Massive autosomal dominant polycystic kidneys.
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Humans↗
Biomedical subjects
D A Goldfarb
Publications and source records attributed to D A Goldfarb.
The use of expanded criteria cadaver and live donor kidneys for transplantation.
The use of expanded criteria donors (non-traditional donors) can help lessen the current shortage of kidneys available for transplantation. The use of older donors has accounted for a large measure of the increase in the organ donation rate; however, the most significant factors found to impact on transplant success negatively traditionally have been shown to be extremes of donor age and last-hour urine output. Less significant variables affecting success rates are average systolic blood pressure, terminal serum creatinine, and days of hospitalization. With the appropriate selection of organs from expanded donors, acceptable outcomes can be obtained. When living donors are selected properly, kidneys with anatomic variants without pathologic significance can be used safely. Kidneys with a heightened potential for the development of progressive disease should not be transplanted. Efforts to decrease the cold ischemia time by increasing the use of kidneys from expanded criteria donors may improve the outcome of transplantation further. Advances in surgical techniques, preservation solutions, and methods for predicting eventual long-term renal function in kidneys from expanded donors will be critical in allowing precise selection criteria for kidneys for transplantation, resulting in the optimum use of a scarce and precious resource. Until options such as xenotransplantation become clinically feasible, the challenge will be to identify which donor organs previously considered suboptimal can be used safely to expand the organ donor pool.
Humans↗
Strategies for transplantation of cadaveric kidneys with congenital fusion anomalies.
PURPOSE: The dire shortage of cadaveric kidneys has led to a gradual expansion of donor criteria in the transplant community. The use of kidneys with anatomical fusion anomalies is uncommon and has not been well defined in the literature. We evaluated the surgical strategies and postoperative outcomes of transplanting cadaveric kidneys with congenital fusion anomalies. MATERIALS AND METHODS: Three cadaveric kidneys with congenital fusion anomalies were procured and transplanted between May 1994 and November 1999. None of the 3 donors had any significant urological history. All fusion anomalies were identified during the organ procurement process. RESULTS: Anomalies included 1 L-shaped cross-fused ectopic and 2 horseshoe kidneys. All 3 kidneys were procured en bloc. One horseshoe kidney with a narrow isthmus was split and the 2 kidneys were transplanted into separate recipients, while the other horseshoe kidney was transplanted en bloc into a single recipient. The L-shaped kidney was transplanted en bloc into 1 patient. All transplants were successful with a serum creatinine of 1.1 to 1.9 mg/dl. CONCLUSIONS: To our knowledge we present the initial case of transplantation of an L-shaped kidney. Cadaveric kidneys with congenital fusion anomalies may be transplanted successfully using various individual technical strategies based on the specific renal anatomy. As such, these kidneys may be used to maximize the increasingly inadequate donor pool.
Algorithms↗
Renal outcome 25 years after donor nephrectomy.
