A kidney transplant recipient with renal cell carcinoma derived from a native kidney

A case of renal cell adenocarcinoma which originated from a left native kidney following kidney transplantation, with widespread metastases, including multiple bone and liver metastases, is presented herein. An extensive clinical investigation, including bone marrow biopsy, liver biopsy abdominal computerized tomography, excretory urography and examination of the gastiointestinal tract failed to determine the site of the primary lesion in this patient. Examination at autopsy revealed a small adenocarcinoma of the left native kidney with lobulated nodular capsular invasion and metastases to the bone and liver.     

Renal cell carcinoma of native kidney in renal transplant recipients

OBJECTIVE: To evaluate the prevalence, prognosis and possible risk factors of renal cell carcinoma (RCC) of the native kidney in renal transplant recipients. PATIENTS AND METHODS: We retrospectively re-examined the follow-up data of 373 consecutive renal transplant recipients at our institution between August 1993 and September 2004. We collected the data of all de novo RCC of the native kidney in the current analysis. RESULTS: Of the 373 patients examined, 12 tumours of the native kidney were diagnosed in 10 individuals. The mean ages at transplantation and diagnosis were 33 and 45.8 years, respectively. Thirteen malignancies were discovered fortuitously. Among the renal ultrasonograms there were two false-negative results. The mean tumour size was 21 mm. Nephrectomy was performed in all cases. Among the 12 kidney malignancies, there were five conventional RCCs and seven papillary RCCs. Half of all tumours were Furhman Grade 3 lesions, and pT1aN0M0 tumours also accounted for all malignancies in the current cohort. One of the 10 patients died, from progression of metastases 6 years after diagnosis. One patient had a local recurrence 2 years after diagnosis. The other eight patients were alive with no evidence of disease at the time of the current report. No significant relationship was detected between RCC occurrence and clinical patient characteristics. CONCLUSIONS: There appears to be a greater risk of RCC of the native kidney in patients with end-stage renal disease. The present results suggest that an annual examination of the native kidney before and after renal transplantation is essential.     

Our experience in eight cases with urinary hydatid disease: A series of 372 cases held in nine different clinics

OBJECTIVES: Hydatid disease, a parasitic infestation caused by the larval stage of the cestode Echinococcus granulosus, is diagnosed commonly in the east and south-east regions of Turkey. The aim of this study is to emphasize the relatively frequent occurrences of echinococcosis in our region, and to discuss therapeutic options and treatment results according to current literature. METHODS: A retrospective 10-year review of nine different clinics' records of the Research Hospital of the Medical School of Yüzüncü Yil University revealed 372 hydatid disease cases that were localized in various organs and treated surgically (271 cases) or drained percutaneously (99 cases). Hydatid disease was diagnosed by ultrasonography (US) and computed tomography scans (CT) and confirmed histopathologically. RESULTS: The involved organ was lung in 203 cases (131 adults, 72 children), liver in 150, spleen in 9, brain in 2, kidneys in 7 cases and the retrovesical area in 1 case. The urogenital system is involved at a rate of 2.15%. Two hundred and seventy-one cases were treated surgically and 99 percutaneously. Two cases with renal hydatid cyst refused the surgical procedure (one had a solitary kidney with hydatid cyst). Albendazole was administered to 192 patients; 93 patients had open surgical procedure and 99 patients underwent percutaneous procedure. Cysts were excised totally in the open surgical procedure; however, involved kidneys were removed totally (four cases) except one. Cystectomy and omentoplasty was performed in one case. Complications were as follows: in six cases, cystic material was spilled into the bronchial cavity during the dissection and a renal hydatid cyst ruptured and spilled retroperitoneally. CONCLUSION: Hydatid disease is a serious health problem in Turkey. The mainly affected organs are liver and lung. It can be treated surgical or by percutaneous aspiration.     

Unilateral renal cell carcinoma with coexistent renal disease: a rare cause of end-stage renal disease.

