Renovascular hypertension complicated with VATER association

We report herein a case of a girl with renovascular hypertension associated with VATER association. Her plasma renin activity and aldosterone were high. The ultrasonic echogram and renogram revealed a right hypoplastic kidney without function and a normal-sized left kidney with normal function. Renal angiography revealed a small diameter right main renal artery and a normal left main renal artery with segmental stenosis of left branching renal arteries in the middle segment. Selective renal vein sampling indicated that renin secretion was primarily from the left kidney. This is the first report of renovascular hypertension complicated with VATER association.     

The x-ray endovascular treatment of renin-dependent arterial hypertension with secondary aldosteronism in patients without vasorenal involvement

In 20% of cases, essential hypertension is of high renin pathogenesis which is indifferent of that of renal lesions. In these patients high blood pressure is closely related to high aldosterone generation due to elevated angiotensin II levels. Adrenal blood portalization is a possible way of abolishing secondary aldosteronism and hyperreninemia in this case. With this, 90-98% aldosterone and 20-30% renin are inactivated, which served as the basis for bilateral electrocoagulation of adrenal central veins in 13 patients with permanent and malignant arterial hypertension. The renin-dependent pattern of essential hypertension was confirmed by a positive BP response to a tested captopril dose (25 mg), the vasorenal one was ruled out on the basis of the peripheral captopril test, captopril pharmacorenography. Bilateral electrocoagulation of adrenal central veins was performed during a phlebographic examination. The manipulation proved to be successful on 11 (85%) left and 9 (70%) right adrenals. Blood pressure became lower in the first day and stable on days 4-5. There was a significant decrease in blood pressure at a year follow-up, in increased aldosterone levels, plasma renin activity with unchanged adrenocorticotropic hormone concentrations. After the manipulation, 2 patients refused to take antihypertensive drugs, 11 patients received lower doses of drugs. The method for abolishing secondary aldosteronism is considered to be promising for further clinical studies. A special attention should be given to patients with diseases concomitant with essential hypertension who have no alternative to surgical treatment.     

A case of primary aldosteronism due to unilateral multiple adrenal adenomas

A 64-year-old female with hypertension, hypokalemia visited our hospital. Endocrinological examinations showed a low level of plasma renin activity and high level of plasma aldosterone. Circadian rhythmicity of plasma aldosterone level was recognized. No change in the plasma level of aldosterone was observed after loading of standing and administration of furosemide. Adrenal scintigraphy, adrenal venous aldosterone assay and CT scan revealed two tumors in the left adrenal. The diagnosis of primary aldosteronism by left adrenal tumors was made from the above findings. A left adrenalectomy was performed and pathological findings showed two adenomas, which had no capsule either and were surrounded by normal adrenocortical tissue. Blood pressure normalized after surgery and the plasma levels of aldosterone and plasma renin activity were normalized.     

A renin-secreting tumor

A 23-year-old white male was referred for hypertension resistant to triple antihypertensive treatment, with hypokalemia, hyperaldosteronism and elevated levels of circulating plasma renin activity (PRA). Renal angiography and echoscans put in evidence an avascular solid mass at the midlower level of the right kidney. Renal vein catheterization with sampling of blood from the lower branches of the right renal vein showed lateralization of renin secretion from that side. After surgical exeresis, the mass (1.0 cm) was diagnosed as a renal hemangiopericytoma on the basis of light and electron microscopy. Tumor exeresis was followed by a prompt normalization of blood pressure and plasma potassium, with a decrease in PRA and aldosterone. Two months after surgery the patient was still normotensive. Circulating levels of inactive (trypsin-activable) renin were around 60% of the total pool of plasma renin, i.e. much lower than those reported in other cases of renin-secreting tumors. After surgery, inactive and active renin fell in parallel, implying that both were secreted by the tumor. Tumoral PRA responded to postural stimulation, but was unresponsive to acute converting enzyme inhibition, suggesting that sympathetic stimuli were still operative, but the negative feedback inhibition by angiotensin II on renin secretion was lost. Acute converting enzyme inhibition by captopril dropped blood pressure; however, during long-term treatment, the drug (3 X 50 mg/day) was ineffective in terms of either blood pressure normalization or relief of secondary hyperaldosteronism. Acute calcium entry blockade by nifedipine (10 mg p.o.) caused an evident blood pressure drop.     

