This article has Open Peer Review reports available.
Renal replacement therapy in ADPKD patients: a 25-year survey based on the Catalan registry
© Martínez et al.; licensee BioMed Central Ltd. 2013
Received: 14 May 2013
Accepted: 4 September 2013
Published: 5 September 2013
Some 7-10% of patients on replacement renal therapy (RRT) are receiving it because of autosomal dominant polycystic kidney disease (ADPKD). The age at initiation of RRT is expected to increase over time.
Clinical data of 1,586 patients (7.9%) with ADPKD and 18,447 (92.1%) patients with other nephropathies were analysed from 1984 through 2009 (1984–1991, 1992–1999 and 2000–2009).
The age at initiation of RRT remained stable over the three periods in the ADPKD group (56.7 ± 10.9 (mean ± SD) vs 57.5 ± 12.1 vs 57.8 ± 13.3 years), whereas it increased significantly in the non-ADPKD group (from 54.8 ± 16.8 to 63.9 ± 16.3 years, p < 0.001). The ratio of males to females was higher for non-ADPKD than for ADPKD patients (1.6–1.8 vs 1.1–1.2). The prevalence of diabetes was significantly lower in the ADPKD group (6.76% vs 11.89%, p < 0.001), as were most of the co-morbidities studied, with the exception of hypertension. The survival rate of the ADPKD patients on RRT was higher than that of the non-ADPKD patients (p < 0.001).
Over time neither changes in age nor alterations in male to female ratio have occurred among ADPKD patients who have started RRT, probably because of the impact of unmodifiable genetic factors in the absence of a specific treatment.
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disease caused by mutations in two genes, PKD1 and PKD2, which encode for polycystin 1 and polycystin 2, respectively: mutations in PKD1 are present in 85% of cases, and mutations in PKD2 in the remaining 15% [1–3]. The abnormalities in these proteins cause the appearance and growth of kidney cysts that gradually impair renal function . ADPKD is the cause of chronic kidney disease (CKD) in 5% of patients undergoing renal replacement therapy (RRT) in the USA , a rate that rises to 7.9% in our geographic area . In 75-82% of patients it is additionally associated with hypertension, which often appears before CKD development , and with other systemic disturbances such as liver cysts, pancreatic cysts, brain aneurysms or colonic diverticula.
Progression of CKD in ADPKD basically depends on a combination of genetic and environmental factors, with hypertension as a significant prognostic factor. CKD is more severe and appears earlier in patients with PKD1 gene mutations than in those with PKD2 mutations (mean age at RRT initiation: 53 and 69 years, respectively) [3, 7]. Also, the prognosis is worse for those harbouring a truncating PKD1 mutation compared with those with missense mutations . Various studies have shown that controlling hypertension slows down CKD development in patients with ADPKD . A recent trial, however, has indicated that reduced glomerular filtration is a function of older age, and that there are no differences between hypertensive and normotensive patients ; hypertensionis present in nearly 90% of ADPKD patients once they have renal failure.
CKD is associated with a high risk of cardiovascular disease: cardiovascular mortality among dialysis patients is 10–30 times greater than among the general population . This fact is explained by the higher prevalence of classic cardiovascular risk factors in such patients: hypertension, diabetes, dyslipidaemia and smoking . Diabetic nephropathy and vascular nephropathy are the primary reasons for initiation of dialysis, specially among the North American population [4, 5]. Moreover, a slowdown in CKD progression has been verified when cardiovascular risk factors are controlled . However, a lower prevalence of these risk factors has been described in patients with ADPKD, with the sole exception of hypertension , which is present in nearly 90% of ADPKD patients once they have renal failure.
The objective of this observational study is to analyse the epidemiological, clinical and survival changes that occur over time in ADPKD patients who have started RRT, as compared with non-ADPKD patients on RRT during the last 25 years.
