Exception Policy Change Increased the Simultaneous Kidney-Liver Transplant Probability of Polycystic Disease in the Centers With High Median MELD at Transplantation.

BACKGROUND: In 2019, Organ Procurement and Transplantation Network/United Network for Organ Sharing changed the exception policy for liver allocation to the median model for end-stage liver disease at transplantation (MMaT). This study evaluated the effects of this change on-waitlist outcomes of simultaneous liver-kidney transplantation (SLKT) for patients with polycystic liver-kidney disease (PLKD). METHODS: Using the Organ Procurement and Transplantation Network/United Network for Organ Sharing registry, 317 patients with PLKD listed for SLKT between January 2016 and December 2021 were evaluated. Waitlist outcomes were compared between prepolicy (Era 1) and postpolicy (Era 2) eras. RESULTS: One-year transplant probability was significantly higher in Era 2 than in Era 1 (55.7% versus 37.9%; P  = 0.001), and the positive effect on transplant probability of Era 2 was significant after risk adjustment (adjusted hazard ratio, 1.76; 95% confidence interval, 1.22-2.54; P  = 0.002 [ref. Era 1]), whereas waitlist mortality was comparable. Transplant centers were separated into the high and low MMaT groups with a score of 29 (median MMaT) and transplant probability in each group between eras was compared. In the high MMaT transplant centers, the 1-y transplant probability was significantly higher in Era 2 (27.5% versus 52.4%; P  = 0.003). The positive effect remained significant in the high MMaT center group (adjusted hazard ratio, 2.79; 95% confidence interval, 1.43-5.46; P  = 0.003 [ref. Era 1]) but not in the low MMaT center group. Although there was a difference between center groups in Era 1 ( P  = 0.006), it became comparable in Era 2 ( P  = 0.54). CONCLUSIONS: The new policy increased 1-y SLKT probability in patients with PKLD and successfully reduced the disparities based on center location.

African American polycystic kidney patients receive higher risk kidneys, but do not face increased risk for graft failure or post-transplant mortality.

African Americans (AA) are disproportionately affected by end-stage renal disease (ESRD) and have worse outcomes following renal transplantation. Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic condition leading to ESRD necessitating transplant. We explored this population with respect to race by conducting a retrospective analysis of the UNOS database between 2005 and 2019. Our study included 10,842 (AA n = 1661; non-AA n = 9181) transplant recipients whose primary diagnosis was ADPKD. We further stratified the AA ADPKD population with respect to blood groups (AA blood type B n = 295 vs AA non-B blood type n = 1366), and also compared this cohort to AAs with a diagnosis of DM (n = 16,706) to identify unique trends in the ADPKD population. We analyzed recipient and donor characteristics, generated survival curves, and conducted multivariate analyses. African American ADPKD patients waited longer for transplants (924 days vs 747 days P < .001), and were more likely to be on dialysis (76% vs 62%; p < .001). This same group was also more likely to have AA donors (21% vs 9%; p < .001) and marginally higher KDPI kidneys (0.48 vs 0.45; p < .001). AA race was a risk factor for delayed graft function (DGF), increasing the chance of DGF by 45% (OR 1.45 95% CI 1.26-1.67; p < .001). AA race was not associated with graft failure (HR 1.10 95% CI 0.95-1.28; p = .21) or patient mortality (HR 0.84 95% CI 0.69-1.03; p = .09). Racial disparities exist in the ADPKD population. They should be continually studied and addressed to improve transplant equity.

Is Peritoneal Dialysis a Suitable Renal Replacement Therapy Option for Polycystic Kidney Disease Patients?