BACKGROUND: Renal cell carcinoma (RCC) is a disorder encompassing a wide spectrum of pathological renal lesions. Coexistence of unilateral RCC and associated pathology in the contralateral kidney is an unusual and challenging therapeutic dilemma that can result in renal failure. So far, data on unilateral RCC with chronic renal failure necessitating renal replacement therapy have not been published. The aim of the present study was to evaluate the incidence of end-stage renal disease (ESRD) from unilateral RCC, and to assess the associated pathology and possible pathogenic factors. METHODS: In 1999, a survey of the 350 patients treated by chronic dialysis in Asturias, Spain, was carried out to identify and collect clinical information on patients with primary unilateral RCC whilst on their renal replacement programme. RESULTS: Seven patients were identified as having ESRD and unilateral RCC, giving an incidence of 2% of patients treated by dialysis. There was a wide spectrum of associated disease and clinical presentation. All patients underwent radical or partial nephrectomy and were free of recurrence 6--64 months after surgery. Six patients were alive and free of malignancy recurrence for 6--30 months after the onset of haemodialysis. CONCLUSION: ESRD is rare in association with unilateral RCC, but does contribute to significant morbidity. However, the data presented here are encouraging and suggest that cancer-free survival with renal replacement therapy can be achieved in such patients.     

Postoperative recurrence in hepatic hydatid disease

Recurrence of hepatic hydatid disease is still a serious problem in endemic areas like our country. In this study, we present the causes and management of recurrences after surgical therapy of the hepatic hydatid cysts. Hepatic hydatid cyst patients treated surgically and followed afterward at Istanbul University, Cerrahpasa Medical Faculty, Department of General Surgery between January 1998 and January 2003 were evaluated retrospectively. During this period, 172 primary patients with hepatic hydatid disease were attended to at our clinic. Morbidity and mortality rates for this series were 5.8% and 0.58%, respectively. Recurrence rate was 4.65% during the follow-up period of 60.5 months (range, 25-84 months). Primary causes of recurrence were thought to be unnoticed cysts with exophytic development due to inadequate incision and exposition and spreading of the disease during conservative operative interventions. It is concluded that selection of the proper incision allowing complete exposition, and performance of pericystectomy in solitary, peripherally located cysts prevent recurrence.     

Management of hydatid disease of the liver—10 years'' experience in a non-endemic area

The incidence of hydatid disease of the liver caused byEchinococcus granulosus in Austria, a nonendemic region in central Europe, is documented over a 10-year period. Since 1984, 28 patients (24 women, 4 men) with hydatid disease of the liver have undergone surgery in our department. A definitive diagnosis was established by combining serological tests (an enzyme-linked immunosorbent assay withEchinococcus multilocularis antigen and an indirect hemagglutination assay withEchinococcus granulosus antigen) and abdominal imaging (computed tomography scan and ultrasonography). Twenty-four patients (85.7%) had uncomplicated echinococcosis; in most of them (16) total closed pericystectomy was done. Four patients (14.3%) had complicated echinococcosis, suffering from intrabiliary rupture and spontaneous perforation into the peritoneal cavity, and in two of these patients (7.1%) pyogenic transformation, one with abscess perforation, occurred. Postoperative complications were significantly more common in patients with complicated echinococcosis and included two bile leaks (necessitating prolonged tube drainage), pleural effusion, atelectasis, and wound infection. One recurrence of hydatid infection due to previous spontaneous perforation required reoperation 4 years after the primary event. The low recurrence rate of uncomplicated echinococcosis (0%) over a mean period of 5.6 years and of 3.6% when peritoneal echinococcosis was included may be the result of the radical surgical technique employed.     

Lupus nephritis in a pediatric renal transplant recipient

A case of aggressive lupus nephritis in a pediatric renal transplant patient is described. She initially presented with end-stage glomerulonephritis for which an underlying etiology could not be determined. Ten months after cadaveric renal transplantation, systemic lupus erythematosus was diagnosed, when she developed diffuse proliferative glomerulonephritis in association with antinuclear antibody, anti-double-stranded DNA antibody and extrarenal manifestations of lupus. It is plausible that she developed recurrent rather thande novo lupus nephritis following transplantation. Reactivation of lupus nephritis in a renal transplant is unusual in adults, and is previously unreported in children.     