The data analysed came from the Catalan Renal Patients Registry, created in 1984 with the aim of assisting the Renal Failure Care Programme in planning its resources. This is a mandatory reporting registry for all patients in Catalonia, a Spanish autonomous community with over 7 million inhabitants. This registry collects socio-demographic and clinical data of all patients who start RRT, including haemodialysis, peritoneal dialysis and renal transplantation and excluding transient patients. The quality of the information is ensured by means of mechanisms of validation and processing of the periodically reported cases, and the performance of a yearly follow-up of all active cases as of 31 December.
Patients who started RRT between 1984 and 2009 were considered and divided into two groups: patients on RRT because of ADPKD [code 41 in the ERA-EDTA classification of Primary Renal Disease (PRD)], and the remaining patients with other reasons for initiation of RRT (remaining ERA-EDTA PRD codes), called the non-ADPKD group. The diagnosis of ADPKD was specified by the clinician at the time of reporting to the Registry, based on the patient’s family history and on ultrasound criteria compatible with the disease. Patients actively on dialysis at 31 December 2009 were also considered when describing the current situation of patients.
Clinical and demographic data
The study follow-up time was divided into three periods of RRT initiation: 1984–1991, 1992–1999 and 2000–2009.
The variables analysed were gender and age, both upon RRT initiation and at the end of the RRT study, and the presence of associated diseases in the 1992–2009 cases. These associated diseases included: hypertension (defined as blood pressure over 140/90), diabetes mellitus, ischaemic or non-ischaemic heart disease, arrhythmia, cerebrovascular disease, peripheral vascular disease, malignancies, chronic respiratory disease, tuberculosis, chronic liver disease, gastroduodenal disorders, bowel disease and joint disease. These co-morbidities were analysed from 2000 to 2009 because from 1992 to 1999 it was not compulsory to notify them and therefore data were not complete.
The first vascular access (temporary catheter, tunnelled catheter, arteriovenous fistula or prosthetic graft) was also analysed in patients who started haemodialysis between 1997 and 2009, divided into two periods: 1997–2003 and 2004–2009.
In patients actively on dialysis as of 31 December 2009, we studied: percentage of patients with hepatitis C virus, percentage of patients who received erythropoietin, and C-reactive protein (CRP) levels in mg/l (CRP >10 mg/l or <10 mg/l), according to the standard method.
In patients who had received a kidney transplant we analysed the type of donor (living or cadaveric), the time elapsed since start of dialysis to transplant surgery, and the age at the time of transplantation.
Regarding those patients who died while on RRT, we analysed their age at the time of death and the causes of death. We classified the causes of death as follows: cardiac, vascular, infectious, cancer, hepatic, social, other and unknown. Survival rates of patients with and without ADPKD during the first 3 years after initiation of RRT, and also patient and renal graft survival from the time of transplantation, when applicable, were also considered.
Software STATA11 was used for statistical analysis. Chi-square test was applied to analyse qualitative variables, and Student’s t-test for the comparison of means of the quantitative variable. The log-rank test was used to verify the survival rates. Statistical significance was set at p < 0.05.
Only the information contained in the fields of the mandatory reporting registry of the Catalan Renal Patients Registry has been used in this observational study. Other interesting information for the purpose of this study, but not included in these forms, was not available. For example, data relating to the period prior to the onset of end-stage renal disease (ESRD) were not available as the study is based on a registry of ESRD.
A total of 20,033 patients in whom RRT had been initiated were registered between 1984 and 2009: 1,586 of them (7.9%) had ADPKD and 18,447 (92.1%) had other nephropathies.
Clinical features of ADPKD and non-ADPKD patients on RRT
ADPKD (n: 1586)
Non ADPKD (n: 18,447)
Age (years) at initiation of RRT in men
57.2 ± 12.7
60.9 ± 16.6
Age (years) at initiation of RRT in women
57.8 ± 12.0
62.5 ± 17.0
First choice of RRT (%)
RRT strategy at 31.12.2009 (%)1
Time from initiation of RRT to TX (years)2
p = 0.09
Age at TX (years)2
Treated with ESA (%)1
CRP (<10 mg/l)1
Associated diseases in patients with and without ADPKD (2000–2009)
ADPKD % (n)
Non-ADPKD % (n)
Most of the patients started RRT with haemodialysis in both groups. However, a prevalence analysis dated 31 December 2009 showed that transplantation was the most prevalent situation among ADPKD patients: 65.4% in the ADPKD group and 49.3% in the non-ADPKD group. The analysis also showed that fewer patients with ADPKD required erythropoietin (p < 0.001) and that they had a lower prevalence of hepatitis C virus (p < 0.001). The CRP levels were lower in the ADPKD group, but this difference did not attain statistical significance (p = 0.124) (Table 1).