Analysis of postoperative biochemical values and clinical outcomes after adrenalectomy for primary aldosteronism

Background

Primary aldosteronism causes hypertension and hypokalemia and is often surgically treatable. Diagnosis includes elevated plasma aldosterone, suppressed plasma renin activity, and elevated aldosterone renin ratio. Adrenalectomy improves hypertension and hypokalemia. Postoperative plasma aldosterone and plasma renin activity may be useful in documenting cure or failure.

Sodium and potassium status, plasma renin and aldosterone profiles in normotensive and hypertensive Johannesburg blacks

Many studies have suggested that there is an association between the sodium status, plasma renin and aldosterone profile and essential hypertension. We measured serum, urine and red blood cell (RBC) sodium and potassium, plasma renin and aldosterone levels in normotensive Whites, normotensive Blacks, mildly hypertensive Blacks, severely hypertensive Blacks and Blacks with malignant hypertension. There were no important differences between the groups studied as regarded the serum sodium, serum potassium and urinary sodium excretion values. However, the urinary potassium excretion was significantly lower in normotensive and hypertensive Blacks than in Whites. RBC sodium concentrations showed no significant differences in the mean values across the range of degrees of hypertension in Blacks, although they tended to be higher in the more severely hypertensive groups. Blacks with mild-to-moderate hypertension as well as the severely hypertensive group had significantly lower plasma renin levels than the normotensive group; only in the malignant hypertensives with advanced renal failure did the plasma renin and aldosterone levels rise.     

The effect of renal arterio- and phlebography on the function of the renin-angiotensin and hypophyseal-adrenal systems

Based on the analysis of a RX-ray study and a selective blood test for the activity of the plasma renin, aldesterone, hydrocortisone and adrenocorticotropic hormone (ACTH) in 57 patients with arterial hypertension--14 persons without renal failure, 14 ones regularly treated by hemodialysis, 29 patients with left orthostatic varicocele--the authors demonstrated the impact of the renal arterio- and phlebography on the hormone levels studied. Arteriography resulted in an increase in the absolute value of the renal vein renin mean 2.1-fold, aldosterone, 3.3-fold and hydrocortisone, 1.7-fold. A 2.2-fold increase in the renin activity and a 2.6-fold increase in the levels of aldosterone and hydrocortisone noted in all the patients were the result of retrograde renal phlebography. No correlations were established between the changes in hormone levels and the central mechanism of the secretion regulation (ACTH). Radiopaque investigations of the patients with arterial hypertension gave 22 per cent of false positive results with regard to the site of renin secretion and 18 per cent of those with regard to the participation of the studied kidney in renin secretion. The authors supposed a possible regulation of adrenal mineralocorticoid performance by a retrograde blood flow appeared through the adrenal central veins that was induced by phlebography--related elevation of blood pressure in the renal vein.     

The diagnosis and treatment of arterial hypertension in patients on hemodialysis treatment and after a kidney allograft

Analysis of examination and treatment of 104 patients who were periodically exposed to hemodialysis and 60 persons who sustained the allotransplantation of a cadaveric kidney enabled the authors to reveal the incidence of arterial hypertension before and after the transplantation. Pronounced arterial hypertension (AH) was documented in patients who were treated with hemodialysis sessions for the proper renal diseases developed in angionephrosclerosis (chronic glomerulo- and pyelonephritis). In those who sustained the transplantation, AH was pronounced in case of acute of chronic rejection, transplanted artery stenosis of renal renin hyperproduction. Higher incidence of AH (2.3-fold higher) was observed in posttransplantation patients with the native kidney left. It correlated with higher peripheral plasma renin activity (RRA). The authors suggested that the scheme should be used for the diagnosis of AH manifestation both before and after the transplantation which included the account for the cause of the disease terminal stage, the character of the AH variance in hemodialysis captopril testing, radiocardiographic examination, indirect renal angiography (99-Tc pertechnetate) or selective blood testing for RPA in case bilateral nephrectomy should be made in two stages. For the patients who had sustained the transplantation of the kidney, the diagnostic scheme should include a double pulse Doppler sonography, arteriographic investigation of the transplant and pharmacorenography with a captopril load test. Advisability of captopril stimulation of renin secretion during the selective sampling of the blood was demonstrated. The authors verified the time-course of renin activity, the concentration of aldosterone, cortisol and adrenocorticotrophic hormone in patients with a history of bilateral nephrectomy, defined the indications for bilateral nephrectomy associated with AH and discussed its possible outcomes.(ABSTRACT TRUNCATED AT 250 WORDS)     

