A case of renovascular hypertension with nephrotic syndrome, accompanied by focal segmental glomerulosclerosis-like lesion in the contralateral kidney]

A 66-year-old male patient of renovascular hypertension with nephrotic syndrome was described. Besides, focal segmental glomerulosclerosis like lesion was accompanied in the contralateral kidney. On admission, his blood-pressure amounted to 220/140 mmHg. Laboratory investigation included; urinary protein 10.5 g/day, serum creatinine 1.8 mg/dl, creatinine clearance 71.4/day, plasma renin activity 10.0 ng/ml/hr, angiotensin I 890 pg/ml and angiotensin II 40.0 pg/ml. Renogram and renal scintigram showed non-functioning pattern of left kidney. Arteriography disclosed approximately more than 95% stenosis of left main artery. Administration was discontinued because of poor control both in blood pressure and in proteinuria. After the left nephrectomy, the former normalized and the latter decreased. Microscopic examination of the right kidney revealed focal segmental glomerulosclerosis like lesion. So far as we know, this report is the first designed to elucidate renovascular hypertension with nephrotic syndrome accompanied by focal segmental glomerulosclerosis like lesion. The relationship between renovascular hypertension and nephrotic syndrome, and microscopic findings has been briefly discussed. It is suggested that the etiology of focal segmental glomerulosclerosis like lesion may be based on compensatory glomerular hyperfiltration caused by renovascular hypertension.     

A case of percutaneous transluminal angioplasty of renovascular hypertension in a solitary kidney

The patient was a 18-year-old woman. Past history included right nephrectomy for right congenital hydronephrosis when she was an infant of 40 days. On examination, her blood pressure was 220/140 mmHg, and plasma renin activity was 4.2 ng/ml/hr. The selective renal arteriogram showed fibromuscular dysplasia of the left main renal artery, and a diagnosis of renovascular hypertension in the solitary kidney with an aberrant artery was made. Treatment with orally-active inhibitor of angiotensin I converting enzyme, Captopril, was started. Her blood pressure became normal after oral administration of Captopril, but her renal function deteriorated. Therefore, percutaneous transluminal angioplasty was performed twice resulting in effective dilatation of the stenotic portion of the left main renal artery. Thereafter, her blood pressure fluctuated between 170/120 and 140/70 mmHg. Eight months later, her blood pressure is now being controlled with mild antihypertensive treatment.     

MRI detection of atrophic kidney in a hypertensive child with a single kidney

The role of magnetic resonance imaging (MRI) in the work-up of secondary causes of pediatric hypertension is typically restricted to that of renovascular causes where main renal artery stenosis is suspected. We report a case of a 10-year-old female child with hypertension, who was thought to have unilateral renal agenesis, because only a solitary left kidney could be visualized on both ultrasound and renal scintigraphy. Our patient underwent magnetic resonance imaging because of suspected renal artery stenosis in her solitary left kidney. At MRI she was found to have a normal left kidney. However, a very tiny, atrophic right kidney was also visualized. A laparoscopic right nephrectomy was performed, which resulted in complete resolution of her hypertension. This case illustrates a possible additional role for MRI in a very small subset of pediatric hypertensive patients: those with a single kidney on ultrasound.     

Paradoxical response of blood pressure to salt loading in renovascular hypertension

We studied the effect of high salt intake on blood pressure in two cases with renovascular hypertension. They had hypertension with hyperreninemia and marked difference in plasma renin activity between both renal veins. Blood pressure significantly decreased after single oral administration of captopril. Renal arteriogram revealed significant stenosis in the main artery to the left (case 1) and right (case 2) kidney. Blood pressure response was evaluated after seven (case 1) and five (case 2) days of low salt and seven days (both cases) of high salt intake. Mean blood pressure in two patients was significantly decreased (case 1; 118 +/- 5.5 to 108 +/- 6.1 mmHg and case 2; 150 +/- 3.8 to 138 +/- 3.1 mmHg). Plasma renin activity was also decreased (case 1; 6.25 to 0.77 ng/ml/hr and case 2; 22.8 to 6.3 ng/ml/hr). In case 2, blood pressure elevated markedly during low salt intake, compared with blood pressure level during normal salt intake. The results suggest that excessive salt intake in patients with unilateral renovascular hypertension produces blood pressure reduction because of suppression of renin-angiotensin system. We concluded that in patients with unilateral renovascular hypertension dietary sodium depletion may be harmful, whereas salt supplement may have a beneficial effect.     