PURPOSE: The extended outcome after kidney donation has been a particular concern ever since the recognition of hyperfiltration injury. Few published reports have examined donor renal outcome after 20 years or greater. Kidney transplantation has been performed at the Cleveland Clinic Foundation since 1963, at which there is extensive experience with live donor transplantation. We assess the impact of donor nephrectomy on renal function, urinary protein excretion and development of hypertension postoperatively to examine whether renal deterioration occurs with followup after 20 years or greater. MATERIALS AND METHODS: From 1963 to 1975, 180 live donor nephrectomies were performed at the Cleveland Clinic. We attempted to contact all patients to request participation in our study. Those 70 patients who agreed to participate in the study were mailed a package containing a 24-hour urine container (for assessment of creatinine, and total protein and albumin), a vial for blood collection (for assessment of serum creatinine) and a medical questionnaire. All specimens were returned to and processed by the Cleveland Clinic medical laboratories. Blood pressure was taken and recorded by a local physician. A 24-hour creatinine clearance and the Cockcroft-Gault formula were used to estimate renal function, and values were compared with an age adjusted glomerular filtration rate for a solitary kidney. RESULTS: Mean patient followup was 25 years. The 24-hour urinary creatinine clearance decreased to 72% of the value before donation. For the entire study cohort serum creatinine and systolic blood pressure after donation were significantly increased compared with values before, although still in the normal range. The overall incidence of hypertension was comparable to that expected in the age matched general population. There was no gender or age difference (younger or older than 50 years) for 24-hour urinary creatinine clearance, or change in serum creatinine before or after donation. Urinary protein and albumin excretion after donation was significantly higher in males compared with females. There were 13 (19%) subjects who had a 24-hour urinary protein excretion that was greater than 0.15 gm./24 hours, 5 (7%) of whom had greater than 0.8. No gender difference was noted in blood pressure, and there were no significant changes in diastolic pressure based on gender or age. CONCLUSIONS: Overall, renal function is well preserved with a mean followup of 25 years after donor nephrectomy. Males had significantly higher protein and albumin excretion than females but no other clinically significant differences in renal function, blood pressure or proteinuria were noted between them or at age of donation. Proteinuria increases with marginal significance but appears to be of no clinical consequence in most patients. Patients with mild or borderline proteinuria before donation may represent a subgroup at particular risk for the development of significant proteinuria 20 years or greater after donation. The overall incidence of proteinuria in our study is in the range of previously reported values after donor nephrectomy.
Adult↗
Are concerns over right laparoscopic donor nephrectomy unwarranted?
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Humans↗
A randomized prospective trial of low-dose OKT3 induction therapy to prevent rejection and minimize side effects in recipients of kidney transplants.
BACKGROUND: We attempted to minimize the undesired side effects and maximize the benefit of OKT3 induction therapy in renal transplantation. METHODS: One hundred and one recipients of kidney-only transplants were randomized to three groups. Each received low-dose 2.5-mg OKT3 induction for 7-14 days, but different premedication on days 0, 1, and 2. Group I was given 250 mg i.v. methylprednisolone at 1 and 6 hr, and group II received another 500 mg at 1 hr before initial OKT3. Group III received Atgam 15 mg/kg on day 0 and began OKT3 on day 1. A CD3+ T-cell cut-off of 50/mm3 was used to guide therapy. Maintenance therapy included cyclosporine and steroids for each patient. However, groups I and II were also given mycophenolate mofetil, and group III received azathioprine as a third agent. All rejections were biopsy confirmed and Banff scored. RESULTS: No differences in demographic or transplant characteristics were noted between groups I, II, and III, and mean follow-up was 25.7 (1-38) months. There was no significant difference in actuarial patient (90%, 91%, 94%) or graft survival (83%, 88%, 84%) at 3 years between the respective groups. Mean creatinine values and infectious complications were similar for each group. No patient experienced acute rejection during induction, and eight patients required dose escalation to sustain suppression of CD3 counts. The incidence of acute rejection at 6 and 12 months was significantly (P=0.004) greater in group III (38.2, 44.1%) than in either group I (15.1, 18.1%) or group II (14.7, 17.6%); relative risk 1.988 (95% CI 1.012-3.906). Formation of anti-OKT3 antibody was significantly (P=0.006) greater in group III (26.5%) than in group I (6%) or group II (2.9%). Group I recipients enjoyed significantly (P=0.001) fewer (2.17) OKT3 side effects on days 0, 1, and 2 than group II (3.03) or group III (2.49), and contained the largest number (61%) of recipients who experienced no side effects. Group I also exhibited the most suppressed profile of OKT3-induced release of tumor necrosis factor-alpha (P=0.006), interferon-gamma (P=NS), and interleukin-6 (P=0.01) on days 0 and 1. CONCLUSIONS: Low-dose 2.5-mg OKT3 with pretreatment of split-dose steroids on days 0, 1, and 2 provides the most effective method for OKT3 induction, which minimizes side effects for most patients. Subsequent maintenance therapy with cyclosporine, mycophenolate mofetil, and steroids provides effective rejection prophylaxis without increased complications for up to 3 years. Predepletion of T cells before exposure to OKT3 does not prevent cytokine release.