BACKGROUND/AIMS: Mounting clinical experience and evidence from scale observational studies have suggested that polycystic kidney disease (PKD) was not a contraindication for peritoneal dialysis (PD). Recent studies have reported that PD may be associated with a better prognosis in PKD than that of non-PKD patients. To solve the problem, we performed a systematic review and comprehensive meta-analysis to compare the outcomes between PKD and non-PKD patients on PD and the all-cause mortality between patients with PKD on PD and hemodialysis (HD). METHODS: We conducted a systematic literature using electronic databases (PubMed, Ovid, Embase and Web of Science) to identify the studies reporting the endpoint events of PKD/non-PKD patients with PD and the all-cause mortality between patients with PKD on PD and HD, such as dialysis adequacy, technique failure, PD-related complications, the mode of RRT change, and all-cause mortality. We searched the literature published February 2018 or earlier. We used both fix-effects and random-effects models to calculate the overall effect estimate. A sensitivity analysis and subgroup analysis were performed to find the origin of heterogeneity. RESULTS: 12 studies with a total of 17,040 patients reported the endpoint events of PKD/non-PKD patients with PD. No significant difference was observed on dialysis adequacy (Kt/V, SMD: -0.02, 95%CI: -0.12-0.08; D: Pcr (4h), SMD: -0.10, 95% CI: -0.26-0.06), technique failure (RR: 0.97, 95%CI: 0.78-1.20), RRT change (RR: 0.96, 95%CI: 0.77-1.19), total PD-associated complications (RR: 1.0, 95%CI: 0.91-1.09) and all-cause mortality (RR: 0.40, 95%CI: 0.33-0.47) in PKD patients, compared with non-PKD subjects undergoing PD. However, the proportion of renal transplantation in PKD patients was higher than that of non-PKD patients (RR: 2.04, 95%CI: 1.88-2.20) with significant heterogeneity (I2 =82.7%, P=0.000). 4 studies with a total of 5,762 patients reported that the all-cause mortality did not differ between the PKD patients on PD and HD (RR: 0.87, 95%CI: 0.72-1.06). CONCLUSION: Our meta-analysis found that the outcomes of given population of PKD patients on PD were at least not inferior as compared to those with other primary kidney diseases, and suggested that PKD might be not absolutely a contraindication for PD. Given the limitations of the proposed, it needs further large-scale studies to assess whether PD is a suitable RRT option for end-stage renal disease (ESRD) patients with PKD.

Dialysis modality and mortality in polycystic kidney disease.

INTRODUCTION: Identifying the appropriate modality between hemodialysis (HD) and peritoneal dialysis (PD) is an unresolved issue in polycystic kidney disease (PKD) patients. This study aims to illustrate whether the mortality and survival are different among individuals receiving HD comparing PD. METHODS: We searched PubMed, EMBASE, and China National Knowledge Infrastructure about cohort studies involving PKD patients with end stage renal disease and comparing HD with PD. We calculated pooled risk ratios (RRs) with 95% confidence intervals (CIs) for dichotomous data. Primary outcomes were the mortality and survival (not the technique survival). FINDINGS: Eight studies involving 7685 PKD patients were identified. There were no significant differences in mortality (RR 1.06, 95% CI 0.84-1.34, P = 0.61) and 5-year survival rate (RR 0.99, 95% CI 0.80-1.22, P = 0.90) between HD and PD. PD was not associated with higher risks of abdominal infections and hernia. However, HD was associated with a higher incidence of renal cyst hemorrhage (RR 2.53, 95% CI 1.05-6.12, P = 0.04). DISCUSSION: There are no significant differences of mortality and survival between different dialysis modalities in PKD. PD may be as successful as HD for PKD patients receiving renal replacement therapy.

Severity of diverticulitis in patients with polycystic kidney disease without transplantation.

