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

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

No abstract available.
Adult ; Angiography, Digital Subtraction ; Angioplasty, Balloon ; *Blood Pressure ; Female ; Fibromuscular Dysplasia/*complications/diagnosis ; Humans ; Hypertension, Renovascular/diagnosis/*etiology/physiopathology/therapy ; Renal Artery Obstruction/diagnosis/*etiology/physiopathology/therapy ; Treatment Outcome

OBJECTIVETo characterize the clinical and angiographic features in children with renovascular hypertension.

METHODClinical data of 14 children (7 male, 7 female; age 0.8-14 years, mean 8.7 years), who were diagnosed with renovascular hypertension by renal angiography in our institute from January 2005 to December 2012 were collected and retrospectively analyzed.

RESULTThe mean blood pressure at the diagnosis was 187/127 mm Hg. Chief complaints of symptomatic patients were headache (29%, 4/14), hypertensive encephalopathy (36%, 5/14), signs of congestive heart failure (14%, 2/14) and hematemesis (7%, 1/14). Renovascular hypertension was found incidentally in 14% (2/14) of patients who were asymptomatic. Conventional renal angiography elucidated the anatomical distribution of lesions in the renal arterial system. It was found that 14% (2/14) of patients had bilateral disease, 50% (7/14) had single stenosis at main or accessory renal artery, while multiple stenoses was seen in 43% (6/14) of children, with involvement of segmental renal artery and small interlobar or arcuate vessels. Compared with catheter angiography, 50% (7/14) of patients with renovascular hypertension, especially intrarenal arterial disease, were missed on computed tomography angiography or magnetic resonance angiography.

CONCLUSIONIt is mandatory to emphasize blood pressure measurement in pediatric clinical practice for early recognition of renovascular hypertension. As children with renovascular hypertension display involvement of multiple arteries, including in smaller intrarenal arteries, digital subtraction angiography is the only method that can reliably diagnose pediatric renovascular hypertension.

Adolescent ; Angiography, Digital Subtraction ; Child ; Child, Preschool ; Female ; Fibromuscular Dysplasia ; diagnosis ; diagnostic imaging ; Humans ; Hypertension, Renovascular ; diagnosis ; diagnostic imaging ; Infant ; Kidney ; diagnostic imaging ; pathology ; Magnetic Resonance Angiography ; Male ; Renal Artery ; diagnostic imaging ; pathology ; Renal Artery Obstruction ; diagnosis ; diagnostic imaging ; Retrospective Studies ; Sensitivity and Specificity ; Tomography, X-Ray Computed

OBJECTIVE: It has been advocated that a reduced injection volume with highly concentrated (1 M) contrast material can produce a sharper bolus peak and an increased intravascular first-pass gadolinium concentration when compared with the use of a lower concentration (0.5 M). A higher concentration would also cause a reduction in dose. The purpose of our study was to test the use of a low dose (0.05 mmol/kg) of gadobutrol in magnetic resonance renography and angiography and compare the findings with a dose of 0.1 mmol/kg. MATERIALS AND METHODS: One-hundred-thirty-four patients referred for magnetic resonance angiography for suspected renovascular disease participated in the study. Contrast enhanced MR renography and angiography were performed after administration of a bolus of 0.1 mmol/kg or 0.05 mmol/kg gadobutrol in randomized patients. The relative signal intensity-time curves of the aorta, peripheral cortex and parenchyma, were obtained. Two radiologists evaluated the angiographic images and evaluated the quality of angiography. RESULTS: The signal intensity with a low dose of gadobutrol was significantly lower in early phases, in the peripheral cortex (for 36, 54, 72 and 90 seconds), the parenchyma (for 36, 54, 72 seconds) and the aorta (for 18, 36, 54, 72 seconds). The decreases in the early phase obtained with a low dose of gadobutrol caused blunter time intensity curves. The difference in the quality scores of the readers for the angiographic images for the use of the two different doses was not statistically significant (p > 0.05). CONCLUSION: A lower dose of gadobutrol can be used for MR renal angiography, but for MR renography the normal dose should be used.
Contrast Media/*administration & dosage ; Female ; Humans ; Hypertension, Renovascular/*diagnosis ; Magnetic Resonance Angiography/*methods ; Magnetic Resonance Imaging/*methods ; Male ; Middle Aged ; Organometallic Compounds/*administration & dosage

OBJECTIVETo increase the probability of successful renal artery angiography with a three-dimensional contrast-enhanced magnetic resonance angiography (3D CE-MRA).

METHODS3D CE-MRA was performed in 33 patients with hypertension. To calculate the scan delay time, a testing contrast agent bolus of 2 ml was injected for single sagittal slice real-time scanning, followed by oblique coronal 3D Fast TOF SPGRE. With maximum intensity projection, a set of images was acquired.

RESULTSExact scan delay time was obtained in all the cases from which clear images were acquired with the exception of two cases in which failure of breath-holding occurred during the scanning.

CONCLUSIONThis examination enhances the performance by adopting elliptical-centric k-space recording for data acquisition for the center in k-space at the beginning of acquisition, and utilizing preceding testing bolus injection and signal intensity/time curve for determining the scan delay time, using slice selection and multiview image reconstruction.

