Acquired cystic disease of the kidneys in chronic hemodialyzed and renal transplant patients

In this study, the appearance of renal cysts in 43 chronic hemodialyzed patients (mean duration of dialysis treatment: 26.3 months) was investigated by sonography with a high resolution (3.5 MHz) sector scanner. In an investigation of the patients' own kidneys, 23 renal transplant patients (mean observation time: 51.3 months after transplantation; mean dialysis treatment before transplantation: 22.8 months) were also studied by sonography. Cysts could be demonstrated in 21 of 43 (49%) patients on maintenance hemodialysis. In 10 of these patients a previous investigation at the beginning of dialysis did not demonstrate any cysts. The diameter of the cysts varied between 5 and 30 mm. With regard to the duration of dialysis, cysts could be demonstrated by sonography in 9 of 23 (39%) dialyzed patients, with a maximum dialysis duration of 2 years, and in 12 of 20 (60%) patients who had been dialyzed for more than 2 years. The development of acquired cystic disease of the kidneys in dialysis patients seems to be promoted by the longer survival of uremic patients. Cysts could be found in the patients' own kidneys in only 4 of 23 (17%) renal transplant patients. The difference in the demonstration of cysts between patients on maintenance hemodialysis and renal transplant patients was statistically significant. This suggests that cystic transformation may possibly be a reversible process.     

Ultrasonographic study on kidneys in patients with chronic renal failure. Part I. Ultrasonic measurement of renal size and analysis of renal ultrasonotomograms

Ultrasonograms of 546 kidneys were obtained in 280 patients undergoing chronic dialysis. Dialysed kidneys could be detected in 529 of the 546 kidneys (96.9%) by ultrasonic examination. The ultrasonic diagnoses on dialysed kidneys were contracted kidney in 313 kidneys (59.2%) and acquired cystic disease of the kidney in 107 kidneys (20.2%). Ultrasonic measurement of the size of kidney (length and thickness) revealed that the kidneys in patients with chronic renal failure were much smaller than normal ones. But the kidneys in patients undergoing dialysis for more than 8 years gradually increased in size with incidence of acquired renal cysts. The kidneys in patients with diabetic nephropathy were greater in length and thickness than those with chronic glomerulonephritis. Sonographic features of dialysed kidneys were unclear renal imaging, unidentified central echoes, cortico-medulla + border and increased parenchymal echogenicity. Irregularity of the renal contour had a tendency to increase in number with incidence of cysts in long-term dialysis patients. The ultrasonograms of the kidneys with diabetic nephropathy showed fewer changes than normal ones. No major complication of the kidney was detected in the present study. However, two retroperitoneal hematomas and one renal cell carcinoma developed within two years after this examination. We believe that regular screening of the kidneys by ultrasonic examination is mandatory in patients on chronic dialysis for early diagnosis and treatment of these complications.     

Ultrasonographic study on kidneys in patients with acute renal failure]

Ultrasonotomograms of 22 kidneys were obtained in 11 patients with renal-acute renal failure (renal-ARF). The underlying diseases of renal-ARF were acute tubular necrosis in 8 patients and acute on-set chronic glomerulonephritis in 3 patients. They were treated by hemodialysis in 10 patients and intermittent peritoneal dialysis in 1 patient. Ultrasonic measurement of the size of kidneys revealed that the thickness (anterior-posterior diameter) and the ratio of thickness to length (T/L) were greater in patients with ARF than in those with chronic renal failure and normal renal function. The patients with a low value of T/L (under 0.60) had a significantly greater urine volume than those with high a value of T/L (0.60 or more). The sonographic features of renal-ARF kidneys were marked increase in parenchymal echogenicity and appearance of hypoechoic swollen renal pyramids with sharpness of the corticomedullary border. In the course of ARF, these sonographic changes gradually disappeared when the patients had recovered from ARF. However, the prognosis was poor in patients with severer sonographic findings. We believe that repeated ultrasonic examination of the kidneys in patients with renal-ARF is useful for not only differential diagnosis of post-renal urinary obstruction but evaluating the course of ARF.     