BACKGROUND: Patients with polycystic kidney disease (PKD) who have had a kidney transplant have an increased risk of diverticular disease and complicated diverticulitis. Literature is limited regarding the severity of diverticulitis in patients with PKD who have not had a transplant. We aim to assess whether patients with PKD, with and without renal transplant, have a similar course of diverticulitis. METHODS: A retrospective review of all adult PKD patients at our institution diagnosed with diverticulitis between 2000 and 2016 was conducted. Patients without documented PKD and diverticulitis were excluded. We compared PKD patients with and without renal transplantation. RESULTS: A total of 41 patients were identified. Mean age was 60 (± 12), and 56% were female. Fourteen patients had undergone renal transplantation. Five (19%) non-transplant patients had complicated diverticulitis, compared to 43% (n = 6) transplanted (p = 0.33). Fifteen (56%) non-transplant and 8 (57%) transplant patients had recurrent diverticulitis (p = 1.00). Three (11%) non-transplant and 5 (36%) transplanted patients had recurrent complicated diverticulitis. Eight (30%) non-transplant and 7 (50%) transplant patients underwent surgery (p = 0.31). All 8 non-transplant patients underwent sigmoid resection with primary anastomosis without diversion. In the transplant group, 3 Hartmann procedures and 1 sigmoid resection with and 3 without diversion were performed. There was one in-hospital death in each group. CONCLUSION: In our group of patients, there was no difference in rate of recurrent diverticulitis, diverticulitis complications, or operative intervention in patients with PKD with and without renal transplant. The renal transplant group had a higher rate of recurrent, complicated diverticulitis.

Incidence and survival of end-stage kidney disease due to polycystic kidney disease in Australia and New Zealand (1963-2014).

BACKGROUND: The aim of this study was to determine whether the incidence and survival of patients with end-stage kidney disease (ESKD) due to polycystic kidney disease (PKD) has changed in Australia and New Zealand. METHODS: Data for all PKD patients who developed ESKD and commenced renal replacement therapy (RRT) was assessed using the Australia and New Zealand Dialysis and Transplant Registry from 1963 to 2014. RESULTS: A total 4678 patients with ESKD due to PKD received RRT during the study period. The incidence rate of ESKD (per million population per year) due to PKD rose by 3.2-fold (1970-2010), but the percentage increase between each decade decreased (54.4, 43.8, 25.6 and 6.57%). The median age of onset of new patients developing ESKD has been stable since 1990. Haemodialysis was the most common initial mode of RRT (between 62 and 76% of patients) whereas 24-29% received peritoneal dialysis. The 5-year survival rate of PKD patients on RRT (censored for transplantation and adjusted for age) improved from 26 to 84%, with the percentage increase between each successive time period being 123, 7, 21, 19 and 7.4%. The percentage of deaths on RRT due to cerebrovascular disease declined from 15 to 6%. CONCLUSIONS: The incidence and age of onset of ESKD due to PKD has remained unchanged in the modern era though patient survival on RRT has continued to improve. These data suggest that the development and implementation of disease-specific treatments prior to RRT is needed to effectively diminish the incidence of ESKD due to PKD.

Is peritoneal dialysis a therapeutic option for polycystic kidney disease? 15 years' experience in a single center.

BACKGROUND: Peritoneal dialysis (PD) is often avoided for patients with polycystic kidney disease (PKD) because of increased risk of complications and technique failure due to limited intra-abdominal space. In this study, we have aimed to determine clinical outcomes, patient and technique survivals in patients with PKD performing PD and to define whether PD is appropriate for these patients. METHODS: Totally 99 patients: 33 with PKD and 66 with diseases other than PKD were included in this retrospective study. All patients started PD between 2001 and 2015 years and have been matched by time of PD therapy initiation. Socio-demographic characteristics, clinical data and complications during the specified period were evaluated. The factors associated with mortality and patient and technique survival were investigated for all patients. RESULTS: The two groups were similar in terms of demographic, baseline and last visit clinical and laboratory parameters, additional systemic diseases, with the exception of higher pretreatment and last visit serum albumin levels in PKD patients (P=0.03 and 0.01 respectively) and younger age of non-PKD patients (P=0.002). Incidence of peritonitis and catheter exit-site/tunnel infections were similar among the two groups (P=0.26 and 0.12 respectively). The two groups were similar in terms of leak and hernia developments (P=0.07 and 0.57, respectively). By the end of the study period; in PKD group, 10 patients had been transferred to HD and had kidney transplantation and only 6 patients had died. In non-PKD group, 19 patients had been transferred to HD, 11 patients had kidney transplantation and 23 patients had died. Mortality was lower in PKD group (log rank=0.034). The two groups were similar regarding death and HD transfer reasons (P=0.35 and 0.36 respectively). The technique survival rates were similar among the two groups (log rank=0.37). CONCLUSIONS: Peritoneal dialysis may be a suitable renal replacement therapy option for PKD patients. PKD is not an additional risk factor in patients treated by PD. Mortality is similar with non-diabetic PD patients. Peritoneal dialysis in PKD patients is associated with a similar overall rate of technique survival, incidences of hernia, leak and infectious complications as in non-PKD patients.