Renal-portal shunt ameliorates renovascular hypertension in pigs

BACKGROUND: Renovascular hypertension is the most common curable form of secondary hypertension. Renin angiotensin system activation depends on the balance between renin production by the kidney and renin degradation by the liver. Thus, we aimed to examine whether deviation of renin-rich blood from the affected kidney into the portal circulation (portalization) can ameliorate renovascular hypertension. METHODS: We selected a porcine model of unilateral renal artery stenosis because the pig's anatomy and physiology are comparable to those of humans and because pigs have already been found capable of developing chronic renovascular hypertension. Angiography and ultrasonography were deliberately used to evaluate renal artery stenosis and the renal-portal shunt. Histology was used to examine the effects of portalization on the kidney and liver after a period of two months. RESULTS: As expected, following the creation of a left renal artery stenosis both renin activity and mean blood pressure measurements increased from 1.23 +/- 0.06 ng/mL/h and 85.6 +/- 0.5 mm Hg at baseline to 4.59 +/- 0.02 ng/mL/h and 126 +/- 1.76 mm Hg, respectively. After portalization renin activity returned to the normal range (1.59 +/- 0.07 ng/mL/h) followed by a concomitant reduction of mean blood pressure to 91 +/- 2 mm Hg. Moreover, a significant correlation was observed between changes in renin activity and blood pressure measurements during the two stages of the experiment. Both the kidney and liver remained macroscopically and microscopically intact at the end of the experiment. CONCLUSION: Portalization of the affected kidney can ameliorate renovascular hypertension and therefore, it might be of benefit in those individuals with fibromascular or atheromatous lesions in the renal artery or its branches not amenable to balloon angioplasty or surgical revascularization.     

Clinical and experimental study on vascular reconstruction for one-kidney renovascular hypertension

Several special problems were noted regarding the vascular reconstruction for renovascular hypertension of the patient with a solitary kidney. It is difficult to use the value of plasma renin activity for decisive diagnosis or determination of surgical indications because most of the one-kidney patients with renovascular hypertension showed a normal plasma renin activity preoperatively and it is theoretically impossible to obtain a ratio of the affected to the opposite renal vein renin level. Most patients presented moderate to severe degree of renal dysfunction so that vascular reconstruction should be the treatment of choice because the conservative therapy with anti-hypertensive drugs such as captopril may further worsen the renal function by decreasing the renal perfusion pressure. Patients showed extensive polyuria immediately after surgery which was attributed to sudden increases in glomerular filtration rate and urinary sodium excretion. There was no correlation between the preoperative serum osmolarity and the postoperative polyuria. Correlation was not obtained between the intraoperative clamping time of the renal artery and the aggravation of the previously existing renal dysfunction. A comparative pathohistological study of primarily vs secondarily nephrectomized kidneys revealed no evidence of parenchymal damage of the kidney after arterial reconstruction. Both acute and chronic animal experiments in which autologous whole blood was forcibly injected into the canine renal artery via extracorporeal shunt under the high pressure of 200 or 300 mmHg showed no light microscopic evidence of acute histological damage of the kidney. It is concluded that the intensive care with an aid of a Swan Ganz catheter during the postoperative polyuric period and the swift starting of hemodialysis when necessary can solve the postoperative problems of one-kidney renovascular hypertension although the sudden rise in renal perfusion pressure after reconstruction may cause an acute hypertensive damage in the level of electron microscopic findings.     

