Twenty-year follow-up of acquired renal cystic disease

Division of Nephrology, Department of Internal Medicine, Kanazawa Medical University, Uchinada, Ishikawa, Japan.
Clinical nephrology (Impact Factor: 1.13). 04/2003; 59(3):153-9. DOI: 10.5414/CNP59153
Source: PubMed


Since 1979 the diseased kidneys of 96 patients on replacement therapy with chronic renal failure due to chronic glomerulonephritis have been followed to investigate the development of acquired cysts and tumors. This is a report of the 20-year follow-up.
Ninety-six patients were followed using periodic CT scan and were divided into hemodialysis, renal transplantation, bilateral nephrectomy and deceased groups during the follow-up. In the hemodialysis group, 36 patients (19 males, 17 females) were followed for 20 years.
Kidney volumes which were 57.8 (1.51) (geometric mean (geometric SD)) ml at start of the follow-up had become 185.3 (2.03) ml 20 years later in males, and in females, 57.3 (1.64) ml had become 99.7 (2.36) ml. The increased rate was 3.2 (2.06) fold in males and 1.7 (2.57) fold in females. This enlargement of the kidneys was due to acquired cysts. Kidney volumes at the 20-year follow-up had increased more significantly than those at the 15-year follow-up in males; however, kidney volumes at the 20-year follow-up had not changed in females, if compared with data at the 15-year follow-up. Kidney volumes in males at 20-year follow-up were significantly larger than those in females (p = 0.0232). Males with more than 3.2-fold in kidney volume increase at the 20-year follow-up were under the age of 40 at entry into this study (p = 0.0055), although the correlation between the degree of kidney volume increase and age was not significant (p = 0.0910). Kidney volumes in the transplantation group remained small. There was no new renal cell carcinoma development after 15-year follow-up except for the local recurrence of a previous operated case. Although 7 of 44 patients died during the past 20 years due to malignancy, no patient died of renal cell carcinoma because of early detection and treatment. One patient died of retroperitoneal bleeding, which is a complication of acquired renal cystic disease.
Male preponderance of acquired cysts was maintained at the 20-year follow-up. There was a tendency for the rate of increase in acquired renal cystic disease to be larger in young males. No one died of renal cell carcinoma, although the incidence of renal cell carcinoma was high.

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    • "Moreover, the risk is considered to increase with duration of dialysis [5]. Several factors could contribute to this higher prevalence, including depressed host immunity [6], impaired antioxidant defense mechanisms, increased synthesis of reactive oxygen [7] [8], and acquired renal cystic formation [9]. On the contrary, the biological behavior of RCC related to ESRD (ESRD-RCC) is generally reported to be less aggressive than that of RCC in the general population [3] [10]. "
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    ABSTRACT: Objectives: Previous studies have reported that elevated pretreatment C-reactive protein (CRP) levels are associated with poor outcome in various malignancies, including renal cell carcinoma (RCC), in the general population. However, there is no evidence of such an association in dialysis patients. Therefore, the aim of this study is to evaluate the prognostic significance of preoperative serum CRP levels in patients with RCC related to end-stage renal disease (ESRD) requiring hemodialysis (HD). Materials and methods: We evaluated 315 patients with ESRD requiring HD who underwent nephrectomy for RCC as the first-line treatment at our hospital from 1982 to 2013. Complete patient- and tumor-specific characteristics as well as preoperative CRP levels were assessed. We defined a serum CRP level >0.5mg/dl as elevated and divided these patients into 2 groups according to their preoperative CRP levels (CRP≤0.5 and >0.5mg/dl). The median follow-up was 51 months. Results: Preoperative CRP levels were elevated in 75 patients (23.8%). The Kaplan-Meier 5-year cancer-specific survival rates were 95.2% and 69.9% in patients with CRP levels≤0.5 and>0.5mg/dl, respectively (P<0.0001). Multivariate analysis identified preoperative CRP level as an independent predictor for cancer-specific survival, along with a pathological TNM stage and tumor grade (CRP>0.5: hazard ratio = 3.47; 95% CI: 1.35-9.18; P = 0.0098). The concordance index of multivariable base models increased after including the preoperative CRP levels. Conclusions: Preoperative serum CRP level might be an independent predictor of postoperative survival in patients with RCC related to ESRD requiring HD. Its routine use, together with the TNM classification and tumor grade, could allow better risk stratification and risk-adjusted follow-up of these patients.
    Full-text · Article · Aug 2014 · Urologic Oncology
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    • "Nevertheless, the AS of PKD1 could be of interest if it is expressed in the kidney. It could provide a way to explain the high incidence of acquired cystic kidney disease observed in patients with CKD [26]. Unfortunately, our clinical study did not put in light a statistical association between ACKD and the AS of PKD1. "
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    ABSTRACT: The control of gene expression in the course of chronic kidney disease (CKD) is not well addressed. Alternative splicing is a common way to increase complexity of proteins. More than 90% of human transcripts are alternatively spliced. We hypothesised that CKD can induce modification of the alternative splicing machinery. During mutation screening in autosomal dominant polycystic kidney disease, we identified in mononuclear cells (PBMC), an alternative splicing event on the exon 30 of PKD1 gene, the gene implicated in this disease. This alternative splice variant was not correlated with the cystic disease but with CKD. To confirm the association between this variant and CKD, a monocentric clinical study was performed with 3 different groups according to their kidney function (CKD5D, CKD3-5 and normal kidney function). An exon microarray approach was used to highlight splicing events in whole human genome in a normal cell model (fibroblasts) incubated with uremic serum. Alternative splicing variants identified were confirmed by RT-PCR. The splicing variant of the exon 30 of PKD1 was more frequent in PBMCs from patients with CKD compared to control. With the microarray approach, despite the analysis of more than 230 000 probes, we identified 36 genes with an abnormal splicing index evocating splicing event in fibroblasts exposed to uremic serum. Only one abnormal splicing event in one gene, ADH1B, was confirmed by RT-PCR. We observed two alternative spliced genes in two different cell types associated with CKD. Alternative splicing could play a role in the control of gene expression during CKD but it does not seem to be a major mechanism.
    Full-text · Article · Dec 2013 · PLoS ONE
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    • "After approval by institutional review boards at all centres, patients with RCCs arising in the context of ESRD (defined as chronic renal failure treated by dialysis or transplantation) and/or who underwent a renal transplantation were identified at each centre. The following variables were recorded: age, sex, symptoms at diagnosis, Eastern Cooperative Oncology Group performance status (ECOG PS), duration of exposure to ESRD, presence of ACKD (defined by more than five cysts on each native kidney assessed by computed tomography [CT] or ultrasound imaging [2]), mode and duration of dialysis, renal transplantation, tumour staging and grading, histologic subtype, renal transplant, cancer-specific survival (CSS), and overall survival. For ESRD patients, surgical treatment of localised RCC was radical nephrectomy. "
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    Full-text · Article · Jan 2013 · BJU International
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