Outcome Comparisons Between Patients on Peritoneal Dialysis With and Without Polycystic Kidney Disease: A Nationwide Matched Cohort Study.

Polycystic kidney disease (PCKD) is the most common hereditary cause of end-stage renal disease. The complications associated with this disease may affect the performance of peritoneal dialysis (PD). The aim of this study was to compare the outcomes between patients on PD with PCKD and without PCKD.We extracted an incident cohort of adult (≥ 20 years old) patients on long-term PD from the Taiwan National Health Insurance Research Database. Patients with PCKD were identified by specific diagnosis codes. We recorded baseline comorbidities, socioeconomic status, timing of referral to a nephrologist, prior hemodialysis history before PD, and the type of PD modalities. We compared the risk of death, technique failure, peritonitis, hospitalization, and outpatient visiting as well as overall medical expenditure between the patients with PCKD and a groups of patients without PCKD who were propensity-score matched (1:3). The analysis was carried out by various Cox regression models that considered competing risk and time-varying coefficients. We enrolled 139 patients with PCKD and 7739 patients without PCKD who started long-term PD between 1999 and 2010. Patients with PCKD were less comorbid and more often treated with automated PD. In the propensity-score matched analysis, both overall survival and technique survival did not differ between the patients and the result was similar for hospitalization and peritonitis after adjusting for the application of automated PD. Furthermore, the overall annual medical expenditures were similar between the patients with and without PCKD. PD patients with PCKD are comparable to PD patients without PCKD in terms of risk of death, peritonitis, technique failure, and hospitalization in the present study. Furthermore, the medical expenses of the 2 groups after initiation of PD are also indistinguishable.

Comparative Study of Outcomes among Patients with Polycystic Kidney Disease on Hemodialysis and Peritoneal Dialysis.

Polycystic kidney disease (PCKD) is the most common hereditary cause of end-stage renal disease, the complications of which may prevent the choice of peritoneal dialysis (PD). The aim of this study was to explore the effects of dialysis modality on outcomes in patients with PCKD. We extracted a cohort of 1417 adult patients with PCKD initiating long-term dialysis therapy in 1999-2010 from the Taiwan National Health Insurance Research Database, among which 125 patients chose PD. The patients on HD were older and had a higher comorbidity index compared to those on PD. We compared the risks for death, hospitalization and medical expenditures between the patients on PD and propensity-score matched patients on hemodialysis (HD). The overall survival did not differ between the patients on PD and HD. The patients on PD tended to have higher hazard ratios (HR) for the first episode of hospitalization (adjusted HR 1.34 [95% CI, 1.04-1.79]). The annual medical expenses were 10% lower for the patients on PD. PD is an equivalent choice of renal replacement therapy to HD for patients with PCKD in terms of survival. Although the patients on PD had a higher risk for hospitalization, the medical expenditure for PD was 10% lower.

Meckel-Gruber Syndrome: a population-based study on prevalence, prenatal diagnosis, clinical features, and survival in Europe.