Renovascular hypertension associated with neurofibromatosis: two cases and review of the literature

The authors report two cases of renovascular hypertension associated with neurofibromatosis. A 19-year-old woman was admitted to our hospital with a complaint of abdominal pain and blood pressure of 180/120 mmHg. Examination revealed café-au-lait spots over her chest and extremities. Peripheral plasma renin activity (PRA) under basal conditions was 2.8 ng/ml/h and increased to 12.6 ng/ml/h after administration of 50 mg captopril. Plasma and urinary catecholamines were normal. Selective renal angiography showed left aneurysmal dilatation of the segmentary branch and right renal artery stenosis with multiple aneurysmal affecting different branches. Blood pressure was controlled by multiple drugs, including beta-blockers and angiotensin-converting enzyme inhibitor. Another patient, a 20-year-old woman, was admitted because of severe arterial hypertension, numerous café-au-lait spots, scoliosis, and mass over the right arm. PRA from the right renal vein was extremely elevated, and selective angiography demonstrated bilateral renal artery stenosis. Aortorenal bypass was performed successfully.     

Unilateral renal angiodysplasia in a girl with hypertension

We report a case of very unusual renal vascular anomaly: angiodysplasia. The patient suffered from acute pyelonephritis immediately after birth. Renal ultrasonography, performed at age 1 day, revealed an enlarged left kidney with heterogeneously increased echogenicity, which involuted rapidly in 3 months. At age 10 years, she presented with severe hypertension during a course of acute pyelonephritis. Peripheral plasma renin activity was high. Computed tomographic angiogram revealed a very small but functioning left kidney. A single, narrow, left renal artery did not have focal stenosis. Pathology examination revealed dysplastic arterioles at the subcapsular area. After left nephrectomy, the blood pressure and the plasma renin activity were normalized. In conclusion, this is an unusual case of renal angiodysplasia, which induced renin-dependent hypertension.     

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.     

Technical consideration in management of renovascular hypertension in an infant with double renal arteries

Renovascular hypertension is a curable disease that has recently been recognized with increasing frequency. A renal scan in a 1-month-old hypertensive white male showed diminished function of the right kidney, and his peripheral vein renin was elevated. Multiple antihypertensive medications failed to control his hypertension, and right kidney function deteriorated. An arteriogram showed two stenotic renal arteries supplying the right kidney. The smaller inferior artery supplied 35% of the kidney. Selective vein renin levels were greater than 15,000 ng/dL on the right side. Technical considerations in the repair of this lesion included midline transabdominal incision to expose the abdominal aorta and the inferior vena cava; dissection of inferior vena cava (IVC) with division of selected lumbar veins; full mobilization of right kidney and transsection of both renal arteries and the renal vein; perfusion of kidney via each renal artery with cold Sach's solution after resection of arterial stenoses; end-to-side microvascular anastomosis of the smaller (2 mm) renal artery to the main renal artery at the hilum with 10-0 nylon over in situ perfusion cannula; renal artery passed under the IVC to the aorta; and right kidney autotransplanted to a new site on the abdominal aorta with an end-to-side (5.0 mm) renal artery to the aorta and an end-to-side renal vein to IVC anastomosis. Following revascularization, perfusion was excellent and the blood pressure returned to normal. At 6 months follow-up, selective renal vein renins were normal and an arteriogram showed no stenosis. Meticulous dissection, cold perfusion, microvascular anastomosis, and autotransplantation salvaged this kidney and resolved the hypertension.     

An infant with systemic hypertension, renal artery stenosis, and neuroblastoma

A newborn girl with neuroblastoma presented with hypertension (blood pressure 200/140 mm Hg). The concentration of active renin in the ipsilateral renal vein was exceedingly high compared with those in the other venous systems, and angiography results showed narrowing of the contralateral 2 renal arteries. The tumor regressed in size after chemotherapy, but the blood pressure remained high. Percutaneous transluminal angioplasty (PTA) for the left renal arteries was performed twice, the first one at 5 months of age, which achieved some success in the recovery of impaired kidney function. At 8 months of age, she underwent radical resection of the neuroblastoma and removal of the right kidney, and the blood pressure promptly returned to normal postoperatively. The current patient represents the second youngest, well-documented case of renovascular hypertension with neuroblastoma in early infancy.     

