Bilateral Renal Disease Research Articles

Screening for renovascular disease with captopril-enhanced renography.

Changes in renal function caused by angiotensin-converting-enzyme (ACE) inhibitors can be detected on 99mTc-DTPA renography so that DTPA scanning before and after a single dose of captopril can be used to screen for renovascular disease. We have performed captopril-DTPA scans with renal arteriography on 104 patients, of whom 27 had renal artery stenosis, all due to atheroma. Using a 5% fall in divided function or a delay of greater than 15 min in time to peak activity on one side after captopril, or the finding of greater than 90% divided function on one side before captopril as criteria for a positive scan, a sensitivity of 93% and specificity of 70% was achieved. The negative predictive value of the test in our population was 93%. Bilateral improvement in renographic function after captopril was seen in patients with accelerated phase hypertension. The presence of bilateral renal artery disease did not reduce the sensitivity of the test, but sensitivity was reduced (75%) in patients with renal impairment. Clinical characteristics in our patients most strongly associated with renal artery stenosis were abdominal bruit, recurrent left ventricular failure, and peripheral vascular disease. In view of the well-publicized risks of ACE inhibitor therapy, care should be exercised in the use of these agents in such patients.

Acquired Structural Genitourinary Abnormalities Contributing to Deterioration of Renal Function in Older Patients with Nephropathic Cystinosis

The natural progression of nephropathic cystinosis to end stage renal disease can be delayed, sometimes by many years, by the reducing agent, cysteamine, which lowers intracellular cystine content to near normal. We report on two patients with nephropathic cystinosis who were treated with cysteamine and developed structural genitourinary abnormalities which may have contributed to an increase in the rate of decline of renal function. One patient, aged 11 years, was found to have massive megacystis and hydroureteronephrosis but no anatomic bladder outlet obstruction. His abnormality was presumed to be related to chronic high urine volumes leading to megacystis and physiologic ureteral obstruction. Vesicostomy stabilized renal function. The second patient, aged 11 1/2 years, was found to have bilateral renal cystic disease which presumably was acquired and may have been related to long-standing hypokalemia. Minor renal abnormalities were found by ultrasound in five additional cystinotic children. We concluded that older children with nephropathic cystinosis may be prone to acquire structural abnormalities of their kidneys or urinary tract.

Pediatric Renal Artery Dysplasia: A Morphologic Study

Renal arteries from 12 pediatric patients who had stenosis due to renal artery dysplasia (9 surgical resections and 3 autopsy cases) were examined by light microscopy. The patients ranged in age from 15 months to 14 years; the male/female ratio was one. Bilateral renal artery disease was clinically diagnosed in 8 of the 12 patients; 6 of the 8 also had aortic anomalies. All of the lesions were designated as forms of dysplasia, a departure from previous reports in which dysplasia was subclassified as fibroplasia and hyperplasia. The most common lesion was medial fibrous dysplasia (6), followed by perimedial elastic dysplasia (4); intimal fibrous dysplasia (1) and medial muscular dysplasia (1) were the least common. Medial fibrous dysplasia was present as often with bilateral as with unilateral renal artery stenosis, while perimedial elastic dysplasia was seen exclusively in patients with bilateral renal artery disease. Three patients who came to autopsy had extrarenal vascular involvement by a similar dysplastic lesion. The three patients who had neurofibromatosis showed no distinctive histologic type of renal artery dysplasia.

Improved Results of Vascular Reconstruction in Pediatric and Young Adult Patients with Renovascular Hypertension

From 1955 to 1988, 56 patients 21 years old or younger underwent surgical treatment for renovascular hypertension at our clinic. The cause of renal artery disease was fibrous dysplasia in 53 patients, Takayasu’s arteritis in 2 or an arterial aneurysm in 1. Bilateral or branch renal artery disease, and extrarenal arterial disease were present in 16, 23 and 11 patients, respectively. The results of 28 patients treated from 1955 to 1977 (group 1) were compared to those of 28 patients treated from 1978 to 1988 (group 2).Hypertension was cured or improved postoperatively in 83% of the patients from group 1 and in 96% from group 2 (p = 0.07). However, this outcome was achieved through surgical revascularization in only 48% of the patients from group 1 compared to 96% from group 2 (p = 0.0002). A multivariate analysis revealed that the only significant variable related to clinical outcome was the era of treatment, which reflects the improved technical efficacy of revascularization during the last decade. Aortorenal bypass and renal autotransplantation have emerged as the preferred revascularization operations. It currently is possible to achieve amelioration of hypertension and preservation of renal function in most young patients with renal artery disease.