Acquired cystic disease of the kidneys and renal cell carcinoma in chronic renal insufficiency without dialysis treatment

We report 3 cases of acquired cystic disease of the kidneys with associated renal carcinoma in 2 of the cases. In all 3 cases, the patients had chronic renal insufficiency due to hypertension but had never required dialysis. Review of 176 reported cases of acquired cystic disease of the kidneys and renal tumors disclosed that 18 patients (including 1 previously reported by us) had never received dialysis treatment. These cases support the hypothesis that acquired cystic disease of the kidney is not restricted to patients treated with maintenance dialysis. Among the 18 patients, hypertension was the most common underlying cause of renal failure. Patients with chronic renal failure due to or associated with severe hypertension should be monitored carefully for the development of both renal cysts and tumors even though they have not started on chronic dialysis.     

Acquired cystic disease of kidney associated with renal cell carcinoma in chronic dialysis patients.

We describe 2 cases of acquired cystic disease of the kidney (ACDK) associated with renal cell carcinoma in patients treated with long-term hemodialysis. Both patients have had dialysis for five and eight years, respectively. Renal cell carcinomas of these patients are small, averaging 2 cm in diameter. They are clear cell type. Atypical epithelial hyperplasia arising from cystic areas can be seen intermingling with carcinoma. This confirms that atypical epithelial hyperplasia is a precursor of renal cell carcinoma. Although the incidence of renal cell carcinoma arising in ACDK is on the rise, the issue of how to manage patients with ACDK remained unsettled and required further study.     

A study of the native kidneys in patients with chronic renal failure. Report 2: Correlation between cystic changes in native kidneys and development of renal tumor

The factors which made acquired cysts develop to renal tumors in chronic hemodialysis patients was investigated from clinical and histological findings. Although three renal tumors were found among 151 patients with chronic renal failure, clinically, one case had acquired cystic disease of the kidneys (ACDK), one case had simple cysts and the other case did not have any cyst. However, the last two cases had multiple small cysts histologically. In ACDK, renal tubules were degenerated and dilated, fused to each other, forming more larger cysts. Furthermore, epithelium of acquired cysts showed columer and dysplastic change, and papillary adenomas were developed into the inner space of the cyst wall. And ACDK kidney without renal tumor also revealed the same findings in histological examinations. It was postulated that renal tumor occurred from the papillary projections of the dysplastic epithelium of cysts. Therefore cysts in uremic patients seemed to be different from the simple cyst in normally functioning kidneys.     

A study of the native kidneys in patients with chronic renal failure. Report 1: Morphological changes and occurrence of acquired cysts of the native kidneys in patients with chronic renal failure

As morphological changes of the native kidneys, the presence of acquired cysts, intra-renal calcification and renal tumor was investigated mainly by ultrasonography and computed tomography in 151 patients with chronic renal failure. 1) Among 140 uremic patients caused by medical diseases. 40 patients had simple cysts, and 37 had acquired cystic disease of the kidneys (ACDK). However 4, uremic patients caused by urological diseases were not seen to be with an acquired cysts. 2) The patients with ACDK were with significantly longer durations of dialysis therapy than those with simple cysts or no cysts. Furthermore, the patients with ACDK were younger than those of the other groups. 3) Intra-renal calcification was more frequently found in patients with cystic disease (simple cyst, ACDK and polycystic disease) than in those without cystic disease with statistic significance. 4) Native kidneys became larger after the occurrence of ACDK. 5) Three cases of renal tumor were found.     