Acute Disease↗
Laparoscopic lymphocelectomy: a multi-institutional analysis.
PURPOSE: Because symptomatic lymphoceles are infrequent, single center studies generally report small numbers of patients. We report a multi-institutional experience with and long-term outcome following laparoscopic lymphocelectomy in 81 patients. MATERIALS AND METHODS: Data were obtained from 9 institutions at which at least 5 cases of laparoscopic lymphocelectomy had been performed. Baseline patient demographics, operative time and blood loss, special operative adjunct techniques, postoperative course, convalescence, complications and lymphocele recurrence data were collected and analyzed. RESULTS: A total of 56 men and 25 women with a mean age of 41 years were included in the study. Lymphocele formed after renal transplantation in 78 patients (96%) and after pelvic lymph node dissection in 3 (4%). Average operating time was 123 minutes with a mean blood loss of 43 ml. Omentopexy was performed in 11 cases (13.6%). No intraoperative stenting of the transplant ureter was performed. Intraoperative complications consisted of laryngospasm, bladder injury, inferior epigastric artery injury and mild renal capsule hematoma in 1 patient each. Conversion to open surgery was required for repair of bladder injury in 1, repair of preexisting hernia in 1, unusually thickened lymphocele wall in 1 and inaccessible lymphocele location in 4 cases. Mean time to ambulation and resumption of regular diet was 1 day, and mean hospital stay was 1.5 days. Postoperative complications included trocar site hernia in 1 and urinary retention in 2. Convalescence averaged 2.5 weeks. During a mean followup of 27 months 5 patients (6%) had lymphocele recurrence. CONCLUSIONS: Laparoscopic lymphocelectomy is safe, minimally invasive and effective. It is an excellent alternative to the conventional open surgical approach.
Adult↗
Significance of serum creatinine pattern and area under the creatinine versus time curve during the first acute renal transplant rejection.
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Adult↗
U-stitch ureteroneocystostomy: a new renal transplantation ureteral reimplantation technique associated with reduced urologic complications.
Urologic complications represent the most frequent complications following renal transplantation and are associated with significant morbidity. We present the results of the first 105 patients who underwent ureteroneocystostomy at our institution using a new surgical ureteral reimplantation technique designed to reduce the incidence of urologic complications after renal transplantation.
Adolescent↗
Laparoscopic retroperitoneal live donor right nephrectomy for purposes of allotransplantation and autotransplantation.
PURPOSE: We report the technique of and initial experience with retroperitoneal laparoscopic live donor right nephrectomy for purposes of renal allotransplantation and autotransplantation. MATERIALS AND METHODS: A total of 5 patients underwent retroperitoneoscopic live donor nephrectomy of the right kidney for autotransplantation in 4 and living related renal donation in 1. Indications for autotransplantation included a large proximal ureteral tumor, a long distal ureteral stricture and 2 cases of the loin pain hematuria syndrome. In all cases a 3-port retroperitoneal laparoscopic approach and a pelvic muscle splitting Gibson incision for kidney extraction were used. In patients undergoing autotransplantation the same incision was used for subsequent transplantation. RESULTS: All procedures were successfully accomplished without technical or surgical complications. Total mean operating time was 5.8 hours and average laparoscopic donor nephrectomy time was 3.1 hours. Mean renal warm ischemia time, including endoscopic cross clamping of the renal artery to ex vivo cold perfusion, was 4 minutes. Average blood loss for the entire procedure was 400 cc. Radionuclide scan on postoperative day 1 confirmed good blood flow and function in all transplanted kidneys. Mean analgesic requirement was 58 mg. fentanyl. Mean hospital stay was 4 days (range 2 to 8), and convalescence was completed in 3 to 4 weeks. CONCLUSIONS: In the occasional patient requiring renal autotransplantation live donor nephrectomy can be performed laparoscopically with renal extraction and subsequent transplantation through a single standard extraperitoneal Gibson incision, thus, minimizing the overall operative morbidity. Furthermore, these data demonstrate that live donor nephrectomy of the right kidney can be performed safely using a retroperitoneal approach with an adequate length of the right renal vein obtained for allotransplantation or autotransplantation.