Sexual and reproductive function in end-stage renal disease and effect of kidney transplantation

Advanced chronic kidney disease is associated with impaired spermatogenesis and testicular damage. Semen analysis typically shows a decreased volume of ejaculate, oligo-or complete azoospermia, and a low percentage of motile sperm. Erectile dysfunction （ED） is also common in patients with chronic renal failure （CRF） and is observed in excess of 50% of these patients. There have been ongoing improvements in survival and quality of life after renal transplantation. One of the most impressive aspects of successful renal transplantation in the young people is the ability of the male patient to father a child. In this article we first review pathophysiology of reproductive failure in end-stage renal disease （ESRD）, then ED in ESRD and its management are discussed, finally sexual function in renal transplant patients and management of ED in these patients are reviewed.     

Anderson–Fabry disease: a case-finding study among male kidney transplant recipients in Austria

The diagnosis of Anderson–Fabry disease is often delayed or even missed. As severe renal manifestations are a hallmark of alfa-galactosidase A (AGAL) deficiency, we tested the hypothesis that Anderson–Fabry disease is under-recognized among male kidney transplant recipients. This nation-wide study in Austria enrolled 1306 patients (ca 65% of all kidney transplanted males) from 30 kidney centers. AGAL activity was determined from filter paper dried blood spots by a fluorescence assay. A positive screening test was defined by an AGAL activity below 1.5 nmol/h/ml. In patients with a positive blood spot-screening test, AGAL activity was re-examined in peripheral blood leukocytes. Genetic testing for mutations in the GLA gene was performed by sequencing to confirm the diagnosis of Anderson–Fabry disease. Two previously not recognized cases with Anderson–Fabry disease were identified. Our study is the first showing that a diagnosis of Anderson–Fabry disease can be missed even in patients who undergo kidney transplantation. Case-finding strategies may be considered a useful tool for diagnosis of this rare disease that may be somewhat more prevalent among kidney transplant recipients compared with dialysis populations.     

End-stage disease of native kidneys in a patient with biopsy-proven Arndt-Gottron scleromyxoedema and recurrence in the transplanted kidney.

End-stage renal disease-stage 5 chronic kidney disease (CKD)-of the native kidneys, related to biopsy-proven Arndt-Gottron scleromyxoedema, developed in a male patient. From 1998 until 2001, the patient was treated by haemodialysis. In June 2001, cadaveric kidney transplantation was performed. In January 2004, a kidney biopsy was performed because of deteriorating renal function revealing relapse of scleromyxoedema with typical concentric narrowing of the arterioles due to accumulation of mucopolysaccharides with severe glomerular ischaemia. Arndt-Gottron scleromyxoedema is an as yet unsuspected cause of stage 5 CKD of the native kidneys. Moreover, the disease can relapse in the transplanted kidney, again leading to intractable transplant stage 5 CKD.     

Impact of kidney transplantation on sleep apnoea in patients with end-stage renal disease.

BACKGROUND: Sleep apnoea is common in patients with end-stage renal disease. Although individual case reports have described an improvement in sleep apnoea following kidney transplantation, there have been no longitudinal studies of a case series to determine what proportion of patients with sleep apnoea improve. METHODS: Dialysis-dependent patients awaiting kidney transplantation and pre-dialysis patients with an identified living donor kidney had overnight polysomnography, which was repeated several months after successful kidney transplantation. Patients were divided into apnoeic and non-apnoeic groups based on an apneoa-hypopnoea index (AHI) > 10/h during pre-transplant polysomnography and, following transplantation, apnoeic patients were further divided into responders and non-responders based on >50% reduction in AHI and/or AHI < 10/h. RESULTS: Eighteen patients (11 men, 7 women), aged 27-65, were studied. Pre-transplant sleep apnoea was present in 11 of 18 (61%) patients. Although transplantation was associated with a significant reduction in blood urea nitrogen and serum creatinine, there were no significant changes in AHI (pre vs post: 20.2 +/- 15.1 vs 23.5 +/- 21.3). Among the 11 apnoeic patients, only three met the criteria for a significant improvement ('responder'). There were no patient characteristics, sleep apnoea indices or renal function changes that distinguished responders from non-responders. CONCLUSIONS: Sleep apnoea improves in a minority of patients with end-stage renal disease following successful kidney transplantation. Specific determinants of improvement were not identified.     