Acute Renal Insufficiency Due to Renal Infarctions in a Patient With Neurofibromatosis

Manifestations of neurofibromatosis in the skin, the eye, and the skeletal and nervous systems have been well documented since the disease was first described in 1882. Stenosing vascular lesions as complications of neurofibromatosis were first reported in 1945. They are being increasingly recognized and most commonly involve the renal artery. Renal artery stenosis (usually proximal), intraparenchymal renal arterial abnormalities, and coarctation of the abdominal aorta often lead to hypertension. However, despite reports of bilateral and severe renal artery disease, renal infarction and resulting renal insufficiency have not been described. We present the case of a 35-year-old woman with neurofibromatosis and chronic hypertension associated with narrowing of right intrarenal arteries. The patient had two separate episodes of left renal infarction documented clinically and radiographically. The second infarct resulted in renal insufficieny. There was no hypercoagulopathy or source for embolism. This case suggests that renal infarction and renal insufficiency are additional complications of neurofibromatosis.

Use of the Gastroduodenal Artery in Right Renal Artery Revascularization

Of 15 patients having revascularization of the right renal artery with the use of the hepatic circulation from May 1984 through March 1987 at the Massachusetts General Hospital, eight patients had this accomplished with end-to-end anastomosis of the gastroduodenal artery and right renal artery. Operative indications were acute azotemic renal failure (three patients), poorly controlled renovascular hypertension (four patients), and staged repair of bilateral renal artery disease (one patient). All revascularizations were successful in restoring renal function or rendering hypertension manageable and were assessed by means of renal flow scans, celiac angiography, or return of function in those patients with a solitary, functioning kidney. All patients survived the operation with one late death caused by myocardial infarction after abdominal aortic aneurysm repair. The gastroduodenal artery may be used as the source for arterial inflow in revascularization of the right renal artery by end-to-end anastomosis in approximately 50% of instances, conferring the advantage of the use of only one anastomosis and obviating the long-term possibility of vein graft failure.

Follow-up of Infants with Bilateral Renal Disease Detected in Utero

Follow-up of Infants with Bilateral Renal Disease Detected in Utero

Severe bilateral renal disease: correlation of antenatal and autopsy findings.

We report 20 infants with severe bilateral renal disease examined by prenatal ultrasound and by autopsy. In 17, the prenatal and pathologic diagnoses correlated well. Although the prenatal and autopsy findings differed in the three remaining cases, the autopsy confirmed the presence of severe bilateral renal abnormalities. All 20 pregnancies were complicated by oligohydramnios, which was severe in 60 per cent. Most of these fetuses had malformation of other organ systems. This series supports the utility of prenatal ultrasound examinations, but emphasizes the need for postnatal evaluation of congenital renal disease including pathologic examination of tissue when possible for correct classification and genetic counselling.

Follow-Up of Infants With Bilateral Renal Disease Detected In Utero. Growth and Renal Function

• We studied 69 infants who had urinary tract abnormalities detected by antenatal ultrasound examination. There were 21 intrauterine or immediate neonatal deaths; in all 21 infants, severe bilateral renal disease incompatible with life was found at autopsy. Six of the liveborn infants with abnormal results of antenatal ultrasound examinations had a normal urinary tract after birth. Of the remaining 42 infants, the prenatal diagnosis was confirmed with renal ultrasound and other studies during the first week of life. Twenty-one of 42 infants had bilateral renal disease. We obtained follow-up data on 19 of 21 of these infants. Twelve of 19 had obstructive uropathy that was treated surgically. After one to 51 (mean, 18) months of follow-up, renal function varied. Ten of 19 patients had a calculated glomerular filtration rate greater than or equal to 79 mL/min/1.73 m 2 . One infant required long-term ambulatory peritoneal dialysis. Renal function (glomerular filtration rate, 74±5 mL/min/1.73 m 2 ) and growth (height percentile, 41±8) were unexpectedly good considering the severity of the urinary tract abnormalities. Prenatal detection of bilateral renal disease followed by careful medical and surgical management results in a favorable outcome with good growth and renal function. ( AJDC 1988;142:453-456)

Use of the gastroduodenal artery in right renal artery revascularization

Of 15 patients having revascularization of the right renal artery with the use of the hepatic circulation from May 1984 through March 1987 at the Massachusetts General Hospital, eight patients had this accomplished with end-to-end anastomosis of the gastroduodenal artery and right renal artery. Operative indications were acute azotemic renal failure (three patients), poorly controlled renovascular hypertension (four patients), and staged repair of bilateral renal artery disease (one patient). All revascularizations were successful in restoring renal function or rendering hypertension manageable and were assessed by means of renal flow scans, celiac angiography, or return of function in those patients with a solitary, functioning kidney. All patients survived the operation with one late death caused by myocardial infarction after abdominal aortic aneurysm repair. The gastroduodenal artery may be used as the source for arterial inflow in revascularization of the right renal artery by end-to-end anastomosis in approximately 50% of instances, confering the advantage of the use of only one anastomosis and obviating the long-term possibility of vein graft failure.