Immunological study on renal cell carcinoma in dialysis patients with acquired cystic disease of kidneys]

The immunological study of the major histocompatibility complex (class I, class II and DR antigens), tumour infiltrating lymphocytes (TIL), regional lymph node lymphocytes (RLNL) and peripheral blood lymphocytes (PBL) was evaluated on the basis of immunohistochemical staining using monoclonal antibodies of each subset of lymphocytes in a series of 16 patients with renal cell carcinoma. Two renal cell carcinomas in dialysis patients with acquired cystic disease of the kidneys (ACDK) were also included in this study. With regard to the immunological environment, a comparative study between renal cell carcinoma accompanied with ACDK and 14 other renal cell carcinoma was carried out. The results are described below: 1) With regard to the expression of MHC antigens in tumour cells, the degrees of expression of MHC class I, class II and DR-antigen in case 1 were higher than that of the other 14 renal cell carcinomas. On the other hand, no expression of MHC was detected in case 2. 2) As to the subsets of TIL, the CD25 (IL-2 receptor) was not expressed in all the renal cell carcinoma. As to the T cell receptor (TCR-alpha/beta chain), the degree of expression was the same in case 1 and the other 14 cases. On the other hand, no TCR was detected in the case 2. As to the other subsets of TIL (CD3, CD4, CD8, CD16 and CD20), the rates of the infiltration were the same in case 1 and the other 14 cases, but those in case 2 were lesser than in all other 14 cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Acquired cystic disease of kidney in chronic dialysis patients

Eight cases of acquired cystic disease of the kidney (ACDK) associated with chronic renal failure and hemodialysis are described. No patient had a family history or clinical evidence of congenital adult polycystic kidney disease (CAPKD). Glomerulonephritis was the cause of renal failure in 6, and pyelonephritis in 2. Massive renal and perirenal hemorrhage necessitated 3 nephrectomies in 2 patients. Single kidney weights did not exceed 280 Gm., a major feature in the distinction of ACDK from CAPKD. Morphologically, in addition to the usual stigmata of end-stage kidneys, 40 to 80 per cent of the renal parenchyma was replaced by small cysts. Continuity of cysts with tubules was established by nephron dissection.     

Acquired renal cystic disease in HD: a study of 82 nephrectomies in young patients.

In order to study the development of acquired renal cystic disease (ARCD) and its potential complications, we studied, macro- and microscopically, 82 surgical specimens of nephrectomy carried out on young patients with chronic renal failure previous to renal transplantation. Statistical correlation of pathological findings with age, sex and time on hemodialysis (HD) have been done. There were 72 cases of ARCD (87.8%). It was statistically correlated with male sex (p less than 0.02) and prolonged time on HD (p less than 0.001) as has been previously reported. Hyperplasia of the cystic epithelium was found in 42 cases (52%), with 18 (22%) showing marked papillary proliferation. Also, there were 22 cases (27%) with renal adenomas. This incidence of hyperplastic and neoplastic proliferations, more than would be common in such a young population (males: 33.5 +/- 9.3 years; females: 35.4 +/- 11.7 years), suggests the potential of patients affected by ARCD to develop neoplasms. Thus, we consider that these patients must be checked periodically to detect possible malignant neoplasms.     

Ultrasonographic evaluation of the femoral cartilage thickness in patients with chronic renal failure

Objective To investigate the effects of chronic renal failure (CRF) on the distal femoral cartilage thickness by using ultrasonography and to determine the relationship between cartilage thickness and certain disease-related parameters. Design Fifty-seven CRF patients (41 male and 16 female) (mean [SD] age, 44.7 [12.1] years) and 60 healthy controls (41 male and 19 female) (mean [SD] age, 43.5 [13.3] years) were enrolled in this study. Demographic and clinical characteristics were recorded. Cartilage thickness measurements were taken from the medial and lateral condyles, and intercondylar areas of both knees. Results Groups were similar in terms of age, weight, height, body mass index and gender (all p>0.05). The mean cartilage thickness was found to be less in CRF patients than in controls (statistically significant for medial condyles and intercondylar areas both in right and the left knees [all p<0.05]). Cartilage thickness showed no correlation with eGFR, and with the levels of serum urea, creatinine, calcium, magnesium, phosphor, hemoglobin, uric acid and as well as steroid use (all p>0.05) in CRF patients. Conclusion In the light of our findings, we imply that patients with CRF have thinner femoral cartilage than healthy controls. This result may support the view that patients with CRF are at increased risk for developing early knee osteoarthritis. Last but not least, clinicians should be aware of the importance of rehabilitation strategies aimed at decreasing onset and progression of knee osteoarthritis in patients with CRF.     