Significant differences (p < 0.001) in the use of different types of vascular access at initiation of haemodialysis were also observed in both the periods analysed (1997–2003 and 2004–2009) in the ADPKD group. An increase in arteriovenous fistulae was observed (74.3% vs 78.6%), and the same was true for tunnelled catheters (2.6% vs 7.5%) and prostheses (2.6% vs 7.5%); there was also a reduction in the number of temporary catheters (22.3% vs 11.7%). When analysing patients with kidney transplants, the number of living donors increased only over the most recent years (10–11 per year), even though cadaveric transplants continue to be more frequent (95.1% of all kidney transplants).
Causes of death of ADPKD and non-ADPKD patients after starting RRT
Cause of death
ADPKD % (n)
Non-ADPKD % (n)
Age1(ADPKD vs non-ADPKD)
69.1 ± 9.8 vs 68.9 ± 12.1
68.8 ± 10.6 vs 69.4 ± 12.6
68.8 ± 9.6 vs 67.9 ± 13.6
67.5 ± 9.3 vs 67.6 ± 12.2
64.1 ± 2.2 vs 61.1 ± 0.9
75.1 ± 6.6 vs 75.7 ± 10.4
71.1 ± 9.9 vs 71.6 ± 12.4
67.9 ± 9.7 vs 70.0 ± 12.3
Survival rates in ADPKD and non-ADPKD patients during the first three years
Non ADPKD (%)
Patients on RRT
Although there is no specific treatment for ADPKD as yet, the therapeutic advances to slow down CKD might cause one to expect that, over time, onset of ESRD in ADPKD patients will have been delayed until a later age. Our data, however, show that the age at initiation of RRT in these patients has not varied since 1984. This result is in concordance with other articles , but some publications [16–18] do indicate that the age at initiation of RRT in ADPKD patients has indeed increased. According to Orskov et al. , age at onset of ESRD among their 693 ADPKD patients, followed up for 18 years (divided into three periods), rose from 55.9 (1990–1995) to 60.6 years (2002–2007), though these findings are subject to a potential bias, given the increasing number of patients in each period and especially the rise in the number of women.
The prognosis of CKD patients who start with RRT has improved over the last few years [19, 20]. In the European Registry, the percentage of patients older than 65 who started RRT increased from 22% in 1980 to 55% in 2005 , and this may be attributed to a better control of cardiovascular risk factors. We have not been able to find in the literature any studies comparing the age at onset of ESRD between patients with and patients without ADPKD. Based on our own results, we would stress that while, over the three periods examined, the age at initiation of RRT in non-ADPKD patients rose by more than 9 years, no such change occurred among ADPKD patients.
Epidemiological studies indicate that within the general population, the prevalence of men undergoing RRT is higher than that of women, in accordance with our findings . These data are in agreement with some other publications that associate male gender with earlier initiation of RRT in ADPKD patients [22, 23]. In contrast, and in accordance with other studies [16, 24], we found no significant gender differences in relation to age at initiation of RRT. Our data show that male gender is a risk factor for initiation of RRT among non-ADPKD patients (ratio, 1.6-1.8), but not among ADPKD patients (ratio, 1.1-1.2).
Although most of our ADPKD patients started RRT with haemodialysis, the prevalence study revealed kidney transplantation to be the most widely used method, as expected based on the age at onset of ESRD in this population. The sparse prescription of peritoneal dialysis among ADPKD patients could be due to large kidneys, to the presence of colonic diverticula, to hernias or to massive polycystic liver disease. However, peritoneal dialysis is a first-line technique for this disease, as has been shown by several studies that revealed no differences concerning survival or risk of peritonitis [25, 26]. On the contrary, it was found that ADPKD patients on peritoneal dialysis had a higher survival rate than those on haemodialysis .