Meckel-Gruber Syndrome is a rare autosomal recessive lethal ciliopathy characterized by the triad of cystic renal dysplasia, occipital encephalocele and postaxial polydactyly. We present the largest population-based epidemiological study to date using data provided by the European Surveillance of Congenital Anomalies (EUROCAT) network. The study population consisted of 191 cases of MKS identified between January 1990 and December 2011 in 34 European registries. The mean prevalence was 2.6 per 100,000 births in a subset of registries with good ascertainment. The prevalence was stable over time, but regional differences were observed. There were 145 (75.9%) terminations of pregnancy after prenatal diagnosis, 13 (6.8%) fetal deaths, 33 (17.3%) live births. In addition to cystic kidneys (97.7%), encephalocele (83.8%) and polydactyly (87.3%), frequent features include other central nervous system anomalies (51.4%), fibrotic/cystic changes of the liver (65.5% of cases with post mortem examination) and orofacial clefts (31.8%). Various other anomalies were present in 64 (37%) patients. As nowadays most patients are detected very early in pregnancy when liver or kidney changes may not yet be developed or may be difficult to assess, none of the anomalies should be considered obligatory for the diagnosis. Most cases (90.2%) are diagnosed prenatally at 14.3 ± 2.6 (range 11-36) gestational weeks and pregnancies are mainly terminated, reducing the number of LB to one-fifth of the total prevalence rate. Early diagnosis is important for timely counseling of affected couples regarding the option of pregnancy termination and prenatal genetic testing in future pregnancies.

Urinary sodium excretion and kidney failure in nondiabetic chronic kidney disease.

Current guidelines recommend under 2 g/day sodium intake in chronic kidney disease, but there are a few studies relating sodium intake to long-term outcomes. Here we evaluated the association of mean baseline 24-h urinary sodium excretion with kidney failure and a composite outcome of kidney failure or all-cause mortality using Cox regression in 840 participants enrolled in the Modification of Diet in Renal Disease Study. Mean 24-h urinary sodium excretion was 3.46 g/day. Kidney failure developed in 617 participants, and the composite outcome was reached in 723. In the primary analyses, there was no association between 24-h urine sodium and kidney failure (HR 0.99 (95% CI 0.91-1.08)) nor on the composite outcome (HR 1.01 (95% CI 0.93-1.09)), each per 1 g/day higher urine sodium. In exploratory analyses, there was a significant interaction of baseline proteinuria and sodium excretion with kidney failure. Using a two-slope model, when urine sodium was under 3 g/day, higher urine sodium was associated with increased risk of kidney failure in those with baseline proteinuria under 1 g/day and with lower risk of kidney failure in those with baseline proteinuria of ⩾ 1 g/day. There was no association between urine sodium and kidney failure when urine sodium was ⩾ 3 g/day. Results were consistent using first baseline and time-dependent urinary sodium excretion. Thus, we noted no association of urine sodium with kidney failure. Results of the exploratory analyses need to be verified in additional studies and the mechanism explored.

Iron indices and survival in maintenance hemodialysis patients with and without polycystic kidney disease.

BACKGROUND: Anemia is less prominent in patients with polycystic kidney disease (PKD). Such iron indices as ferritin and transferrin saturation (TSAT) values are used to guide management of anemia in individuals on maintenance hemodialysis (MHD). Optimal levels of correction of anemia and optimal levels of TSAT and ferritin are unclear in chronic kidney disease patients and have not been studied specifically in PKD. METHODS: We studied 2969 MHD patients with and 128 054 patients without PKD from 580 outpatient hemodialysis facilities between July 2001 and June 2006. Using baseline, time-dependent and time-averaged values with unadjusted and multivariable adjusted analysis models, the survival predictabilities of TSAT and ferritin were studied. RESULTS: PKD patients were 58 ± 13 years old and included 46% women, whereas non-PKD patients were 62 ± 15 years old and 45% women. In both PKD and non-PKD patients, a time-averaged TSAT between 30 and 40% was associated with the lowest mortality. Time-averaged ferritin between 100 and <800 ng/mL was associated with the lowest mortality in PKD patients, although this range was 500 to <800 ng/mL in non-PKD patients. CONCLUSIONS: In MHD patients with and without PKD, there was a U-shaped relationship between the average TSAT and mortality, and a TSAT of 30-40% was associated with the best survival. However, an average ferritin of 100-800 ng/mL was associated with the best survival in PKD patients, whereas that of non-PKD patients was 500-800 ng/mL. Further studies in PKD and non-PKD patients are necessary to determine whether or not therapeutic attempts to keep TSAT and ferritin levels in these ranges will improve survival.

