Renal replacement therapy in multiple myeloma and systemic amyloidosis

Renal failure frequently complicates both multiple myeloma and systemic amyloidosis. Renal replacement therapy (RRT) may be poorly tolerated and its role in such patients is not clearly defined. Of fifty patients (26 males and 24 females) referred to a single centre because of renal failure associated with multiple myeloma or systemic amyloidosis 37 progressed to end-stage renal failure and 30 of these patients received RRT. Nine patients have been treated by CAPD, 13 by haemodialysis, and 8 patients have required both forms of dialysis. Overall one year and two year survival rates were 66% and 57% respectively. The median duration on RRT was 7.5 months (range 1-96 months) with a 51% one year, and a 46% two year survival rate. Of 7 patients with amyloidosis who underwent renal transplantation, 3 died within 6 months of transplantation. Undiagnosed cardiac involvement contributed to this early mortality. We conclude that renal replacement therapy is appropriate for some patients with multiple myeloma and systemic amyloidosis who develop endstage renal failure. Careful assessment and selection of patients is necessary prior to renal transplantation.     

Bilateral renal carcinoma in von Hippel-Lindau Disease

Von Hippel-Lindau disease, one of the phakomatoses, is believed to be a disorder of mesodermal differentiation. Renal lesions, usually cysts or adenocarcinomas with an occasional hemangioblastoma, occur in approximately two thirds of all patients. The renal neoplasms previously reported have been multiple, bilateral, and usually beyond resection. A thirty-eight-year-old white male with a cerebellar hemangioblastoma and bilateral renal adenocarcinoma underwent suboccipital craniotomy, right heminephrectomy, and left radical nephrectomy. No evidence of recurrent disease can be identified ten months postoperatively. An aggressive approach in this systemic disease appears to be warranted.     

Unilateral nephrectomy in dogs with renal disease: 30 cases (1985-1994)

OBJECTIVE: To evaluate indications for and complications, efficacy, and effects on renal function of unilateral nephrectomy in dogs with renal disease, and to evaluate the role that scintigraphy had in the decision to excise a kidney. DESIGN: Retrospective case series. ANIMALS: 30 dogs with renal disease that underwent unilateral nephrectomy. A comparison group of 12 dogs with renal calculi that underwent renal scintigraphy but not nephrectomy was included. RESULTS: Indications for nephrectomy included renal or ureteral calculi (n = 10), renal mass (8), chronic pyelonephritis (5), perirenal mass (3), severe hydronephrosis and hydroureter (3), and renal hypoplasia with ureteral ectopia (1). None of the dogs were azotemic before surgery. Renal scintigraphy apparently influenced the decision to perform nephrectomy, because in 14 of 16 dogs that underwent nephrectomy, the affected kidney contributed < or = 33% of the total glomerular filtration rate, but in 6 of 8 comparison dogs that underwent nephrotomy, the affected kidney contributed > 33% of total glomerular filtration rate. Complications of nephrectomy included oliguria (5) and organ laceration (2). Mean +/- SD final serum creatinine concentration for 16 dogs alive at least 6 months after nephrectomy was 2.2 +/- 1.8 mg/dl. Three dogs had chronic renal failure of undetermined cause at the time of death. Nephrectomy did not completely resolve the underlying disease in 13 dogs. Renal function was evaluated in 6 dogs 2 to 3.5 years after nephrectomy and was impaired in 4. None of the dogs were anemic, azotemic, proteinuric, or hypertensive. Survival time varied depending on the underlying disease. CLINICAL IMPLICATIONS: Multiple factors contributed to the decision to perform nephrectomy. Unilateral nephrectomy resulted in few serious complications and was not detrimental to the remaining kidney, but did not always resolve the underlying disease.     