Diabetes and coronary artery disease impose similar cardiovascular morbidity and mortality on renal transplant candidates.

BACKGROUND: In renal transplant candidates (RTC), diabetes and coronary artery disease (CAD) are commonly observed. However, whether diabetes imparts a cardiovascular risk equivalent to that of CAD and whether CAD adds to the cardiovascular risk associated with diabetes is unknown. METHODS: To assess the interplay between diabetes and CAD as a determinant of major adverse cardiovascular events (MACE), 288 high-risk RTC (56.4+/-8.1 years old, 72% males) underwent a comprehensive cardiovascular evaluation including coronary angiography. Patients were divided into four groups based on the diagnoses of diabetes and CAD (>70% narrowing), and followed up for 1-60 months (median, 17). The primary endpoint was the composite incidence of fatal/non-fatal MACE. RESULTS: During follow-up, 80 MACE occurred. Patients with diabetes (P=0.03) or CAD (P<0.0001) had a worse long-term prognosis. However, only in patients without diabetes was CAD associated with an increased incidence of MACE (10.6% vs 45.9%, P<0.0001). In patients with diabetes, the endpoints were not different between those with and without CAD. No difference occurred in the long-term prognosis of patients with diabetes (with or without CAD) and patients without diabetes with CAD. CONCLUSIONS: We concluded that in high-risk RTC, diabetes confers a cardiovascular risk comparable to that of CAD in patients without diabetes, independent of coronary obstruction. In patients with diabetes, concomitant CAD does not add to the already very high cardiovascular risk of this population.     

Cardiac involvement of hydatid disease

Objectives|Echinococcosis is a serious health issue occurring in some geographical region of the world. Cardiac involvement is rare and early diagnosis and prompt surgical intervention are critical.Subjects and Methods: Six patients with cardiac hydatid cysts underwent surgical treatment in our institution between April, 1996 and March, 2002. Five of the patients were female and one was male. Average age was 40±5 years with a range of 19 to 72 years. Cysts were located in the right ventricular outflow tract in two patients, the left ventricular outflow tract in one, the right atrial in one, the right ventricular in one and the right atrioventricular groove in one. Five patients were operated on using standard cardiopulmonary bypass techniques, and one was operated on without cardiopulmonary bypass.Results: In the perioperative and the early postoperative period, no cardiac problems was observed. On control echocardiography, a ventricular septal defect was detected in one patient in the late postoperative period. The ventricular septal defect was repaired using standard cardiopulmonary bypass and was closed with a teflon patch. Patients were followed up for a mean period of 3.4±2.5 years. No mortality or recurrence was observed during the follow-up period.Conclusions: When hydatid cyst is diagnosed, the possibility of cardiac involvement should also be investigated. The treatment of cardiac hydatid cyst is surgical extraction of the cyst.     