The factors related to greater ADPKD severity are primarily carrying a PKD1 mutation, especially if truncating, and hypertension [3, 8, 27]. Other relevant factors include: age at diagnosis [28, 29], increased number and volume of the cysts, left ventricle hypertrophy , proteinuria , haematuria, microalbuminuria, hyperuricaemia , low HDL-cholesterol levels, high urinary sodium and an increase in urinary osmolality . Cardiovascular risk factors increase the rate CKD progression [4, 12], with both entities boosting each other, giving rise to a cardiorenal syndrome. ADPKD is a typical model of type IV cardiorenal syndrome , as the cause of CKD is primary renal failure and secondary cardiovascular involvement. We have observed a lower prevalence of diabetes among patients with ADPKD, but the differences in comparison with non-ADKPD patients have not been found to be significant with regard to hypertension, similarly to other studies [14, 35–37]. The data published on associated diseases in ADPKD and non-ADPKD patients are either incomplete  or have concerned only cardiovascular events . We have observed that cardiovascular diseases, malignancies and respiratory diseases are more frequent among non-ADPKD patients on RRT. This higher rate of co-morbidities in non-ADPKD patients could be explained by the higher prevalence of diabetes and by initiation of RRT at an older age. The lower cardiovascular morbidity among ADPKD patients could be associated with higher haemoglobin levels in this group . In correspondence with Abbott and Agodoa’s results , we found that fewer ADPKD patients on dialysis need erythropoietin compared with the non-ADPKD group (77.9% vs 91.9%). CKD favours a chronic inflammation status with increased inflammatory markers such as C-reactive protein (CRP) . In accordance with other articles, we observed no significant differences between the groups with regard to CRP levels, although they were lower among ADPKD patients on dialysis, probably owing to the lower prevalence of associated diseases .
The hugely significant genetic factor in ADPKD could be related to a fatal renal prognosis that is quite difficult to modify with the currently available therapeutic tools which improve cardiovascular factors. Stimulation of the renin-angiotensin system (RAS) has been associated with a sombre ADPKD prognosis owing to faster growth of the renal cysts . The HALT trial is underway to elucidate the contribution of angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) and the strict control of hypertension to the slowing of decline in renal function . Increased use of RAS-blocking anti-hypertensive drugs could imply that ADPKD patients will start on RRT later and later . However, although ACEIs and ARBs are widely used for ADPKD in our population (specific data not available), this assumption is not in accordance with our findings.
Kidney transplantation is for the moment the best therapeutic choice for ADPKD patients with ESRD. Longer survival of both patient and graft has been observed in recipients of kidney transplants with ADPKD [20, 42], in agreement with our results. This increase in survival could be explained by the ADPKD patients’ younger age, lower rate of diabetes, lower prevalence of dyslipidaemia and anaemia, and also a lower prevalence of co-morbidities, mainly cardiovascular ones. It has not been possible to show that immunological factors may play a role in this survival difference. No explanation for a longer graft survival, other than the own patient’s survival, is available. The relatively low but increasing percentage of pre-emptive living donor transplantation is due to the transplantation policies of Spain, which has a highly successful programme for cadaveric transplantation and is encouraging living donor transplantation to overcome the current relative scarcity of cadaveric donors.
The primary cause of death among ADPKD patients on RRT, in our cohort, is cardiovascular disease, followed by infections, as reported by several other authors [43–45]. Various articles have shown higher survival of ADPKD patients on RRT as compared to non-ADPKD patients, which concurs with our data [46, 47]. We found no differences as to the age of death, even though ADPKD patients started RRT earlier than non-ADPKD patients; this indicates a longer survival of ADPKD patients on RRT. Longer survival of ADPKD patients has been associated with: starting RRT at a younger age, lower prevalence of the cardiovascular risk factors and higher haemoglobin levels, all of which are factors applicable to our results [48, 49]. In another study, ADPKD patients on RRT still showed a higher survival rate than diabetes-free non-ADPKD patients , though such a difference was not observed by other authors .