Epidemiology and mortality in dialysis patients with and without polycystic kidney disease: a national study in Taiwan.

BACKGROUND: Polycystic kidney disease (PCKD) is one of the most common inherited disorders in end-stage renal disease patients. It is generally thought that the survival of PCKD patients undergoing dialysis surpasses that of general dialysis patients, but the cause of this improved survival is not clear. METHODS: Using Taiwan's national health insurance claims data, we performed a longitudinal cohort study to investigate the survival and impact of comorbidities on mortality in dialysis patients with and without PCKD. We excluded patients without diabetes mellitus (DM) in a further analysis. A Cox proportional hazards model was used to identify the risk factors for all-cause mortality. The Kaplan-Meier method was used to describe overall patient survival. RESULTS: Five hundred and one (2.25%) of 22,298 nondiabetic incident dialysis patients had PCKD. We found no significant difference in survival rates between those with and without PCKD. Being male, being over 65 years old and having congestive heart failure or cerebrovascular accident were each found to be independent predictors of mortality in the PCKD dialysis patients. CONCLUSIONS: Taiwan has a lower incidence rate of PCKD than Western countries. In Taiwan, there is little difference in the long-term survival between dialysis patients with and without PCKD.

Hemoglobin level and survival in hemodialysis patients with polycystic kidney disease and the role of administered erythropoietin.

Interventional trials indicate adverse outcomes when hemoglobin >13 g/dL is targeted in patients with chronic kidney disease (CKD) who receive erythropoiesis-stimulating agents (ESAs). It is not clear whether high-achieved hemoglobin with minimal to no ESA administration as observed in some patients with polycystic kidney disease (PKD) is also associated with poor outcomes. Survival models were examined to assess the association between hemoglobin increments and mortality in a 6-year cohort of 2,402 PKD and 110,875 non-PKD hemodialysis patients across infrequent versus frequent ESA therapy defined as ESA < 25% of cohort time versus otherwise, respectively. Mortality risk was estimated by Cox proportional regression [hazard ratio (HR) and 95% of confidence interval] analysis. Patients with PKD were aged 58 ± 13 years and included 46% women 14% Blacks, respectively. Fully adjusted death HRs of time-averaged hemoglobin increments <11.0, 12.0 to <13.0 g/dL (reference: 11.0 to <12.0 g/dL) for frequent ESA therapy were 2.57 (1.48-4.48), 0.60 (0.43-0.82), and 0.81 (0.50-1.29), whereas for infrequent ESA therapy they were 1.33 (0.47-3.78), 0.28 (0.13-0.61), and 0.22 (0.09-0.57), respectively. Hence, in patients with PKD who require infrequent ESA, incrementally higher achieved hemoglobin including > 13.0 g/dL exhibits better survival; this incremental survival gain of higher hemoglobin is not observed in patients with PKD receiving frequent ESA administration, in whom hemoglobin concentration > 13 exhibits increased mortality.

Peritoneal dialysis in polycystic kidney disease patients. Report from the French peritoneal dialysis registry (RDPLF).

BACKGROUND: It is commonly believed that polycystic kidney disease (PKD) patients on peritoneal dialysis (PD) are over-exposed to technique failure and peritonitis compared with other patients. This study was carried out to assess whether PKD is associated with technique failure and to evaluate the outcome of PKD patients on PD. METHODS: This was a retrospective cohort study based on the data of the French Language Peritoneal Dialysis Registry. We analysed 4162 incident non-diabetic patients who started PD between January 2002 and December 2007. The end of the observation period was 31 December 2008. RESULTS: Among 4162 patients, there were 344 PKD patients and 3818 patients who had another underlying nephropathy. PKD patients were younger, had a lower Charlson comorbidity index, were more frequently treated by automated PD and were less frequently assisted than other patients. For the PKD patients, the main reason for PD cessation was renal transplantation. In the multivariate analysis, comorbidities and centre size were associated with technique survival, and no association between PKD and technique failure was observed. There was no statistically significant association between PKD and peritonitis or between PKD and enteric peritonitis. On multivariate analysis, patient survival was associated with PKD and with patient age, comorbidities and the modality of assistance. Centre size was not associated with patient survival. CONCLUSION: PD is a suitable method for at least a subgroup of PKD patients reaching end-stage renal disease in a country where renal transplantation is available.