En bloc nephrectomy in patients undergoing post-chemotherapy retroperitoneal lymph node dissection for nonseminomatous testis cancer: indications, implications and outcomes

PURPOSE: We review the indications for nephrectomy at post-chemotherapy retroperitoneal lymph node dissection, identify patients at risk for nephrectomy and assess the impact of nephrectomy on outcome. MATERIALS AND METHODS: Using a computerized data base and chart review we retrospectively reviewed the records of 848 patients who underwent retroperitoneal lymph node dissection after chemotherapy. RESULTS: En bloc nephrectomy was performed at retroperitoneal lymph node dissection after chemotherapy in 162 of the 848 patients (19%). The indications for nephrectomy included contiguous involvement of perirenal structures in 73% of the cases, renal vein thrombosis in 6%, a poorly functioning or nonfunctioning renal unit in 5% and a combination of these conditions in 16%. Pathological studies of the hilum revealed cancer in 20% of the cases, teratoma in 49% and fibrosis in 31%. Patients requiring nephrectomy had significantly more advanced disease and larger disease volume at presentation and after chemotherapy. There were no significant differences in perioperative morbidity or mortality compared with patients who did not undergo nephrectomy. Only 3 patients required perioperative dialysis and none required long-term renal support. CONCLUSIONS: These findings support en bloc nephrectomy at post-chemotherapy retroperitoneal lymph node dissection in select patients with large volume perihilar retroperitoneal disease.     

Treatment of end-stage renal failure after heart transplantation

BACKGROUND: Five to 10% of heart-transplant recipients develop end-stage renal failure (ESRF). Little is known about the outcome of these patients under renal replacement therapy. METHODS: We conducted a retrospective study in 16 men (mean age 52.8+/-7.4 years at heart transplantation) who developed ESRF 5.3+/-2.1 years later. Results. Haemodialysis (HD) was the first-line treatment (mean Kt/V 1.35+/-0.4). Vascular access was unsuccessful in six patients (37.5%) due to peripheral arteriopathy and they were treated with tunnelled catheters for an average 15 months without bacterial infection. Mean weight was 68.4+/-10 kg at onset of HD and 61.7+/-9 kg one month later. Despite this reduction in extracellular overload, one antihypertensive drug was required in 75% of patients and two drugs in 12.5%. One patient tolerated automated peritoneal dialysis (PD) for 16 months (weekly Kt/V 2.1) despite persistent anuria. Renal transplantation (RT) was contraindicated in eight patients because of aortoiliac arteriopathy (n=5), poor general status (n=2), or ischaemic heart disease (n=1). RT was performed in eight patients with no acute episode of heart or renal graft rejection. There were no serious infectious complications. Three months after RT, mean serum creatinine was 115 micromol/l. One patient developed post-transplant lymphoproliferative disorder 3.5 months after RT and was successfully treated with transplant nephrectomy. Sudden death occurred in two patients 18 and 33 months after RT. Overall patient survival was 100, 78, and 59%, 1, 2 and 3 years after HD onset respectively. Using a time-dependent variable, the Cox model analysis demonstrated that heart-transplant recipients with ESRF have a relative risk of death 3.2 times higher than those without ESRF (95% CI = 1.3-7.8). CONCLUSIONS: HD, PD, and RT can be useful for the treatment of ESRF after heart transplantation. After initiating HD, patient survival is nearly the same as that reported in patients in Europe undergoing HD for other causes. But ESRF seems to reduce life expectancy in heart-transplant recipients.     