Adult↗
Quantitative assessment of the first acute rejection as a predictor of renal transplant outcome.
BACKGROUND: Acute rejection (AR) of the transplanted kidney has been identified as the major risk factor for the development of chronic rejection and immunological graft loss. However, the clinical presentation and response to AR therapy can vary considerably between recipients. METHODS: We studied the first AR episode in 201 kidney-only recipients transplanted between January 1987 and June 1998 who were biopsied between April 1993 and June 1998 and were graded using the Banff schema. All patients received cyclosporine-based immunosuppression. There were 134 cadaver donor (66.7%) and 67 live donor (33.3%) recipients followed for a mean of 46.2 (range 4-128) months. All Banff grade 1-3 and 40/78 borderline (BL) cases were treated for rejection after biopsy. These patients were compared with a contemporaneous control population who did not experience AR. Demographic risk factors associated with graft loss were identified in both univariate and multivariate analysis. Daily (0-18) serum creatinine (SCr) values during and after the AR were plotted for each patient to generate curves and calculate area under the serum creatinine versus time curve (mg/dl/day). Four response patterns to treatment were identified according to the velocity of % increase (V1) and decrease (V2) of serum creatinine. These were identified as rapid rise and fall (n=62); rapid rise and slow fall (n=43); slow rise and fall (n=55); and slow rise and rapid fall (n=41). Kaplan-Meier graft survivals were compared between the groups. RESULTS: Any Banff grade was associated with increased risk for graft loss (P=0.0001). However, no significant differences were observed between the Banff BL and B1-3 groups, or among those BL patients who were treated or remained untreated for AR. Multivariate analysis identified a black recipient (P=0.03, risk ratio 2.0) and area under the serum creatinine versus time curve (P=0.0001, risk ratio 3.2) as significant risk factors for graft loss. The AR response pattern RS resulted in a significantly (P=0.0072) diminished 5-year graft survival (45%) compared with the other groups. Serum creatinine pattern, but not Banff grade, was also a significant (P=0.025) predictor of re-rejection. CONCLUSIONS: These data suggest that all Banff grades, including BL, carry a significant risk for graft loss, and the initial response to antirejection therapy can predict long-term graft outcome. They support the practice of treating AR promptly and definitively and suggest that the RS subgroup of rejecting grafts could be targeted for additional antirejection therapy. This subgroup can be identified by 10 days of AR therapy, and should be the subject of further study.
Acute Disease↗
Contemporary evaluation and management of Cushing's syndrome.
Cushing's syndrome, characterized by unregulated cortisol secretion, may be caused by a variety of adrenal, pituitary, or other tumors. The best biochemical test for establishing the diagnosis is determination of 24-h urinary free cortisol. The specific causes for Cushing's syndrome may be further differentiated by plasma adrenocorticotrophic hormone (ACTH). Primary adrenal cortical diseases are associated with low levels of ACTH and are considered ACTH-independent. Pituitary disease and the ectopic ACTH syndrome are associated with normal or elevated ACTH levels and are considered ACTH-dependent. Adrenal forms of Cushing's syndrome may result from either adenoma or carcinoma. The diagnostic approach to Cushing's syndrome and the clinical, biochemical, and radiographic features that distinguish adrenal adenoma and carcinoma are the subjects of this paper. 65-75% of CS [14, 15]. Most cases of Cushing's disease are the result of pituitary adenomas; however, corticotrope hyperplasia is responsible for a small minority of cases. Ectopic production of ACTH from a variety of tumors (bronchial carciniod, thymoma, oat-cell carcinoma, pheochromocytoma, islet-cell tumor, and prostate cancer) accounts for 10-15% of CS. Primary adrenocortical diseases account for the remaining 20-30% of CS, including benign adenoma (10-15%), adrenocortical carcinoma (5-10%), and adenomatous hyperplasia (5%). The purpose of this review is to present a contemporary approach to the evaluation and management of patients with Cushing's syndrome, emphasizing the primary adrenal etiologies pertinent to urologists.