Dissecting aneurysm of renal artery owing to subadventitial angioma

A case of renovascular hypertension caused by a dissecting aneurysm of the main right renal artery owing to a subadventitial angioma is described. The right kidney was not functioning but, nevertheless, an aortorenal bypass was done and function of the kidney was recovered. Although the hypertension was controlled for 6 months it recurred. A nephrectomy was performed and the hypertension was cured.     

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.     

A case of renovascular hypertension accompanied by focal segmental glomerulosclerosis like lesion in the contralateral kidney

A 19-year-old female patient with renovascular hypertension accompanied by focal segmental glomerulosclerosis like lesion in the contralateral kidney is described. She was admitted to our hospital for uncontrollable hypertension which was first pointed out at check-up when she was 18 years old. On admission physical examination revealed remarkable hypertension and a bruit around the umbilical region. Laboratory findings disclosed renal dysfunction and slight proteinuria (0.8-1.1 g/day). Arteriography revealed severe narrowing of the right renal artery suggesting fibromuscular dysplasia. Taken together, renovascular hypertension was diagnosed. She underwent an operation to reconstruct the artery with autotransplantation of the ipsilateral kidney in the pelvic cavity, and sections were taken from both kidney for histological evaluation during the procedure. After the operation her blood pressure normalized and proteinuria decreased. And interestingly, the kidney specimens revealed that the left side consisted of focal segmental glomerulosclerosis like lesion whereas the right was only of ischemic change. Hyperfiltration theory has recently been highlighted experimentally. And the etiology of the renal findings in this case may be based on such hemodynamic alteration as has been described in animal models. We present the case so that it may be of some help to understand how focal segmental glomerulosclerosis is brought about in humans.     

Therapeutic experience in renal hypertension: response treatment in 47 cases.

We report our experience in the treatment of 47 cases of renal hypertension patients with renovascular hypertension or hypertensive reno-parenchymal disease surgical therapy is essential and non-surgical therapy has its limitations. The necessity for an operation can be based on the determination of bilateral renal vein plasma renin values. Blood should be drawn from both renal veins in patients in an upright position. Surgical treatment usually produces excellent results in patients in whom renin activity on the side of the lesion is more than normal and more than 2 times that on the uninvolved side. It is advisable to perform an operation within 4 years of the onset of renal hypertension. Complete surgical cure of renal hypertension is accomplished more frequently in patients with fibromuscular dysplasia than in those atherosclerosis. The therapeutic effect of an operation in correcting hypertension is virtually the same, regardless of whether the arterial stenotic lesions are in the main renal artery or in the intrarenal arterial branches. We have found nephrectomy or dacron bypass graft to be the surgical procedure of choice in renovascular hypertension cases.     

Therapeutic guide for renovascular hypertension with reevaluation of surgical treatment

Therapeutic guide for renovascular hypertension has been greatly changed by a development of beta-blockers and captopril, and an introduction of percutaneous transluminal angioplasty (PTA) The accepted opinion was that surgical therapy was superior to drug therapy since Hunt & Strong reported the follow-up results in 1973. However, efficacy of drug therapy was reevaluated by an appearance of beta-blockers and captopril and the number of patients applied to operation was decreased. Further, since PTA was widely used in clinical practice from the end of 1970s, surgical therapy for renovascular hypertension was hardly or never considered. Has the necessity of surgical therapy really ceased to exist? Recently, we encountered 2 cases of bilateral renovascular hypertension and reevaluated the necessity of surgical therapy during the course of treatment. The first case was in a 43-year-old male, for whose bilateral renovascular stenosis a bilateral PTA was applied. One year later a complete occlusion of the right renal artery and re-stenosis of the left renal artery developed. Thus, removal of the right kidney and the auto-transplantation of the left kidney were conducted. The second case was in a 17-year-old female with bilateral renovascular stenosis complicated by moya-moya disease. PTA was conducted for the left kidney with shorter range of stenosis and auto-transplantation was conducted for the right kidney with longer range of stenosis. The prognosis was favorable in both cases and hypertension was cured or improved. We recognized and re-evaluated the necessity of surgical therapy for patients who were unsuccessful to PTA or patients with bilateral renovascular hypertension from our experience and literatures.(ABSTRACT TRUNCATED AT 250 WORDS)     