Over the last few years initiation of RRT has been delayed in non-ADPKD patients owing to cardiovascular risk factors being better controlled. ADPKD patients have a lower prevalence of cardiovascular risk factors and also less co-morbidity, which favours longer survival. Still, over time no changes in age at onset of ESRD have occurred among ADPKD patients who have started RRT, probably because of the impact of unmodifiable genetic factors in the absence of a specific treatment. However, there is reasonable hope that disease progression in patients with ADPKD can be delayed with newer therapeutic approaches which are currently being tested.
RT chairs the Inherited Renal Diseases Unit at Fundació Puigvert. She also chairs the working group on inherited kidney disorders within the Spanish Society of Nephrology and is the President of the Scientific Committee for the AIRG-E.
The research group belongs to a Consolidated Research Group (AGAUR 2009/SGR-1116) and to REDINREN (Spanish Renal Network for Research 16/06, RETICS, Instituto de Investigación Carlos III). RT is recipient of an intensification grant from the program I3SNS from the Spanish Ministry of Health. This project was partially financed with grant FIS 12/01523.
- Harris PC: 2008 Homer W. Smith Award: insights into the pathogenesis of polycystic kidney disease from gene discovery. J Am Soc Nephrol. 2009, 20: 1188-1198. 10.1681/ASN.2009010014.View ArticlePubMedGoogle Scholar
- Torra R, Badenas C, Darnell A, Nicolau C, Volpini V, Revert L, et al: Linkage, clinical features, and prognosis of autosomal dominant polycystic kidney disease types 1 and 2. J Am Soc Nephrol. 1996, 7: 2142-2151.PubMedGoogle Scholar
- Hateboer N, Dijk MA, Bogdanova N, Coto E, Saggar-Malik AK, San Millan JL, et al: Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet. 1999, 353: 103-107. 10.1016/S0140-6736(98)03495-3.View ArticlePubMedGoogle Scholar
- Collins AJ, Foley RN, Herzog C, Chavers B, Gilbertson D, Ishani A, et al: US Renal Data System 2010 Annual Data Report. Am J Kidney Dis. 2011, 57: A8, e1-A8,526.View ArticleGoogle Scholar
- Catalan Transplant Organisation HD: Catalan Renal Registry. Generalitat de Catalunya. 2009, Barcelona: Generalitat de Catalunya, Servei Catala de la Salut, Statistical report. 20-11-2011. Ref Type: CatalogGoogle Scholar
- Taylor M, Johnson AM, Tison M, Fain P, Schrier RW: Earlier diagnosis of autosomal dominant polycystic kidney disease: importance of family history and implications for cardiovascular and renal complications. Am J Kidney Dis. 2005, 46: 415-423. 10.1053/j.ajkd.2005.05.029.View ArticlePubMedGoogle Scholar
- Audrezet MP, Cornec-Le GE, Chen JM, Redon S, Quere I, Creff J, et al: Autosomal dominant polycystic kidney disease: comprehensive mutation analysis of PKD1 and PKD2 in 700 unrelated patients. Hum Mutat. 2012, 33: 1239-1250. 10.1002/humu.22103.View ArticlePubMedGoogle Scholar
- Cornec-Le GE, Audrezet MP, Chen JM, Hourmant M, Morin MP, Perrichot R, et al: Type of PKD1 Mutation Influences Renal Outcome in ADPKD. J Am Soc Nephrol. 2013, 24: 1006-1013. 10.1681/ASN.2012070650.View ArticleGoogle Scholar
- Chapman AB, Stepniakowski K, Rahbari-Oskoui F: Hypertension in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis. 2010, 17: 153-163. 10.1053/j.ackd.2010.01.001.View ArticlePubMedPubMed CentralGoogle Scholar