Cushing Syndrome↗
Acute colonic pseudo-obstruction (Ogilvie's syndrome) after renal transplantation.
BACKGROUND: Acute colonic pseudo-obstruction (Ogilvie's syndrome) in the immunosuppressed patient is associated with increased morbidity and mortality. Renal transplant recipients possess several comorbidities that increase the risk of acute pseudo-obstruction of the colon. The aims of this study were to present our experience with this syndrome and to evaluate the potentiating factors in these patients. A review of the literature for pseudo-obstruction following renal transplantation is presented. METHODS: Seven patients who developed Ogilvie's syndrome were identified in a retrospective review of 550 kidney-only transplants. Pretransplant data, potential risk factors, presentation, management, and outcome details were retrieved. The medical literature was reviewed using Medline. RESULTS: Seventy-eight patients with Ogilvie's syndrome in the early posttransplant period have been reported. The associated morbidity and mortality was heightened in this immunocompromised population. Obese transplant recipients (body mass index >30 kg/m2) were at significantly increased risk for developing this syndrome. CONCLUSION: A broad armamentarium of treatment options is available, but the key to successful resolution lies in early recognition.
Colonic Pseudo-Obstruction↗
Artificial urinary sphincter insertion in renal transplant recipients.
The prosthetic urinary sphincter has contributed significantly to the improved management of urinary incontinence during the past 25 years. However, the safety of these devices in immunosuppressed patients is not well reported. We describe the successful insertion of the AMS 800 artificial urinary sphincter in two renal transplant recipients.
Adult↗
Successful outcomes in pheochromocytoma surgery in the modern era.
PURPOSE: We describe our experience with surgical management, complications and treatment outcome of histologically confirmed pheochromocytoma. MATERIALS AND METHODS: The records of 113 patients who underwent surgical excision of pheochromocytoma were reviewed and assessed for preoperative medical treatment, intraoperative findings, postoperative hospitalization and complications. RESULTS: There were no surgical mortalities. Average length of stay in the intensive care unit was 1.2 days. There were only 6 major cardiovascular complications all of which occurred in patients who received preoperative medications, including 5 with alpha blockade. Patients receiving no preoperative alpha blockade required an average of 956 cc less in total intraoperative fluids, which approached statistical significance, and 479 cc less fluids on postoperative day 1, which was statistically significant. CONCLUSIONS: Preoperative alpha-adrenergic blockade is not essential in pheochromocytoma patients. Calcium channel blockers are just as effective and safer when used as the primary mode of antihypertensive therapy. Surgery for pheochromocytoma is safe in the modern era.
Adolescent↗
A randomized prospective controlled trial of oral acyclovir versus oral ganciclovir for cytomegalovirus prophylaxis in high-risk kidney transplant recipients.