Determinants of late allograft nephrectomy

When loss of graft function occurs more than six months after transplantation, allograft nephrectomy is not routinely performed at the time of graft failure. It is usually performed only on those patients who subsequently develop specific complications. However, little is known about the characteristics that make patients more likely to require allograft nephrectomy. The purpose of our study was to identify risk factors for the subsequent need for allograft nephrectomy in patients with graft failure occurring more than 6 months after transplantation. Forty-one patients were studied. Inclusion criteria were: loss of graft function > or = 6 months after transplantation, resumption of dialysis and initiation of weaning from immunosuppression. Thirty patients were treated with cyclosporine + prednisone +/- azathioprine and 11 with azathioprine + prednisone. Mean follow-up time was 17.8 months, ranging from 6 months to 6.1 years. Recipient age, sex and race, original renal disease, donor, donor source (cadaveric vs living related), HLA compatibility, levels of panel reactive antibodies, occurrence of initial delayed graft function, causes of graft failure and tapering of immunosuppression were similar in patients with and without allograft nephrectomy. Using univariate analysis, allograft nephrectomy was found to be significantly more frequent in patients with a history of 2 or more episodes of acute rejection than in patients with no rejection episode: 83% vs 30% (p = 0.03). In addition, allograft nephrectomy was found to be significantly more frequent if the immunosuppressive regimen included cyclosporine (62% vs 27.3%; p = 0.04). Using multivariate analysis however, the number of previous episodes of rejection was found to be the only significant predictor for allograft nephrectomy. None of the other variables considered in the multivariate analysis, including the type of immunosuppressive therapy, was identified as a significant predictor for the need to perform allograft nephrectomy. In summary, the need for late allograft nephrectomy was correlated with the number of previous episodes of acute rejection. Patients with a history of numerous rejection episodes should thus be considered more likely to require allograft nephrectomy once immunosuppression is withdrawn. Possible interventions to reduce or prevent the need for nephrectomy include more gradual tapering of immunosuppression at the time of graft failure or indefinite low-dose immunosuppressive therapy.     

Polymorphism in the microsatellite located in the promoters of the Tnfa and Tnfb genes of different mouse strains

BACKGROUND: Hyperparathyroidism is common in patients with renal disease. These patients may require operation for this disease if it cannot be controlled by medical therapy. Because these patients continue to have renal failure, the risk of recurrence and reoperation is high. METHODS: Sixty-nine patients with renal failure underwent operation for hyperparathyroidism. These patients were followed up on dialysis or after transplantation. RESULTS: Sixty-nine patients, aged 2 to 71 years old, with end-stage renal disease required parathyroidectomy for hyperparathyroidism 6.2 +/- 4.2 (standard deviation) years after beginning dialysis. Thirty-six patients had undergone renal transplantation (creatinine = 1.6 +/- 0.4 mg/dL). All patients had elevated parathyroid hormone (PTH) levels. Sixty-eight patients had hyperplasia; 1 patient had adenoma. Six patients required reoperation for recurrent hyperparathyroidism 30 to 123 months after their initial parathyroidectomy. CONCLUSION: Patients with end-stage renal disease are prone to abnormalities of calcium metabolism. They frequently develop parathyroid hyperplasia. Recurrence can occur following operation because of continuing renal failure.     

Vascular complications of kidney transplantation

584 kidney transplantations (208 from cadaveric donors, 376 from living relative donors) were performed in Medical Faculties of the Istambul University in 1986-1997. Thrombosis of the renal artery was observed in 2 patients (0.35%). One of them had diffused arterial atherosclerosis 4 months after the kidney transplantation. In spite of two successful thrombectomies, the patient died 3 months after the last surgical procedure from cerebral thrombosis. The other patient underwent nephrectomy. Hypertension was observed in 63 patients. In 5 of them about 50% stenosis of the anastomotic area was detected by doppler duplex scan and selective angiogram. Transluminal angioplasty was performed in one patient, open surgical correction in one case. Renal vein thrombosis took place in 1 (0.2%) patient. In 2 cases (0.35%) 5 and 12 years after the transplantation aortic aneurysmal dissection was observed.     