Follow-up of infants with bilateral renal disease detected in utero. Growth and renal function.

We studied 69 infants who had urinary tract abnormalities detected by antenatal ultrasound examination. There were 21 intrauterine or immediate neonatal deaths; in all 21 infants, severe bilateral renal disease incompatible with life was found at autopsy. Six of the live-born infants with abnormal results of antenatal ultrasound examinations had a normal urinary tract after birth. Of the remaining 42 infants, the prenatal diagnosis was confirmed with renal ultrasound and other studies during the first week of life. Twenty-one of 42 infants had bilateral renal disease. We obtained follow-up data on 19 of 21 of these infants. Twelve of 19 had obstructive uropathy that was treated surgically. After one to 51 (mean, 18) months of follow-up, renal function varied. Ten of 19 patients had a calculated glomerular filtration rate greater than or equal to 79 mL/min/1.73 m2. One infant required long-term ambulatory peritoneal dialysis. Renal function (glomerular filtration rate, 74 +/- 5 mL/min/1.73 m2) and growth (height percentile, 41 +/- 8) were unexpectedly good considering the severity of the urinary tract abnormalities. Prenatal detection of bilateral renal disease followed by careful medical and surgical management results in a favorable outcome with good growth and renal function.

Simultaneous aortic reconstruction and renal revascularization: Risk factors and late results in eighty-nine patients

From 1973 through 1984, graft replacement of infrarenal aortic aneurysms (N = 56) or occlusive disease (N = 33) was performed in conjunction with simultaneous renal revascularization in 89 patients. Isolated renal artery stenosis was corrected by unilateral reconstruction in 56 patients (63%), but the remaining 33 (37%) had diffuse involvement that required either bilateral renal artery grafts or unilateral revascularization of solitary kidneys. The incidence of hypertension (greater than 18090 mm Hg) refractory to preoperative medical therapy (88%), severe coronary disease documented by angiography (40%), and postoperative azotemia (33%) or oliguric renal failure (15%) was significantly higher among patients with bilateral renal artery disease (p < 0.05). In addition, this group had twice the early mortality rate (15%) of patients having unilateral renal artery lesions (7.1%). During a mean follow-up interval of 37 months, medical control of hypertension was enhanced in 46 of the 80 operative survivors (58%), and renal function improved or remained stable in 63 survivors (79%). Five-year actuarial survival presently is 65% for the entire series, with a cumulative mortality rate of 38% among patients who underwent aneurysm resection (mean age 64 years) in comparison to 15% (p = 0.03) for those patients with aortoiliac occlusive disease (mean age 60 years).

Answer to Diagnostic Challenge II Bilateral Multicystic Dysplastic Renal Disease

Answer to Diagnostic Challenge II Bilateral Multicystic Dysplastic Renal Disease

Critical perfusion pressure for renal function in patients with bilateral atherosclerotic renal vascular disease: Textor SC, Novick AC, Tarazi RC, et al. Ann Intern Med 1985; 102: 308-14

Critical perfusion pressure for renal function in patients with bilateral atherosclerotic renal vascular disease: Textor SC, Novick AC, Tarazi RC, et al. Ann Intern Med 1985; 102: 308-14

Unilateral Presentation of Adult-Type Polycystic Kidney Disease in Children

Adult polycystic kidney disease is characterized by autosomal dominant inheritance, diffuse bilateral cystic kidney disease and onset during the young adult years. Relatively few cases of adult-type polycystic kidney disease in children have been reported. We describe a 4-year-old girl who appeared to have multiple, unilateral simple renal cysts by excretory urography and ultrasonography. There was no known familial renal cystic disease. When renal histology was consistent with adult-type polycystic kidney disease, screening nephrosonograms revealed that the mother also had the disease. We review 10 published cases of unilateral presentation of adult-type polycystic kidney disease in children. This report emphasizes the necessity of screening studies in parents of children with diffuse unilateral or bilateral cystic renal disease. Awareness of the unexpected unilateral presentation of adult-type polycystic kidney disease in children may allow a nonoperative diagnosis to be made.

Pharmaco-epidemiological considerations in patients with arthritis and vascular disease of the kidney.