BACKGROUND: Posttransplantation cytomegalovirus (CMV) infection remains a significant cause of morbidity in kidney transplant recipients. We performed a randomized prospective controlled trial of oral acyclovir versus oral ganciclovir for CMV prophylaxis in a group of renal allograft recipients considered at high risk for CMV disease due to the use of OKT3 induction therapy. METHODS: A total of 101 recipients of cadaveric (83) and zero haplotype-matched live donor (18) kidney transplants were entered into the trial. A total of 22 D-R- patients received no prophylaxis. Twenty-seven D+R-, 29 D+R+, and 23 D-R+ patients were randomized to receive 3 months of either oral acyclovir (800 mg q.i.d.) or oral ganciclovir (1000 mg t.i.d.). Doses were adjusted according to the level of renal function. The D+R- patients were also given CMV immune globulin biweekly for 16 weeks. Surveillance blood cultures were obtained at transplantation, at months 1, 2, 3, and 6, and when clinically indicated. The primary study end points were time to CMV infection and disease the first 6 months after transplantation. RESULTS: The mean follow up was 14.4 months. Both agents were well tolerated, and no drug interruptions for toxicity occurred. CMV was isolated in 14 of 39 (35.9%) acyclovir-treated and 1 of 40 (2.5%) ganciclovir-treated recipients by 6 months (P=0.0001). Symptomatic CMV disease occurred in 9 of 14 (64%) of the acyclovir patients, two with tissue-invasive disease. Infection rates for acyclovir vs. ganciclovir, respectively, stratified by CMV serology were: D+R-, 54 vs. 0%, P=0.0008; D+R+, 43 vs. 6.6%, P=0.01; D-R+, 8.3 vs. 0%, P=NS. No patient developed CMV infection while taking oral ganciclovir, however three delayed infections occurred 2-7 months after finishing therapy. Each patient had been previously treated for acute rejection. CONCLUSIONS: Oral acyclovir provides effective CMV prophylaxis only for recipients of seronegative donor kidneys. Oral ganciclovir is a superior agent providing effective CMV prophylaxis for recipients of seropositive donor kidneys. Recipients who are treated for acute rejection are at risk for delayed CMV infection during the first posttransplantation year.
Acute Disease↗
Transplantation of pediatric en bloc cadaver kidneys into adult recipients.
BACKGROUND: To maximize the renal donor pool, cadaveric pediatric en bloc kidneys have been transplanted as a dual unit by some transplant centers. We compared the short- and long-term outcomes of adult recipients of cadaveric pediatric en bloc renal transplants versus those of matched recipients of cadaveric adult kidneys. METHODS: Thirty-three adults who received pediatric en bloc kidney transplants between April 1990 and September 1997 were retrospectively identified and were compared with 33 matched adults who received adult cadaveric kidney transplants. The groups were identical for transplantation era, immunosuppression, recipient sex, race, cause of renal failure, mean weight, and follow-up duration (37.8 vs. 37.5 months). The mean recipient age study versus control was lower (36.3 vs. 48.9 years, P=0.0003). Results. There was no difference between the en bloc and adult donor groups in the 3-year patient survival rates (95% vs. 87%, P=0.16) or the 3-year graft survival rates (87.3% vs. 84.2%, P=0.35). Further, there was no difference in en bloc patient or en bloc graft survival time stratified by recipient age (14-44 vs. >45 years, P=0.11), en bloc donor age (<24 vs. >24 months, P=0.39), or recipient weight (<60, 61-75, >75 kg; P=0.60). Differences in serum creatinine (mg/dl) for the en bloc versus the control group at the time of discharge (3.0 vs. 7.8 mg/dl, P=0.06), at 1 year (1.4 vs. 2.0 mg/dl, P=0.06), and at 2 years (1.1 vs. 1.6 mg/dl, P=0.14) had dissipated by the time of the 5-year follow-up examination (1.1 vs. 1.6 mg/dl, P=0.14). Vascular complications were more prevalent in the en bloc group: renal vein thrombosis (one case), thrombosis of donor aorta (two cases), arterial thrombosis of one renal moiety (two cases), and renal artery stenosis (two cases). There were no differences between groups in delayed graft function, acute or chronic rejection, posttransplant hypertension, posttransplant protein-uria, or long-term graft function. CONCLUSIONS: Collectively, these data indicate that transplanting pediatric en bloc kidneys into adult recipients results in equivalent patient and graft survival compared with adult cadaveric kidneys. Further, the data also suggest that pediatric en bloc kidneys need not be strictly allocated based on recipient weight or age criteria.
Adolescent↗
Renal and adrenal surgery during pregnancy.
Retroperitoneal surgery during pregnancy is rarely indicated. Major considerations are the optimal timing of surgery and the consequences of nonoperative management on mother and fetus. We report on the first partial nephrectomy performed during pregnancy and one nephroadrenalectomy. When indicated, the second trimester is the ideal time to perform nonobstetric surgery during gestation.
Adrenalectomy↗