Unmasking of primary hyperaldosteronism by renal transplantation

BACKGROUND: Primary hyperaldosteronism is an uncommon cause of hypertension in the general population. Given the mechanism of action of aldosterone clinical manifestations may not occur in the setting of end stage renal disease. However, if a successful renal transplant is performed clinical manifestations may occur. METHODS: We present a case of a patient with a preexisting adrenal adenoma who only presented with clinical signs of hyperaldosteronism after renal transplantation. Patients' work-up included plasma aldosterone, plasma renin activity, serum cortisol, and estimation of trans tubular potassium gradient. RESULTS: The patient's serum aldosterone was markedly elevated with a relatively suppressed plasma renin activity. Trans tubular potassium gradient was high in the presence of hypokalemia. CONCLUSION: Previously silent hyperaldosteronism may be unmasked by a successful renal transplant.     

The aldosterone/renin ratio as a screening test for primary aldosteronism.

OBJECTIVE: To investigate the utility of random outpatient plasma aldosterone and renin measurements in screening for primary aldosteronism (PA) in hypertensive patients. DESIGN: Clinical records of hypertensive patients who had aldosterone and renin determinations over a 3-year period were analysed retrospectively. The patients were arbitrarily classified into five groups defined according to the aldosterone level and the aldosterone/renin (A/R) ratio: PA (plasma aldosterone > 500, A/R ratio > or = 1,000; essential hypertension (EHT) (aldosterone < 1,000, A/R < 1000); secondary hyperaldosteronism (SHA) (aldosterone > or = 1,000, A/R < 400); low-renin hypertension (LRHT) (aldosterone < 500, A/R > or = 1,000); miscellaneous hyperaldosteronism (MHA) (aldosterone > or = 1,000, A/R 400-1000). SETTING: A hypertension clinic in a large teaching hospital. PATIENTS AND RESULTS: The sample of 216 hypertensives were classified as follows: EHT 51%, LRHT 8%, PA 32%, SHA 7%, and MHA 3%. Mean plasma potassium values were significantly lower (P < 0.0001) in the PA group compared with essential hypertensives, but plasma K+ was of little utility in identifying individual patients with PA, as 71% were normokalaemic. Adrenal computed tomography scans were performed on 38 of 69 patients with PA, and revealed definite unilateral or bilateral adrenal masses in 7 and probable unilateral masses in a further 4. Five patients underwent unilateral adrenalectomy, confirming adrenal adenomas in 4 and nodular hyperplasia in 1. The minimum prevalence of PA due to adrenal adenoma or hyperplasia was 3.3% of all hypertensives and 10.1% of patients with biochemical PA defined by A/R ratio. Patients with biochemical PA had more target organ damage, and in general responded more favourably to regimens with spironolactone than to conventional therapy. CONCLUSIONS: A random outpatient aldosterone and renin measurement identifies a group of hypertensives with biochemical PA in which there is a significant prevalence of adrenal adenomas, increased prevalence of target organ damage and a good response to spironolactone. These results have major implications for the investigation and management of patients with severe or resistant hypertension.     

Accelerated hypertension due to intramural dissection of accessory renal artery

A case of sudden, accelerated hypertension due to a spontaneous, idiopathic isolated dissecting aneurysm of an accessory right renal artery is reported. Diagnostic studies including selective angiography, split renal function tests, and plasma renal vein renin levels showed the dissected vessel to be the cause of hypertension.     

Plasma renin activity and plasma aldosterone in acute renal failure

Plasma renin activity and plasma aldosterone were determined by radioimmunoassay methods in 20 patients in oliguric phase, in 11 patients in polyuric phase and in 7 patients in convalescent phase of acute renal failure of various origin. The oliguric phase of acute renal failure was characterized by significant increase of plasma renin activity and plasma aldosterone. There was no direct dependence between them. Direct dependence was found between plasma aldosterone and serum potassium in the oliguric phase of acute renal failure, indirect dependence between plasma aldosterone and serum sodium was found before as well as after haemodialysis. These findings prove a direct influence of hyperkalemia and depletion hyponatremia upon aldosterone secretion in the oliguric phase of acute renal failure. Haemodialysis led to a further increase of plasma renin activity caused by ultrafiltration as well as successive dehydration and application of some drugs. The mean value of plasma aldosterone was not significantly changed after haemodialysis. Plasma renin activity decreased very slowly in the polyuric and convalescence phase of acute renal failure, while plasma aldosterone concentration was already in polyuric phase non-significantly different from the control group. There was no direct dependence in the various phases of acute renal failure between plasma renin activity, plasma aldosterone, systolic and diastolic pressure.The renin-angiotensin-aldosterone system significantly participates in the pathogenesis of acute renal failure in man, but various causes of acute renal failure, different drugs, as well as therapeutic procedures do not make it possible to quantify it in detail.Charcoal haemoperfusion in acute poisonings led only to non-significant increase of plasma renin activity and decrease of plasma aldosterone.     