Evaluation of the balance between angiogenic and antiangiogenic circulating factors in patients with breast and gastrointestinal cancers

BACKGROUND: Laparoscopy is believed to result in possible clinical benefits for the patient. We report our experience with renal laparoscopy in dialysis patients and compare the results with those from non-dialysis patients. METHODS: Between December 1994 and April 1997, 19 dialysis patients underwent laparoscopic nephrectomy or nephroureterectomy at our hospital. The group consisted of 11 female and eight male patients (mean age 45 years). In nine patients the indication for nephrectomy was chronic pyelonephritis. Nephroureterectomy for vesicoureteral reflux with recurrent episodes of pyelonephritis or analgesic nephropathy for exclusion of transitional cell carcinoma of the upper urinary tract was considered in nine other patients. Laparoscopic bilateral nephrectomy for drug-resistant hypertension was performed in one patient. In comparison, a consecutive group of non-dialysis patients who had undergone renal laparoscopy was reviewed. RESULTS: In the dialysis group, one patient had to be converted to open nephrectomy due to bleeding. Six dialysis patients required blood transfusions compared with none in the non-dialysis group. There were four complications in the dialysis group and two in the non-dialysis group. Both groups had comparable results for operative times, analgesic consumption, postoperative start of oral intake and mobilization, and duration of hospitalization and convalescence. CONCLUSIONS: Laparoscopic nephrectomy in dialysis patients has acceptable results. The higher transfusion rate is probably due to a lower preoperative haemoglobin and is not aggravated by possible affects of the clotting system in patients with chronic uraemia.     

Management conditions during dialysis therapy influence the occurrence of complications after renal transplantation?

We investigated whether the management condition of patients during dialysis therapy has an influence on the occurrence of complications after renal transplantation. Thirty-one patients who underwent renal transplantation were investigated: thirteen received kidneys from living related donors and 18 received cadaveric transplants. The relations between weight gain ratio, cardiothoracic ratio (CTR) and blood pressure during dialysis and the rate of episodes of acute rejection or infection after renal transplantation were analyzed. The rate of acute rejection tended to be higher among patients whose CTR was less than 45% than in those whose CTR was 45% or more. There was no relation found between the rate of infection after transplantation and weight gain ratio. CTR, or blood pressure during dialysis therapy. These results suggest the possibility that the management condition of patients during dialysis therapy influences the rate of acute rejection after these patients undergo renal transplantation.     

Role of the kidney in regulating plasma immunoreactive beta-melanocyte-stimulating hormone

An analysis of the factors that influence the increase in plasma immunoreactive beta-melanocyte-stimulating hormone (beta-MSH) concentration in chronic renal failure showed that: (a) the increase correlated with the increase in serum creatinine concentrations; (b) beta-MSH was not cleared from the plasma by haemodialysis; (c) beta-MSH concentrations increased with length of time on dialysis and increased further after bilateral nephrectomy but there was no further increase with time; (d) beta-MSH levels decreased to normal after renal transplantation; and (e) beta-MSH was excreted in urine only when plasma levels rose to well above those of chronic renal failure (in Nelson's syndrome). These findings suggest that the kidney regulated plasma beta-MSH by a non-excretory mechanism and is the major site of beta-MSH metabolism.     

Impact of renal cadaveric transplantation on survival in end-stage renal failure: evidence for reduced mortality risk compared with hemodialysis during long-term follow-up

Despite a superior quality of life and a favorable cost effectiveness, it has not been well established thus far whether renal cadaveric transplantation contributes to superior survival probability of end-stage renal disease patients in Europe, because the mortality rate on dialysis is lower compared with the United States. This analysis was undertaken to compare the mortality of wait-listed patients and transplant recipients during long-term follow-up, including the possibility of a retransplant in a single-center study. The study cohort included 309 consecutive patients, ages 17 to 72 yr, being registered on the waiting list of the Renal Transplantation Center of Mannheim since the initiation of the transplantation program on June 3, 1989. Follow-up was terminated on September 30, 1997, with a mean of 4.15 yr. A total of 144 renal cadaveric transplants (four retransplants) was performed during the follow-up period. A Cox regression model considering the time-dependent exposure to the different therapy modalities was applied for statistical analysis. Patients being removed from the waiting list or coming back to dialysis after transplantation were censored at time of withdrawal or graft failure. Transplantation resulted in a lower hazard ratio, which was 0.36 (95% confidence interval, 0.15 to 0.87) when the hazard of the wait-listed group was taken as 1.00. The underlying incidence rate of death was 0.026 per patient-year (0.032 on dialysis versus 0.016 with functioning graft). Performing the evaluation on an intention-to-treat basis without censoring the lower risk of the transplanted group was still pronounced according to a hazard ratio of 0.44 (95% confidence interval, 0.22 to 0.89). Thus, patients receiving a renal cadaveric transplantation have a substantial survival advantage over corresponding end-stage renal disease patients on the waiting list even in the setting of a single transplantation center where mortality on regular dialysis therapy was comparatively low.     