Non-steroidal anti-inflammatory drugs (NSAIDs) may produce acute renal failure, papillary necrosis and interstitial nephritis. These adverse drug reactions are rare but have been reported in patients with congestive heart failure, cirrhosis, renal parenchymal disease, lupus nephritis and hypertension. All these conditions may be associated with hypovolaemia and an activated renin-angiotensin system, when renal blood flow and glomerular filtration depend on local renal prostaglandin biosynthesis. A severe impairment of renal function may occur when this synthesis is inhibited by NSAID treatment. It is possible that 1 in 100 of elderly patients have renal parenchymal disease, 1 in 100 arteriolar nephrosclerosis, 1 in 200 unilateral or bilateral renal artery stenosis and an unknown number suffer from atheroembolic renal disease. Fortunately, only a small proportion of 'at risk' patients given NSAIDs appear to develop renal failure. Perhaps bilateral renal disease or salt depletion are necessary factors? Whatever the explanation, NSAIDs should be used with caution in the elderly.

Critical perfusion pressure for renal function in patients with bilateral atherosclerotic renal vascular disease.

We studied renal plasma flow and glomerular filtration rate during graded blood pressure reduction induced with sodium nitroprusside infusion in 16 hypertensive patients with atherosclerotic renovascular disease. Eight patients with unilateral disease tolerated pressure reduction from 205 +/- 9 (SE)/103 +/- 2 mm Hg to 146 +/- 6/84 +/- 3 mm Hg (p less than 0.01) with no change in total renal function. In 8 other patients with bilateral renal arterial stenosis (all arteries 70% or more stenosed), similar pressure reduction produced marked but reversible decrements in plasma flow (152 +/- 28 mL/min to 66 +/- 13 mL/min; p less than 0.01) and glomerular filtration rate (38 +/- 8 mL/min to 16 +/- mL/min; p less than 0.01). In 4 patients restudied after revascularization, sensitivity of renal function to pressure changes was no longer present. These data indicate that vascular stenosis to the entire renal mass may limit function and provide a means for evaluating patients at risk for loss of renal function during antihypertensive therapy.

Pure primary hyperaldosteronism due to adrenal cortical carcinoma

A 47-year-old woman is described who had pure primary hyperaldosteronism due to an adrenal cortical carcinoma. This may represent the first such case in which modern laboratory tests allowed specific diagnosis and exclusion of hypersecretion of other adrenal steroids, and also the first reported case in which modern localizing procedures have been utilized. Other interesting facets of the case include calcification of the tumor, visualization with 131I iodomethylnorcholesterol, metaplastic histologic changes, and coexistent bilateral renal artery fibromuscular disease.

99mTc-aprotinin: A new tracer for kidney morphology and function

Aprotinin (Ap), a low-molecular-weight polypeptide (6500 dalton), is a protease inhibitor which is electively and stably accumulated in the kidney. In 112 adult patients, with either uni- or bilateral renal disease with different degrees of renal impairment (from normal GFR to advanced renal failure), renal scans were performed by means of Ap labelled with 99mTc. Highly satisfactory renal scans were obtained in all patients. In 20 patients with renal failure (serum creatinine 1.8-8.5 mg/dl, mean 4.7) a comparison was made of the renal scans obtained with 99mTc-Ap and with 99mTc-DMSA. 99mTc-Ap was slightly better than 99mTc-DMSA, especially in patients with far advanced renal failure. Some aspects of the pharmacokinetics of 99mTc-Ap were studied in 72 cases. In 22 of these patients plasma clearance of 99mTc-Ap was determined by the single injection method using a two-compartment model. In patients with GFR greater than 90 ml/min plasma clearance of 99mTc-Ap was 67.6 +/- 8.4 SD ml/min. A good correlation was observed between plasma clearance of 99mTc-Ap and GFR (r = 0.74). After IV injection 99mTc-Ap was stably fixed by the kidney. Renal radioactivity remained stable between the second and eighth hour after the injection. Urinary excretion of radioactivity measured in 35 patients in the first and in the second 2-h interval after IV injection of 99mTc-Ap was negligible in all patients (2.7 +/- 1.5 SD percent of the dose in the first 2 h; 2.8 +/- 1.4 SD between the second and fourth hour). 99mTc-Ap is an excellent agent for renal imaging. It also seems promising for renal function studies.

Renal Failure Associated With Captopril

To the Editor.— We are beginning to see certain clinical situations wherein captopril therapy has been temporally associated with the development of acute renal failure. 1,2 For the most part, these observations have been made in persons exhibiting serious degrees of renal artery stenosis in the setting of a solitary kidney or with bilateral renal artery disease. We have observed two such persons (ages 63 and 55 years) with chronic renal failure (serum creatinine level, 3 to 4 mg/dL) and high-grade renal artery stenosis, both of whom had a solitary functioning kidney and experienced reversible renal failure (serum creatinine level, 7 to 7.3 mg/dL) concomitant with captopril administration. Although renal biopsies were not performed, clinical history, urinalysis, and rate of recovery after captopril therapy discontinuation argued against short-term toxic effects of the drug as being responsible for the renal failure. In patients with serious renal artery stenosis, overvigorous diuresis and/or