Adolescent ; Adult ; Aged ; Contrast Media ; Female ; Humans ; Hypertension, Renovascular ; diagnosis ; Image Enhancement ; methods ; Imaging, Three-Dimensional ; methods ; Magnetic Resonance Angiography ; methods ; Male ; Middle Aged ; Renal Artery

Primary reninism is a rare cause of hypertension manifesting along with hypokalemia. A high level of plasma renin activity and a high level of serum aldosterone are the whole markers of primary reninism. Upon making the diagnosis of primary reninism, other more common causes of aldosteronism must be differentiated, such as renovascular hypertension and primary aldosteronism. Primary reninism is commonly caused by juxtaglomerular cell tumor, which is one of the curable causes of hypertension, and this can be successfully treated by conservative surgery. We report here on a case of primary reninism that was caused by juxtaglomerular cell tumor that developed in a 22-year-old female patient. She was recently diagnosed with hypertension and hypokalemia. She had markedly elevated plasma renin activity and an increased serum aldosterone concentration. Computed tomography revealed a mass located in the right kidney and selective renal vein sampling suggested that the mass was secreting an excess of renin. Right nephrectomy was done and her hypertension with hypokalemia was successfully treated. We report here a case of primary reninism that presented with juxtaglomerular cell tumor along with a review of the literature
Aldosterone ; Diagnosis ; Female ; Humans ; Hyperaldosteronism ; Hypertension* ; Hypertension, Renovascular ; Hypokalemia* ; Kidney ; Nephrectomy ; Plasma ; Renal Veins ; Renin* ; Young Adult

Neurofibromatosis(NF) is an autosomal dominant disorder, probably of neural crest origin that affects all three germinal layers. It is a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Type 1(von Recklinghausen disease) is the most common neurocutaneous disorder among the eight subtypes. Previous reports showed various involvements in the renal organ. Renovascular hypertension is the most common major manifestation of renal involvement in this disease. However, we experienced a case of ectopic kidney concurrent with neurofibromatosis type 1. The diagnosis of neurofibromatosis had been made by typical skin manifestation on physical examination, and ectopic kidney was discovered accidentally during routine abdominal sonography. The etiological basis of this association is not clear. We report a rare case of coexisting neurofibromatosis and ectopic kidney in a 7-year-old girl with a brief review.
Child ; Diagnosis ; Female ; Humans ; Hypertension, Renovascular ; Kidney* ; Neural Crest ; Neural Plate ; Neurocutaneous Syndromes ; Neurofibromatoses* ; Neurofibromatosis 1 ; Physical Examination ; Skin Manifestations

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 +/- 18 years) with unilateral renal artery stenosis who underwent renal artery revascularization and captopril renal scintigraphy with 99mTc-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: Eight 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 < or= 38) ; 92% and 50% (BCfun > or= 1%) ; 92% and 75% (CBren > or= 1), and 90% and 60% (CNren > or= 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.
Arteritis ; Blood Pressure ; Captopril* ; Constriction, Pathologic ; Diagnosis* ; Fibromuscular Dysplasia ; Humans ; Hypertension ; Hypertension, Renovascular* ; Kidney ; Radionuclide Imaging ; Renal Artery Obstruction* ; Renal Artery* ; ROC Curve

BACKGROUND: Renovascular hypertension has variable etiologic diseases and therapeutic outcomes. We performed a retrospective analysis of the causes and treatment results of renovascular hypertension to elucidate long-term prognosis with respect to blood pressure and renal function. METHODS: We reviewed patients who were admitted to Seoul National University Hospital for evaluation of renovascular hypertension in period from January, 1983 to December, 2002. Diagnosis of renovascular hypertension was made by combination of positive functional studies such as captopril renal scintigraphy and/or captopril test and angiographic demonstration of significant stenoses in one or both of renal arteries. Patients who were followed up for more than six months were included and classified according to the etiologic disease and treatment modalities. Responses of blood pressure and renal function in each patient were periodically evaluated. RESULTS: Of 74 patients included, 37 were male and 37 were female. The median age of the patients was 40. Atherosclerotic renal artery stenosis was the most common diagnosis (31, 41.9%), followed by Takayasu's arteritis (21, 28.4%) and fibromuscular dysplasia (14, 18.9%). Control of blood pressure and preservation of renal function were significant in medical, radiological, and surgical therapy group. There was no significant difference in response of blood pressure and renal function to treatment among treatment groups. Poor response of blood pressure and deterioration of renal function were observed more frequently in patients with atherosclerotic renal artery stenosis than in those with Takayasu's arteritis or fibromuscular dysplasia. CONCLUSION: Atherosclerosis was the most common etiologic diagnosis of renovascular hypertension. Medical treatment showed satisfactory treatment results as compared to radiologic and surgical revascularization. Patients with atherosclerotic renal artery stenosis showed poorer control of blood pressure and preservation of renal function.
Atherosclerosis ; Blood Pressure ; Captopril ; Constriction, Pathologic ; Diagnosis ; Female ; Fibromuscular Dysplasia ; Humans ; Hypertension, Renovascular* ; Male ; Prognosis ; Radionuclide Imaging ; Renal Artery ; Renal Artery Obstruction ; Retrospective Studies ; Seoul ; Takayasu Arteritis