The diagnosis of renovascular disease

Renovascular disease is an important cause of remediable hypertension in childhood. Specific diagnostic procedures currently available to investigate affected children include Doppler and computed duplex sonography, angiotensin converting enzyme (ACE) inhibitor sensitisation of radionuclide imaging, captopril-stimulated plasma renin activity, hypotensive responses to ACE inhibitor, renal vein renin measurements, renal angiography and magnetic resonance angiography. Carbon dioxide digital subtraction angiography and computerised tomographic and spiral angiography are also available and may play an important future role in such evaluations. Utilising this array of procedures it is usually possible to define the anatomical and functional status of the renal vasculature and be guided towards the most appropriate therapeutic manoeuvres. Received October 14, 1996; received in revised form December 12, 1996; accepted December 16, 1996     

Percutaneous transluminal renal artery dilatation for fibromuscular dysplasia with special reference to the acute effects on the renin-angiotensin-aldosterone system and blood pressure

Twelve hypertensive patients (mean age 46.6 years, range 37-55 years) with fibromuscular dysplasia of the renal artery were treated with percutaneous transluminal renal angioplasty (PTRA) and the effects on the renin-angiotensin-aldosterone system and blood pressure were studied in the acute phase. The technical result of PTRA measured by angiography and reduction of PRA and aldosterone excretion was satisfactory in 11 patients. In spite of this only three patients were cured of their hypertension and two patients were improved at six months follow-up. During PTRA an immediate rise in plasma renin activity was noted in patients without beta-receptor blockade but not in patients treated with beta-receptor blocking agents suggesting a beta-receptor mediated release. This peak in renin release was not accompanied by any rise in systemic blood pressure. The blood pressure response in the acute phase did not show any regular pattern. We conclude that PTRA can serve as a model for studying effects of 'clamping' and 'declamping' of the renal artery in man.     

Plasma renin activity revealed renal artery stenosis concealed by aneurysms

We describe a case of renovascular hypertension with renal artery stenosis concealed by aneurysms. Arteriography demonstrated no apparent renal artery stenosis, but did reveal aneurysms on the left renal artery. Captopril-loaded renoscintigraphy could not detect disturbed renal perfusion. High basal and exaggerated plasma renin activity after captopril administration were the only clues indicating renovascular hypertension. A reduction of the systemic blood pressure and normalized plasma renin activity after resection of the aneurysms confirmed preoperative renovascular hypertension. Fibromuscular dysplasia was an underlying cause of the arterial deformity. In cases of hypertension accompanied by renal artery aneurysms, the captopril-challenge test can be a useful tool to detect renal artery stenosis concealed by the aneurysms.     

肾球旁细胞器瘤的临床分型与诊治探讨

目的 介绍一种新的肾球旁细胞器瘤临床分型方法 ,并对其临床诊治进行探讨. 方法 收集肾球旁细胞器瘤患者5例,结合PubMed搜索到较大的系列病例报道66例,对71例病例资料进行分型.对患者的临床表现、实验室检查和影像学检查进行分析.临床资料包括血压;实验室检查包括血钾、血浆肾素和醛固酮、静脉分段取血检测;影像学检查包括超声、CT、IVU、选择性肾血管造影等. 结果 依据血压和血钾水平,71例肾球旁细胞器瘤可分为3种类型:典型、非典型和静止型.57例典型病例特点:高血压、低血钾、高醛固酮和低肾素;12例非典型病例的特点:高血压、正常血钾;2例静止型病例特点:正常血压和血钾.对于具有高血压、低血钾、高肾素和高醛固酮的患者,应考虑典型肾球旁细胞器瘤的可能;对于高血压合并肾肿瘤的患者,应考虑非典型球旁细胞器瘤的可能;对于单纯肾脏肿瘤,应考虑静止型肾球旁细胞器瘤的可能. 结论 肾球旁细胞器瘤划分为典型、非典型和静止型3类的依据是血压和血钾.肾球旁细胞器瘤代表了一种可以手术纠正的高血压病变,肾肿瘤鉴别诊断中应考虑肾球旁细胞器瘤的可能.保留肾单位手术是首选治疗方法 .     