Nephroblastoma and end-stage renal failure with bilateral cystic kidneys

A child of 10 years 5 months presenting with chronic renal failure had bilateral cystic kidneys. Biopsy of a right lower-pole solid mass revealed nephroblastoma. At bilateral nephrectomy, both kidneys were both replaced by variable-sized cysts with a unifocal nephroblastoma on the right. Renal failure with nephroblastoma is uncommon and is usually either a manifestation of the Drash syndrome or a complication of chemotherapy. The need to assess both kidneys in a child with any other renal abnormality in addition to a renal mass should always be considered.     

Acquired cystic disease in chronically rejected renal transplants.

Acquired cystic disease has been documented to complicate most forms of chronic renal damage; it has only infrequently been described in transplanted kidneys. Five patients with noncystic ESRD and chronically rejected transplants in which acquired cystic disease arose are reported. The diagnosis of acquired cystic disease was established in examination of transplant nephrectomies from four patients and a core biopsy from the fifth. The allografts were in place from 44 to 80 months; three patients were treated with hemodialysis before the diagnosis of acquired cystic disease, whereas two received peritoneal dialysis. Three of the four patients evaluated had cysts in the native kidneys. Although papillary hyperplasia of lining epithelium was evident in four specimens, only one kidney was the site of neoplasms in the form of multiple small tubular adenomas. No malignant neoplasms were noted in this study or in the few similar previous ones; however, it is possible that chronically rejected transplanted kidneys may harbor neoplasms with the same malignant potential as those in acquired cystic disease in native kidneys.     

Acquired renal cystic disease in end-stage renal disease: an autopsy study of 155 cases

Autopsy reports on 155 successive end-stage renal disease (ESRD) patients and 147 control patients without ESRD, matched for age, race and gender, were collected. Cystic transformation of the kidneys was noted in 58% of the ESRD patients and 13.6% of the control patients. There were 25 ESRD patients with renal adenoma and 3 with renal cell carcinoma. The single best predictor of cystic transformation or tumorous degeneration was patient's age. A statistically significant association between cystic transformation and gender, as well as the cause of ESRD, was also found. In contrast to previous reports we were unable to document a strong association between the incidence of either cystic transformation or tumorous degeneration with the duration of dialysis, nor did these two parameters correlate with mode of dialysis. We suggest that cystic transformation of the kidneys in ESRD is related to age and renal failure per se, is not strongly associated with duration of dialysis and is independent of mode of dialysis. Concomitant tumorous degeneration was frequent, but this was usually an incidental autopsy finding. Renal malignancy was uncommon and metastases were absent.     

Acquired cystic kidney disease: occurrence in patients on chronic peritoneal dialysis

Acquired cystic kidney disease (ACKD) is a well-known complication of long-term hemodialysis. To the best of our knowledge, only six patients on continuous ambulatory peritoneal dialysis have been reported to develop this disease. We herein report two such cases, and concluded that the morphology of ACKD seems to be independent of the type of dialysis and that hemodialysis is not necessary for the development of ACKD.     