In vivo and in vitro diclofenac sodium evaluation after rectal application of soft gelatine capsules enabling application induced transformation (AIT) into a semisolid system of liquid crystals (SSLC) for controlled release

BACKGROUND: Patients with von Hippel-Lindau (VHL) disease are at risk for the development of end-stage renal failure from the treatment of localized renal cell carcinoma. Transplantation with its attendant immunosuppression may predispose patients to tumor recurrence; however, there is little information regarding the outcome with this approach. In this article, we review the North American and European experience with renal transplantation in this patient population. METHODS: The study group comprises 32 patients who have VHL rendered anephric secondary to localized renal cell carcinoma and who have undergone renal transplantation. Patients were identified from North American (n=18) and European (n=14) registries. The outcome of the study group is compared with a cohort of 32 renal transplant recipients without VHL from the Cleveland Clinic Unified Transplant Data Base, who were matched for donor source, gender, age, transplant status (primary vs. regraft), and date of transplantation. RESULTS: The 23 men and 9 women in the study group received transplants between 1974 and 1996. The average age at transplantation was 36 years, and the average duration of dialysis before transplantation was 26 months. Patients have been followed for 48+/-35 months. There was no statistically significant difference in graft survival, patient survival, or renal function between the study and control groups. There were five deaths in both the study and control groups. In the study group, three patients died with metastatic disease. There was no difference in the duration of dialysis before transplantation between patients who developed metastatic disease and those who did not. CONCLUSION: These data support the utility of renal transplantation as an effective form of renal replacement therapy in this unique population, with a limited risk of recurrent cancer.     

Risks and costs of end-stage renal disease after heart transplantation

OBJECTIVES: To estimate the risks and costs of end-stage renal disease (ESRD) after heart transplantation. BACKGROUND: Previous studies have shown high rates of ESRD among solid-organ transplant patients, but the relevance of these studies for current transplant practices and policies is unclear. Limitations of prior studies include relatively small, single-center samples and estimates made before implementing suggested practice changes to reduce ESRD risk. METHODS: Medicare beneficiaries who underwent heart transplantation between 1989 and 1994 were eligible for study inclusion (n=2088). Thirty-four patients undergoing dialysis or who had the diagnosis of ESRD before or at transplantation were excluded from the study. ESRD was defined as any patient undergoing renal transplantation or requiring dialysis for more than 3 months. Mortality and ESRD events were recorded up to 1995. ESRD risk was estimated using the Kaplan-Meier product-limit estimator and logistic regression analyses. Linear regression was performed to determine expenditures for treating ESRD, and we developed long-term models of the risk and direct medical costs of ESRD care. RESULTS: The annual risk of ESRD was 0.37% in the first year after transplant and increased to 4.49% by the sixth posttransplant year. There was no significant trend in the risk of ESRD based on the year of transplantation, even after adjusting for patient characteristics. The average cumulative 10-year direct cost of ESRD per patient undergoing heart transplantation exceeded $13,000. CONCLUSIONS: In a large, national sample of patients undergoing heart transplantation, ESRD is not rare, even for patients undergoing transplant after the development of new practices intended to reduce its occurrence. ESRD remains an important component of the costs of heart transplantation.     