Renovascular hypertension. Clinical and diagnostic clues.

Renovascular hypertension (RVH), although relatively rare, is the most frequent among the secondary forms of arterial hypertension; in addition interventional radiology has remarkably increased, because of its relative invasiveness, the possibilities of treating and in many cases curing RVH bypassing the traditional surgical approach. For these reasons in recent years a number of screening tests has been developed and added to renal angiography and to the measurement of plasma renin which, still now, represent the reference methods among the morphological and the functional tests respectively. These new and promising techniques include the magnetic resonance angiography, the spiral computed tomography, the renal scintigraphy and the ultrasound scanning of renal arteries with the associated measurement of velocimetric indices. In selected populations all these methods have been shown to possess an high specificity and sensitivity but if applied to a general population of hypertensive patients their positive predictive values are going to be necessarily low because of the low prevalence of the disease. Accordingly, it is mandatory for the physician, before sending patients to these investigations, to preselect those who, on the basis of a thorough clinical examination are more likely to harbour a renal artery stenosis.     

Mesangial proliferative glomerulonephritis with aldosterone-producing adenoma

We describe a case of mesangial proliferative glomerulonephritis (MesPGN), the clinical symptoms of which were exacerbated by aldosterone-producing adenoma (APA). A 45-year-old man, who had had a history of hypertension for several years, presented with renal derangement, with serum creatinine at its upper normal limit and with microhematuria and proteinuria. He also presented with hypokalemia, with a plasma aldosterone concentration at its upper normal limit and plasma renin activity at its lower normal limit. After the administration of spironolactone, we resected his left adrenal gland, which had a nodular lesion as well as aldosterone hypersecretion. The treatment normalized his arterial blood pressure and serum potassium concentration. Although his proteinuria disappeared with the reduction in arterial blood pressure, the microhematuria continued. The administration of losartan because of the histological finding of MesPGN reduced the amount of hematuria. A dissociated response of hematuria and proteinuria to antihypertensive treatment indicated that MesPGN was coincidental with APA.     

Left renal infarction due to dissecting aneurysm of the renal arterial branch

Primary renal artery dissection is a rare condition that causes renal infarction and renovascular hypertension. We describe a case of a 48-year-old man who was admitted for left renal infarction due to a primary dissecting aneurysm of renal artery. He was diagnosed by a contrast enhanced abdominal CT scan and a renal angiography demonstrating a dissection at the branch of the left renal artery. He was treated with conservative medical management, including anticoagulation therapy. Forty-five primary renal artery dissections in 38 patients from previous reports were analyzed. Based on this analysis, many cases of primary renal artery dissection were male and had a high blood pressure. Primary renal artery dissection occurred at the right side (12 cases), left side (19), and bilaterally (7). The renal arterial branches were rarely involved in primary renal artery dissection. Assays of peripheral blood renin activity were performed in 15 patients, and all cases had a high value. Therefore, the present case is thought to be unique because he was a normotensive patient with normal plasma renin activity and the renal arterial branch was only dissected.     

Juxtaglomerular cell tumor of the kidney—a new classification scheme

ObjectiveTo introduce a new classification scheme of juxtaglomerular cell tumor (JCT) of the kidney for differential diagnosis of hypertension and renal cell carcinoma.MethodsFive cases of JCT have been diagnosed and treated surgically in our hospital during the last 4 years. Through a search in PubMed, we incorporated 7 large series of case reports of JCT into a review of 71 cases previously published in the literature. Clinical presentations (blood pressure), laboratory examinations [serum potassium, plasma renin activity (PRA), aldosterone (ALD), and renal venous sampling for renin assay], and imaging examinations [ultrasonography, computerized tomography (CT), excretory urography, and selective renal angiography] were summarized.ResultsThe 71 cases of JCTs can be classified into 3 types, which are typical type, atypical type, and non-functioning type. The 57 typical cases had the typical characteristics of hypertension, hypokalemia, hyperaldosteronism, and high renin. The 12 atypical cases had hypertension with normal potassium levels, and the 2 non-functioning cases had normal blood pressure and normal potassium levels.ConclusionsThe classification of typical, atypical, and non-functioning JCTs depends on blood pressure and serum potassium. JCT of the kidney should be considered in patients with hypertension and renal tumor, and nephron-sparing surgery is the first choice.