Renovascular hypertension in children. Report of seven cases treated surgically including two cases of renal autotransplantation

Seven children between the ages of three and seventeen years have been treated surgically for renovascular hypertension. Two additional cases treated elsewhere but studied subsequently are cited because of special features of interest. Two patients had primary nephrectomy because of irreparable vascular lesions and contralateral intact kidneys. One patient was treated with midsegmental partial nephrectomy for intimal and medial fibroplasia. Another patient with duplex renal arteries and stenosing lesions involving the upper main renal artery to the left kidney and lower segmental artery to the right kidney was treated by left nephrectomy and right lower pole resection. One patient with subadventitial fibroplasia was treated with a hypogastric artery autograft, and two patients were treated with autotransplantation, one having had simultaneous contralateral nephrectomy for a nonfunctioning kidney because of a previously unsuccessful splenorenal bypass.     

Heart and renal failure in renovascular hypertension caused by giant cell arteritis--case report

We report a case of a male teenager with severe heart and acute renal failure as the dominant clinical manifestations of renovascular hypertension (RVH) caused by atypical giant cell arteritis (GCA). Unrecognized RVH and treatment of the consequent heart failure by angiotensin-converting enzyme inhibitors (ACEI) probably contributed to progression of renovascular disease to bilateral renal artery occlusion. Recurrent "flash" pulmonary edemas could not be prevented until surgical revascularization of the only functioning right kidney was achieved by an aortorenal bypass. Prompt post-operative normalization of heart function and arterial hypertension occurred despite the histopathological finding of the resected renal artery compatible with GCA and 4-year duration of significant renovascular disease. At the last check-up, the patient was asymptomatic, with normal arterial pressure on the prescribed treatment: carvedilol, hydrochlorothiazide, prednisolone 20 mg daily and aspirin. Subsequent follow-up is necessary to observe the evolution of GCA as an exceptionally rare cause of RVH.     

Acute renal thrombosis induced by angiotensin-converting enzyme inhibitors in patients with renovascular hypertension

Acute reversible renal failure is a widely recognized potential complication of angiotensin-converting enzyme inhibitor (ACEI) administration in renovascular hypertension, particularly in bilateral artery stenosis or stenosis involving a solitary kidney. We report herein 2 cases of a rare but severe complication, i.e., acute renal artery thrombosis. Whereas marked hypotension does not seem to be necessary to trigger acute renal failure following ACE inhibition, by contrast, the fall in blood pressure could play an important role in our patients. Since single-dose ACEI has been advocated in order to improve the predictive value of renal scintigraphy or plasma renin activity measurements in the diagnosis of renovascular hypertension, the potential risk of this approach should be stressed.     

Long-term immunosuppressive treatment of a child with Takayasu's arteritis and high IgE immunoglobulins

A 7-year-old child presented with a severe form of Takayasu's arteritis, with two consecutive episodes involving the right testis and then the left kidney 6 months later. The renal artery obstruction was accompanied by severe hypertension. An aortography showed a complete obstruction of the left renal artery and a narrowing of the right subclavian artery. Plasma renin activity was high. Serum immunoglobulins were within the normal range, except for an increase in IgE (880 /l). Despite 4 months', treatment with antihypertensive drugs, prednisone, cyclophosphamide, and anticoagulant, the blood pressure never returned to normal and the left renal function remained completely absent. A nephrectomy was performed which immediately normalized plasma renin activity and blood pressure. The child was subsequently treated with alternateday prednisone for 3 months, alternating with 3 months of cyclophosphamide or, later, azathioprine. Persantine (dipyridamole) and acetylsalicylic acid were administered continuously. The right radial pulse returned to normal within 2 years. An 8-year follow-up failed to detect any new episode of arteritis. The right kidney showed signs of compensatory hypertrophy. Finally, a recent arteriography demonstrated not only a normal right renal artery blood flow but almost total disappearance of the right subclavian artery obstruction. However, the IgE remained abnormally high (2,023 g/l).