Treatment of uncontrolled hypertension by therapeutic renal infarction

Certain hemodialysis patients need to be made anephric, either surgically or physiologically. Bilateral renal infarction with shredded absorbable gelatin sponge (Gelfoam) was performed on a woman with malignant hypertension being maintained on chronic center dialysis who was too great a surgical risk for bilateral nephrectomy. Peripheral embolization complicated the procedure resulting in a forefoot amputation for dry gangrene two months later. Her postinfarction peripheral plasma renins remained elevated, and she remained hypertensive but was more easily managed with fewer drugs. This technique has been successfully used by others in 1 patient with chronic renal failure and heavy proteinuria and another with hypertension and a solitary kidney. If, as in our case, postinfarction plasma renins remain elevated and hypertension persists, bilateral nephrectomy could be performed at a later date or infarction could be repeated.     

Patients surviving more than 10 years on haemodialysis. The natural history of the complications of treatment

BACKGROUND: The purpose of this survey was to describe the natural history of complications in 52 long-surviving haemodialysis patients to obtain a clearer picture of the impact these patients have on the dialysis population. This is important as they are often no longer suitable for transplantation and therefore are destined to remain on dialysis for the rest of their lives. METHODS: The patients who survived for more than 10 years on haemodialysis alone were studied. Information was obtained from patients' records and from the renal unit computer. RESULTS: Mean age at start of dialysis was 43 years and mean duration of HD 14.5 years. Renal failure was most commonly due to polycystic kidney disease or glomerulonephritis. Sixty-two per cent of patients developed cardiovascular disease, 78% complained of joint pains, 72% had a parathyroidectomy, and 50% developed carpal-tunnel syndrome. Two hundred and forty-five episodes of infection were recorded, 41% related to vascular access acquired in hospital or on immunosuppression. Only three infections occurred which could be described as opportunistic. Twelve patients were hepatitis C positive. In the 37 patients who have died, cardiovascular disease was the most common cause of death. Compared to other patients who started on dialysis before 1986 but who had a successful transplant the survival of patients on haemodialysis is much worse. CONCLUSION: Long-term survival on renal replacement therapy is dependent on successful transplantation. Complications, morbidity, and mortality are high after 10 years of dialysis.     

Efficacy of multimodality therapy in advanced renal cell carcinoma

OBJECTIVES: The integration of systemic biologic response modifier (BRM) therapy and surgery to treat metastatic renal cell carcinoma (RCC) is an evolving approach. The purpose of this study was to evaluate the efficacy of this form of multimodality therapy in patients with metastatic RCC. METHODS: Between 1988 and 1996, 14 patients at our institution underwent initial BRM therapy followed by surgical resection of primary and metastatic RCC lesions. Patient records were reviewed to determine the response to BRM therapy, progression-free survival rate, and overall survival rate. The mean follow-up for the entire group was 43.5 months. RESULTS: After BRM therapy, 9 patients manifested an objective response and 5 patients had stable disease. All patients were then rendered disease-free by surgical excision of residual or recurrent metastatic lesions and the primary tumor. The cancer-specific survival rate at 3 years was 81.5%. Currently, 7 patients are alive and disease-free (mean follow-up 41.4 months), 3 patients are alive with recurrent disease (mean survival 48.3 months), 3 patients died of metastatic disease (mean survival 27.9 months), and 1 patient died of an unrelated cause 54.4 months after therapy. CONCLUSIONS: The results of this study suggest that adjunctive surgery after BRM therapy can extend the survival of selected patients with metastatic RCC. Aggressive surgical resection of stable or responding lesions after BRM therapy should be considered in the management of these patients.     