脊柱包虫病的诊断与治疗

目的:探讨脊柱包虫病的诊断及治疗方法。方法:1990年10月～2010年10月手术治疗脊柱包虫病患者9例,病程为3个月～12年。临床表现为午后低热、腰背疼痛和腰背部包块,术前脊髓神经功能Frankel分级A级1例,B级2例,C级2例,D级4例。包虫病8项免疫试验均为阳性。术前均行影像学检查,病变单纯累及胸椎2例(T8、T9 1例,T11、T12 1例),腰椎2例(L3 1例,L1、L2、L3 1例),胸腰段4例(T12、L1 2例,T12、L1、L2 2例),骶椎1例。X线片、CT检查误诊为椎体结核5例、转移瘤2例、脊索瘤1例、腰大肌脓肿1例。MRI检查7例诊断为脊柱包虫病;2例病变单纯累及腰椎者缺乏囊中囊典型信号改变,误诊为脊柱转移瘤。均行椎管减压病灶清除植骨内固定术,其中病变单纯累及胸椎的2例与骶骨1例行后路全椎板切除减压,2例腰椎病变者行椎板间开窗椎管减压,4例胸腰段病变者行脊柱侧前方入路经横突椎管减压。7例术前诊断脊柱包虫病者术前口服抗包虫药阿苯达唑2～3周,所有患者术后继续服用阿苯达唑3个月。结果:手术时间210～330min,平均260min;术中失血量170～470ml,平均300ml。7例术前MRI检查诊断为脊柱包虫病者术后病理诊断为脊柱细粒棘球蚴病,2例术前MRI误诊为脊柱转移瘤者术后病理诊断为腰椎泡状棘球蚴病。随访1～10年,平均4.6年。1例腰椎泡状棘球蚴病患者术后8个月植骨未愈合,经理疗、促骨生长药物治疗,1年后复查X线片显示骨性愈合;其余8例患者植骨区骨性融合,愈合时间为5～8.5个月,平均7个月。末次随访患者脊髓神经功能Frankel分级改善6例,无变化3例。随访期间无复发。结论:MRI检查对脊柱细粒棘球蚴病有诊断价值,对腰椎泡状棘球蚴病易误诊,后者的确诊有赖于病理检查;脊柱包虫病在药物治疗的基础上采用手术治疗可取得较好疗效。     

Improving survival in the very young renal transplant recipient

Sixty-one patients ranging in age from 6 weeks to 30 months have undergone renal transplantation over a 19-year period. The overall survival is 80% with a 90% survival in those patients transplanted since 1980 (41 patients). Seventy-five percent of the original allografts have survived with 83% of the last 41 allografts surviving. The unique challenges presented by the very young renal transplant recipient are outlined along with those aspects of management felt to be most important in ensuring a successful outcome.     

Routine ultrasonography surveillance of native kidneys for renal cell carcinoma in kidney transplant candidates

Renal cell carcinoma (RCC) has a high incidence in the kidney transplant population and annual surveillance detects these tumors early in their natural history. Minimal guidelines exist regarding RCC surveillance in ESRD patients awaiting transplant. A retrospective review of our kidney transplant database examined the outcomes of annual ultrasonographic surveillance during initial kidney transplant evaluation and upon annual reassessment. Of 2642 patients listed for transplant, 145 patients were found to have masses during initial kidney transplant evaluation or annual imaging consistent with new complex cystic disease or RCC. A total of 71 patients had RCC identified, with 52 found on initial kidney transplant evaluation and 19 identified on annual surveillance. Male gender and African‐American race were independently associated with RCC (P<.05). RCC was detected a median of 2.0 years after listing (two annual ultrasonography studies). Patients with complex cysts were more likely to undergo transplantation (48.7%) compared to patients with RCC (21.1%; P<.001). There was no significant difference in survival between RCC patients and those found to have complex cystic disease, suggesting incidental RCC can be diagnosed early in the natural history and at a curable stage through implementation of a biennial surveillance program.     

Dissecting aortic aneurysm in a renal transplant recipient

There have been few reports of surgical repair of acute aortic dissection in renal transplant recipients. The incidence, operative risk, or perioperative management of aortic dissection with functioning allografts remains unknown. Herein we report our experience in successful treatment of type I dissecting aortic aneurysm in a renal transplant patient. A 35-year-old man was admitted to our hospital complaining of severe chest pain. He had undergone a living renal transplant from his mother for chronic renal failure caused by immunoglobulin A nephropathy 11 years prior to admission. An immunosuppressive regimen had been maintained continuously. Preoperative chest computed tomography demonstrated a thoracic dissecting aortic aneurysm (DeBakey classification type I). An emergent graft replacement for the ascending aorta was placed under circulatory arrest. Although continuous hemodiafiltration was required postoperatively because of deteriorated renal function, he recovered uneventfully and his renal function returned to preoperative values. He was discharged on postoperative day 26 without any complications.