- Higashihara E, Horie S, Muto S, Mochizuki T, Nishio S, Nutahara K: Renal disease progression in autosomal dominant polycystic kidney disease. Clin Exp Nephrol. 2012, 16: 622-628. 10.1007/s10157-012-0611-9.View ArticlePubMedPubMed CentralGoogle Scholar
- Longenecker JC, Coresh J, Powe NR, Levey AS, Fink NE, Martin A, et al: Traditional cardiovascular disease risk factors in dialysis patients compared with the general population: the CHOICE Study. J Am Soc Nephrol. 2002, 13: 1918-1927. 10.1097/01.ASN.0000019641.41496.1E.View ArticlePubMedGoogle Scholar
- Haroun MK, Jaar BG, Hoffman SC, Comstock GW, Klag MJ, Coresh J: Risk factors for chronic kidney disease: a prospective study of 23,534 men and women in Washington County, Maryland. J Am Soc Nephrol. 2003, 14: 2934-2941. 10.1097/01.ASN.0000095249.99803.85.View ArticlePubMedGoogle Scholar
- Khan SS, Kazmi WH, Abichandani R, Tighiouart H, Pereira BJ, Kausz AT: Health care utilization among patients with chronic kidney disease. Kidney Int. 2002, 62: 229-236. 10.1046/j.1523-1755.2002.00432.x.View ArticlePubMedGoogle Scholar
- Molnar MZ, Lukowsky LR, Streja E, Dukkipati R, Jing J, Nissenson AR, et al: Blood pressure and survival in long-term hemodialysis patients with and without polycystic kidney disease. J Hypertens. 2010, 28: 2475-2484.PubMedPubMed CentralGoogle Scholar
- Reed BY, McFann K, Reza BM, Nobkhthaghighi N, Masoumi A, Johnson AM, et al: Variation in age at ESRD in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2008, 51: 173-183. 10.1053/j.ajkd.2007.10.037.View ArticlePubMedPubMed CentralGoogle Scholar
- Orskov B, Romming S, Feldt-Rasmussen B, Strandgaard S, V: Improved Prognosis in Patients with Autosomal Dominant Polycystic Kidney Disease in Denmark. Clin J Am Soc Nephrol. 2010, 5: 2034-2039. 10.2215/CJN.01460210.View ArticlePubMedPubMed CentralGoogle Scholar
- Schrier RW, McFann KK, Johnson AM: Epidemiological study of kidney survival in autosomal dominant polycystic kidney disease. Kidney Int. 2003, 63: 678-685. 10.1046/j.1523-1755.2003.00776.x.View ArticlePubMedGoogle Scholar
- Patch C, Charlton J, Roderick PJ, Gulliford MC: Use of antihypertensive medications and mortality of patients with autosomal dominant polycystic kidney disease: a population-based study. Am J Kidney Dis. 2011, 57: 856-862. 10.1053/j.ajkd.2011.01.023.View ArticlePubMedGoogle Scholar
- Stewart JH: End-stage renal failure appears earlier in men than in women with polycystic kidney disease. Am J Kidney Dis. 1994, 24: 181-183.View ArticlePubMedGoogle Scholar
- Jacquet A, Pallet N, Kessler M, Hourmant M, Garrigue V, Rostaing L, et al: Outcomes of renal transplantation in patients with autosomal dominant polycystic kidney disease: a nationwide longitudinal study. Transpl Int. 2011, 24: 582-587. 10.1111/j.1432-2277.2011.01237.x.View ArticlePubMedGoogle Scholar
- Jager KJ, van Dijk PC, Dekker FW, Stengel B, Simpson K, Briggs JD: The epidemic of aging in renal replacement therapy: an update on elderly patients and their outcomes. Clin Nephrol. 2003, 60: 352-360. 10.5414/CNP60352.View ArticlePubMedGoogle Scholar
- Gretz N, Zeier M, Geberth S, Strauch M, Ritz E: Is gender a determinant for evolution of renal failure? A study in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 1989, 14: 178-183.View ArticlePubMedGoogle Scholar
- Wakai K, Nakai S, Kikuchi K, Iseki K, Miwa N, Masakane I, et al: Trends in incidence of end-stage renal disease in Japan, 1983–2000: age-adjusted and age-specific rates by gender and cause. Nephrol Dial Transplant. 2004, 19: 2044-2052. 10.1093/ndt/gfh317.View ArticlePubMedGoogle Scholar