Renal artery angioplasty for renovascular hypertension and preservation of renal function: long-term angiographic and clinical follow-up

OBJECTIVE: Percutaneous transluminal angioplasty of stenoses of the renal artery can be used to treat hypertension and renal insufficiency. Although many studies have been published on the short-term results of this procedure, few long-term studies are available. SUBJECTS AND METHODS: One hundred ninety-five patients (123 men and 72 women 19-79 years old; mean age, 56 years) with stenosis of the renal artery and hypertension underwent renal percutaneous transluminal angioplasty at our institution. The stenosis was unilateral in 66% of patients, bilateral in 26%, and in a solitary functioning kidney in 8%. Renal insufficiency was present in 31% of patients. After renal percutaneous transluminal angioplasty, long-term clinical and angiographic follow-up was evaluated by life-table analysis. RESULTS: In patients with fibromuscular disease, blood pressure returned to normal in 57%, improved in 21%, and was unchanged in 21%. In patients with atherosclerotic stenosis, blood pressure returned to normal in 12%, improved in 51%, and was unchanged in 37%. After percutaneous transluminal angioplasty, renal function improved in 48% of patients with renal insufficiency due to bilateral stenosis or stenosis in the single functioning kidney, whereas none of the patients with unilateral stenosis of renal artery and renal insufficiency had any notable improvement. Long-term follow-up showed a high rate (82%) of patency of revascularized arteries and a low rate (21%) of hypertension recurrence at 5 years. CONCLUSION: Renal percutaneous transluminal angioplasty is useful for treating hypertension and for reestablishing renal function. Its effects on blood pressure and renal function are long-lasting in the large majority of patients.     

CASES--clinical audit; scenarios for evaluation and study

The incidence of bilateral involvement it is generally estimated to be 5% to 10%. It shows association with certain congenital anomalies and it has an increased occurrence of familial cases. The records of 9 children (5 boys, 4 girls) diagnosed at Vall d'Hebron Hospital with bilateral Wilms tumor between 1976-1995 were analyzed. Six patients had synchronous tumors and 3 had metachronous lesions. Genitourinary malformations were present in 4 children and another had hemihypertrophy. Two children were brothers. Eight patients underwent pre-operative radiation therapy and/or chemotherapy. Five patients had nephrectomy on one side (3 of them had metachronous presentation) and partial nephrectomy on the other side. The other children had bilateral partial nephrectomy or tumorectomy. Seven out of the nine patients are alive (78%). The two children who died presented with stage IV tumors and high grade malignant. One boy suffers cardiomyopathy. All survivors have normal renal function. With the proven efficacy of chemotherapy, bilateral renal salvage procedures were demonstrated to be effective in controlling disease without compromising renal function or survival. The innovative approaches developed for the treatment of bilateral Wilms tumor may influence the treatment of unilateral Wilms.     

Outcome of renal replacement therapy in antineutrophil cytoplasmic antibody-associated systemic vasculitis

Antineutrophil cytoplasmic antibody-associated systemic vasculitis (AASV) frequently leads to end-stage renal disease (ESRD). Potentially fatal disease activity can continue after the onset of ESRD in both dialysis and transplant patients, despite the immunosuppressive effects of uremia and rejection prophylaxis, leading to concerns that such patients have greater morbidity and mortality. To assess the outcome of AASV patients receiving renal replacement therapy, a retrospective analysis of 59 patients from our unit who received chronic dialysis, renal transplantation, or both, was performed. The survival of AASV patients with ESRD was comparable to national registry controls, as were both graft and patient survival after renal transplantation. Ther is no evidence that standard immunosuppressive protocols should be altered for AASV patients receiving renal transplants. The rate of relapse of vasculitis for patients on chronic dialysis and after transplantation was 0.09 and 0.02 per patient per year, respectively. These rates are lower than those of other series and support the contention that continued immunosuppression after ESRD, as practiced in our unit, is warranted. Relapses usually responded to cyclophosphamide and high-dose prednisolone treatment. Significantly, vasculitic flare-ups in dialysis patients were sometimes initially misdiagnosed as dialysis complications, leading to fatal delays in effective treatment. Follow-up by physicians experienced in the diagnosis and treatment of vasculitis activity should continue in these patients.