Progression of chronic renal failure in children with dysplastic kidneys

The aim of this study is to describe progression of chronic renal failure (CRF) in children with renal malformations and to study factors influencing this progression. We reviewed retrospectively 176 children with CRF secondary to renal dysplasia, reflux nephropathy or renal obstruction with at least 5 years of follow-up. Serum creatinine was recorded at least every third month, and an estimated glomerular filtration rate (eGFR) was calculated. Number of febrile urinary tract infections (UTI), blood pressure, albuminuria (UaUc), and number of functioning kidneys was also recorded. We found that the development of renal function could be separated into three time periods: (1) During the first years of life, 82% of the children showed early improvement of their kidney function, which lasted until a median age of 3.2 years (median improvement 6.3 ml/year). (2) From the age of 3.2 years until 11.4 years, 52.5% of the studied children showed a stable kidney function, whereas in 47.5%, kidney function immediately started to deteriorate. (3) Around puberty, 42.9% started deterioration in kidney function, whereas 57.1% even after puberty showed a stable function. Patients with UaUc >200 mg/mmol deteriorated faster (−6.5 ml/min per 1.73 m² per year compared with −1.5 ml/min per 1.73 m² per year) in those with UaUc <50 mg/mmol. Children with more than two febrile UTIs, hypertension or an eGFR at onset of less than 40 ml/min per 1.73 m² deteriorated faster than the others. Most children experienced early improvement of kidney function. The further prognosis, early or late deterioration of kidney function or stable function during the whole follow-up, was related to albuminuria, number of febrile UTIs, eGFR at onset of deterioration, hypertension and puberty.     

Dyslipidaemia and the progression of renal disease in chronic renal failure patients.

BACKGROUND: Dyslipidaemia is common in patients with chronic renal failure (CRF), and there is increasing evidence to support the role of dyslipidaemia as a contributing factor in the progression of chronic renal disease. However, few prospective studies have been carried out which address the possible relationship between dyslipidaemia and the rate of progression of renal disease in patients with renal failure. METHODS: Between January 1985 and December 1997, we prospectively assessed the risk of CRF progression to dialysis in a cohort of 138 patients. Forty CRF patients reached end-stage renal disease (ESRD) and had to start supportive therapy during the follow-up period [group ESRD(+)]. The remaining 98 CRF patients served as controls [group ESRD(-)]. Potential clinical and laboratory risk factors for more rapid CRF decline to dialysis, including lipid abnormalities and baseline creatinine clearance were determined at the start of the follow-up period. RESULTS: Several significant differences were found in univariate analysis between the two groups of CRF, ESRD(+) and ESRD(-), namely a shorter follow-up period, a lower level of baseline creatinine clearance, a faster rate of creatinine clearance decline, a higher level of serum triglycerides, fibrinogen, total homocyst(e)ine and proteinuria, and a lower level of serum high-density lipoprotein in the ESRD(+) group than in the ESRD(-) group. However, by multivariate Cox analysis proteinuria [relative risk (95% confidence interval) 1.32 (1.16-1.50) for each g/day P = 0.001], baseline creatinine clearance [0.53 (0.40-0.70) for each 10 ml/min, P = 0.001] and chronic interstitial nephritis and hypertensive nephrosclerosis [0.38 (0.17-0.84) for presence, P = 0.005] were the only significant risk factors for CRF progression to dialysis. Hypertriglyceridaemia and male gender were selected in the final model, but were of borderline significance. CONCLUSIONS: These results suggest a limited role for dyslipidaemia in the progression of chronic renal disease to dialysis in CRF patients, in contrast with the powerful influence of proteinuria, baseline creatinine clearance and nephropathy type in predicting this progression.     

Acquired cystic disease of the kidney and renal carcinoma in haemodialysis patients: ultrasonographic evaluation.