- Schrier R, McFann K, Johnson A, Chapman A, Edelstein C, Brosnahan G, et al: Cardiac and renal effects of standard versus rigorous blood pressure control in autosomal-dominant polycystic kidney disease: results of a seven-year prospective randomized study. J Am Soc Nephrol. 2002, 13: 1733-1739. 10.1097/01.ASN.0000018407.60002.B9.View ArticlePubMedGoogle Scholar
- Abbott KC, Agodoa LY: Polycystic kidney disease at end-stage renal disease in the United States: patient characteristics and survival. Clin Nephrol. 2002, 57: 208-214. 10.5414/CNP57208.View ArticlePubMedGoogle Scholar
- Li L, Szeto CC, Kwan BC, Chow KM, Leung CB, Kam-Tao LP: Peritoneal dialysis as the first-line renal replacement therapy in patients with autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2011, 57: 903-907. 10.1053/j.ajkd.2011.01.019.View ArticlePubMedGoogle Scholar
- Gabow PA, Johnson AM, Kaehny WD, Kimberling WJ, Lezotte DC, Duley IT, et al: Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease. Kidney Int. 1992, 41: 1311-1319. 10.1038/ki.1992.195.View ArticlePubMedGoogle Scholar
- Dicks E, Ravani P, Langman D, Davidson WS, Pei Y, Parfrey PS: Incident renal events and risk factors in autosomal dominant polycystic kidney disease: a population and family-based cohort followed for 22 years. Clin J Am Soc Nephrol. 2006, 1: 710-717. 10.2215/CJN.01581105.View ArticlePubMedGoogle Scholar
- Yium J, Gabow P, Johnson A, Kimberling W, Martinez-Maldonado M: Autosomal dominant polycystic kidney disease in blacks: clinical course and effects of sickle-cell hemoglobin. J Am Soc Nephrol. 1994, 4: 1670-1674.PubMedGoogle Scholar
- Ecder T, Edelstein CL, Fick-Brosnahan GM, Johnson AM, Chapman AB, Gabow PA, et al: Diuretics versus angiotensin-converting enzyme inhibitors in autosomal dominant polycystic kidney disease. Am J Nephrol. 2001, 21: 98-103. 10.1159/000046231.View ArticlePubMedGoogle Scholar
- Ozkok A, Akpinar TS, Tufan F, Kanitez NA, Uysal M, Guzel M, et al: Clinical characteristics and predictors of progression of chronic kidney disease in autosomal dominant polycystic kidney disease: a single center experience. Clin Exp Nephrol. 2012, 17: 345-351.View ArticlePubMedGoogle Scholar
- Helal I, Reed B, Schrier RW: Emergent early markers of renal progression in autosomal-dominant polycystic kidney disease patients: implications for prevention and treatment. Am J Nephrol. 2012, 36: 162-167. 10.1159/000341263.View ArticlePubMedGoogle Scholar
- Torres VE, Grantham JJ, Chapman AB, Mrug M, Bae KT, King BF, et al: Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011, 6: 640-647. 10.2215/CJN.03250410.View ArticlePubMedPubMed CentralGoogle Scholar
- Virzi GM, Corradi V, Panagiotou A, Gastaldon F, Cruz DN, de CM, et al: ADPKD: Prototype of Cardiorenal Syndrome Type 4. Int J Nephrol. 2010, 2011: 490795-PubMedPubMed CentralGoogle Scholar
- Abbott KC, Agodoa LY: Polycystic kidney disease in patients on the renal transplant waiting list: trends in hematocrit and survival. BMC Nephrol. 2002, 3: 7-10.1186/1471-2369-3-7.View ArticlePubMedPubMed CentralGoogle Scholar
- Lukowsky LR, Molnar MZ, Zaritsky JJ, Sim JJ, Mucsi I, Kovesdy CP, et al: Mineral and bone disorders and survival in hemodialysis patients with and without polycystic kidney disease. Nephrol Dial Transplant. 2012, 27: 2899-2907. 10.1093/ndt/gfr747.View ArticlePubMedGoogle Scholar
