks of pregnancy. Renal replacement therapy was performed for 4 days since birth. After 8 years, the patient is with CKD stage 2, with intact tubular function. Recent reports suggest that ARB fetopathy might manifest as renal tubular dysgenesis and nephrogenic diabetes insipidus, in contrast to mild alterations of glomerular filtration. Tubular dysfunction may induce CKD progression and growth retardation. Patients with ARB fetopathy should be monitored until adulthood. The ARB exposure period might be a critical factor in determining the severity and manifestations of fetopathy.

• Journal of Physiology & Pathology in Korean Medicine
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• v.18 no.1
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• pp.84-92
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• 2004

The present study examined the effects of Taekunyukmijiwhang-tang (TV) on blood pressure and renal function in nitric oxide (NO)-dependent hypertensive rats. A phamacological inhibition of nitric oxide synthase (NOS) for 4-6 weeks produces renal vasoconstriction, renal dysfunction, and progressive severe hypertension. Treatment of rats with NG-Nitro-L-arginie methylester (L-NAME) (100 mg/L, 6 weeks), which is a nonspecific NOS inhibitor, cause a sustained increase in systolic blood pressure (SBP), along with the decrease in expression of ecNOS in the kidney and thoracic aorta. The expression of Na, K-ATPase α1 subunit in the kidney was also reduced in the L-NAME induced hypertensive rats group. The renal functional parameters including urine osmolality (Uosm), creatinine clearance (Ccr), which is an index of glomerular filtration (GFR) were decreased in rat with L-NAME induced hypertension. while solute-free water reabsoption (TcH₂O) was unchanged in all experimental group. However, the group combined treated with TV and L-NAME did not develop hypertension and expression of ecNOS in the aorta was restored. The expression of Na/sup +/, K/sup +/-ATpase α1 subunit in the kidney was markedly restored in L-NAME-induced hypertension rats by administration of TV along with the restoration of urinary volume (UV) and sodium excretion (UNaV), whlie Na/sup +/, K/sup +/-ATPase /β1 subunit was not altered. These results suggest that TV attenuates an increase in SSP in the L-NAME induced hypertension and restores partially renal function, which seems to be caused by up-regulation of expression of Na/sup +/, K/sup +/-ATPase α1 subunit in the kidney and ecNOS in thoracic aorta.

• The Korean Journal of Nuclear Medicine
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• v.24 no.2
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• pp.325-331
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• 1990

Radionuclide renal scintigraphies were performed in a 24-year-old man with right renal artery branch stenosis. Captopril enhanced $^{99m}Tc-DTPA$ renal scintigraphy revealed no abnormal finding in the right kidney. But, $^{99m}Tc-DMSA$ renal scintigraphy showed regional cortical photon deficient area corresponding to the area supplied by the stenotic branch artery. The defect size increased in captopril enhanced $^{99m}Tc-DMSA$ renal scintigrphy and nearly disappeared after successful transluminal renal angioplasty. This case suggests that the captopril enhanced $^{99m}Tc-DMSA$ renal scintigraphy may be a useful method in the evaluation of renovascular hypertension, especially due to branch renal srtery stenosis.

• Clinical and Experimental Pediatrics
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• v.55 no.10
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• pp.367-370
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• 2012

Urinary tract infection (UTI) is a common bacterial illness in children. Acute pyelonephritis in children may lead to renal scarring with the risk of later hypertension, preeclampsia during pregnancy, proteinuria, and renal insufficiency. Until now, vesicoureteral reflux (VUR) has been considered the most important risk factor for post-UTI renal scar formation in children. VUR predisposes children with UTI to pyelonephritis, and both are associated with renal scarring. However, reflux nephropathy is not always acquired; rather, it reflects reflux-associated congenital dysplastic kidneys. The viewpoint that chronic kidney disease results from renal maldevelopment-associated VUR has led to questioning the utility of any regimen directed at identifying or treating VUR. Despite the recognition that underlying renal anomalies may be the cause of renal scarring that was previously attributed to infection, the prevention of renal scarring remains the goal of all therapies for childhood UTI. Therefore, children at high risk of renal scar formation after UTI should be treated and investigated until a large clinical study and basic research give us more information.