Ultrasonography was performed in 661 dialysis patients and acquired cystic disease of the kidney was found in 156 (125 men and 31 women). A higher incidence of cystic disease was found in males. There was no significant difference between the patients with and those without acquired cystic disease in terms of average age, but the duration of haemodialysis in those with acquired cystic disease was significantly longer. There was an increased incidence of cystic disease in patients with glomerulonephritis and the duration of haemodialysis in these patients was significantly longer. This suggests that the increased incidence of acquired cystic disease of the kidneys in the patients with glomerulonephritis is simply related to the longer duration of treatment. Twelve patients with renal carcinoma were found in this study. The average age at diagnosis of renal carcinoma was not significantly different between the patients with and those without acquired cystic disease, but the duration of dialysis was significantly longer in renal carcinoma patients with acquired cystic disease. The incidence of renal carcinoma in dialysis patients with acquired cystic disease was 3.85% and in those without it was 1.19%. These rates are considerably higher than those found in the general population and indicate that the risk of renal carcinoma is higher in dialysis patients both with and without acquired cystic disease.     

获得性肾囊性疾病合并肾癌11例临床分析

目的 探讨获得性肾囊性疾病(ACKD)合并肾癌的诊断治疗策略.方法 回顾性分析11例终末期肾衰竭、获得性肾囊肿合并肾癌患者的临床资料.男8例,女3例.平均年龄55(37～68)岁.行血液透析至发现肾脏病变时间平均为4.8(2.8～7.4)年.结果 11例均行肾癌根治术.术后病理报告:透明细胞癌3例,乳头状癌6例,嫌色细胞癌1例,乳头状腺瘤1例.病理分期T1a9例,T1b 2例.11例术后随访平均55(17～83)个月.1例术后24个月发现肺转移;1例单侧发病者术后22个月对侧复发,行肾癌根治术;1例死于心血管疾病;1例随访19个月后失访;无瘤生存7例.结论 ACKD与肾癌有较高的相关性.终末期肾衰竭患者透析前氮质血症时间较长或透析时间＞3年者,应排除ACKD.超声及CT检查对早期诊断存在价值.除关注ACKD恶性变倾向外,对长期肾衰竭患者的其他并发症如心脑血管疾病、糖尿病等也应足够重视并行积极治疗.

Abstract：

Objective To discuss the diagnosis and treatment of acquired cystic kidney disease complicated by kidney cancer. Methods Clinical data of 11 patients with acquired cystic kidney disease complicated by kidney cancer were analyzed retrospectively. Eight patients were male and three were female. The mean age was 55 years old (range 37 to 68). The time of hemodialysis ranged from 2.8 to 7. 4 years, mean 4. 8 years. Results Follow-up ranged from 17- 83 months, mean 55 months. One patient died of cardiovascular disease. Lung metastasis was detected in one patient two years after surgery. Seven patients survived free of tumor recurrence and there was no follow-up on one patient. Conclusions Increased incidence of cancer was observed in patients with end-stage renal disease who have undergone long-term dialysis. In particular, renal cell carcinoma (RCC) showed an excess incidence in ACKD patients. RCC showed an increased prevalence compared with the general population. Patients with predialysis azotemia or a dialysis duration of longer than 3 years should be screened for ACKD. Sonegraphy or CT scanning are useful for early diagnosis of ACKD. We should pay close attention to complications, including ACKD malignant tendency, in patients who have been taking long-term dialysis and positive therapy.     

Acquired renal cystic disease: implications for the urologist

Acquired cystic kidney disease has become increasingly recognised as a significant risk in patients with end-stage renal disease, especially in those maintained on chronic haemodialysis and peritoneal dialysis. A review of the literature indicates that nearly 50% of patients on dialysis for more than 3 years develop renal cystic changes. The major complications of this condition are neoplasia and spontaneous renal haemorrhage. The risk of developing renal carcinoma has been estimated to be more than 30 times higher in dialysis patients with cystic changes than in the general population. Our experience with 5 patients is reported, including 3 with renal tumours and 1 with metastatic disease. Careful surveillance of dialysis patients using yearly ultrasonography and computed tomography is recommended. The evolving indications for radical nephrectomy in this disease are discussed.