- Menon V, Rudym D, Chandra P, Miskulin D, Perrone R, Sarnak M: Inflammation, Oxidative Stress, and Insulin Resistance in Polycystic Kidney Disease. Clin J Am Soc Nephrol. 2010, 6: 7-13.View ArticlePubMedGoogle Scholar
- Torra R, Darnell A, Cleries M, Botey A, Revert L, Vela E: Polycystic kidney disease patients on renal replacement therapy: data from the Catalan Renal Registry. Contrib Nephrol. 1995, 115: 177-181.View ArticlePubMedGoogle Scholar
- Helal I, Reed B, Mettler P, Mc FK, Tkachenko O, Yan XD, et al: Prevalence of cardiovascular events in patients with autosomal dominant polycystic kidney disease. Am J Nephrol. 2012, 36: 362-370. 10.1159/000343281.View ArticlePubMedPubMed CentralGoogle Scholar
- Herbelin A, Urena P, Nguyen AT, Zingraff J, scamps-Latscha B: Elevated circulating levels of interleukin-6 in patients with chronic renal failure. Kidney Int. 1991, 39: 954-960. 10.1038/ki.1991.120.View ArticlePubMedGoogle Scholar
- Torres VE, Chapman AB, Perrone RD, Bae KT, Abebe KZ, Bost JE, et al: Analysis of baseline parameters in the HALT polycystic kidney disease trials. Kidney Int. 2012, 81: 577-585. 10.1038/ki.2011.411.View ArticlePubMedGoogle Scholar
- Johnston O, O’Kelly P, Donohue J, Walshe JJ, Little DM, Hickey D, et al: Favorable graft survival in renal transplant recipients with polycystic kidney disease. Ren Fail. 2005, 27: 309-314.View ArticlePubMedGoogle Scholar
- Orskov B, Sorensen VR, Feldt-Rasmussen B, Strandgaard S: Changes in causes of death and risk of cancer in Danish patients with autosomal dominant polycystic kidney disease and end-stage renal disease. Nephrol Dial Transplant. 2012, 27: 1607-1613. 10.1093/ndt/gfr467.View ArticlePubMedGoogle Scholar
- Fick GM, Johnson AM, Hammond WS, Gabow PA: Causes of death in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1995, 5: 2048-2056.PubMedGoogle Scholar
- Rahman E, Niaz FA, Al-Suwaida A, Nahrir S, Bashir M, Rahman H, et al: Analysis of causes of mortality in patients with autosomal dominant polycystic kidney disease: a single center study. Saudi J Kidney Dis Transpl. 2009, 20: 806-810.PubMedGoogle Scholar
- Haynes R, Kheradmand F, Winearls CG: Survival after starting renal replacement treatment in patients with autosomal dominant polycystic kidney disease: a single-centre 40-year study. Nephron Clin Pract. 2012, 120: c42-c47. 10.1159/000334429.View ArticlePubMedGoogle Scholar
- Roscoe JM, Brissenden JE, Williams EA, Chery AL, Silverman M: Autosomal dominant polycystic kidney disease in Toronto. Kidney Int. 1993, 44: 1101-1108. 10.1038/ki.1993.355.View ArticlePubMedGoogle Scholar
- Alam A, Perrone RD: Management of ESRD in patients with autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis. 2010, 17: 164-172. 10.1053/j.ackd.2009.12.006.View ArticlePubMedGoogle Scholar
- Fourtounas C, Panteris V, Valis D: Survival after end-stage renal disease in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2002, 39: 660-View ArticlePubMedGoogle Scholar
- Perrone RD, Ruthazer R, Terrin NC: Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality. Am J Kidney Dis. 2001, 38: 777-784. 10.1053/ajkd.2001.27720.View ArticlePubMedGoogle Scholar
- Lee PW, Chien CC, Yang WC, Wang JJ, Lin CC: Epidemiology and mortality in dialysis patients with and without polycystic kidney disease: a national study in Taiwan. J Nephrol. 2012, 12 (26): 755-762.Google Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2369/14/186/prepub
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.