[Combined liver-kidney and kidney after liver transplantation: indications and experiences from a nephrological perspective at a single center]. / Die kombinierte und sequentielle Leber-Nieren-Transplantation: Indikationen und Zentrumserfahrungen aus Sicht des Nephrologen.

Combined liver-kidney transplantations (CLKT) and kidney after liver transplantations (KALT) are established treatments for patients with end-stage hepatic and renal disease and the number of transplantations has continuously increased over the past few years. The most frequent indications for CLKT in adults are polycystic kidney disease with severe liver involvement and liver cirrhosis of different origins with concomitant chronic kidney failure due to chronic glomerulonephritis or diabetic nephropathy. In children, CLKT is most frequently required due to primary oxalosis type I. At present the main indication for KALT still is calcineurin inhibitor-induced chronic nephrotoxicity, emphasizing the need for a nephron-sparing long-term immunosuppression in liver transplant recipients. Compared to KALT, the indications for CLKT are not as well defined and the decision must therefore be made on a case-by-case basis by a multidisciplinary team of experienced clinicians to avoid unnecessary transplantations of both organs in patients with reversible kidney failure, given the scarcity of organs for transplantation worldwide. In hepatorenal syndrome CLKT should only be considered if the GFR is lower than 20 ml/min for more than three months or if the patient has been on renal replacement treatment for more than one month. In CLKT, there appears to be a certain immunological protection for the kidney transplant by the liver transplant.

Effect of pioglitazone on survival and renal function in a mouse model of polycystic kidney disease.

BACKGROUND/AIMS: Cystic epithelia in polycystic kidney disease display features similar to malignant cells. Thiazolidinediones have been shown to have anti-neoplastic properties, therefore we tested the hypothesis that pioglitazone reduces cyst formation, improves renal function, and prolongs survival in a mouse model of polycystic kidney disease. METHODS: PC-Pkd1-KO mice, which have homozygous mutations of the Pkd1 gene in principal cells, were used. On the day after giving birth, mothers were fed standard mouse chow with or without pioglitazone (30 mg/kg chow). After weaning, the assigned diet was continued. At 1 month of age, blood pressure was measured and animals were sacrificed to determine kidney weight, body weight, and serum urea. Kidneys were evaluated for proliferation using Ki-67, apoptosis using TUNEL analysis, and cyst number using MRI. Survival was observed. RESULTS: Pioglitazone did not alter renal function, cell proliferation, apoptosis, or cyst formation in animals with polycystic kidney disease, however it did increase survival. Pioglitazone reduced blood pressure in PC-Pkd1-KO, but not in controls. CONCLUSION: These findings suggest that pioglitazone may have a unique antihypertensive effect in polycystic kidney disease, and that such an effect may promote improved survival.

Effect of type 2 diabetes on mortality risk associated with end-stage kidney disease.

AIMS/HYPOTHESIS: Patients with end-stage kidney disease (ESKD) and patients with diabetes mellitus experience higher mortality rates than the general population. Whether ESKD imparts the same excess in mortality risk for those with diabetes as it does for those without diabetes is unknown. METHODS: Included in the study were all white patients aged > or =25 years with incident ESKD and type 2 diabetes (n = 4,141) or with incident ESKD but without diabetes (n = 13,289) in Australia from 1991 to 2005, and all the individuals aged > or =25 years without ESKD and with type 2 diabetes (n = 909) or without ESKD without diabetes (n = 10,302) enrolled in the AusDiab Study--a nationwide Australian representative cohort--from 1999 to 2005. Excess mortality was analysed in patients with ESKD by diabetes status, using age-, sex- and diabetes-status-specific standardised mortality ratios (SMRs) in the first 8 years after first renal replacement therapy among ANZDATA patients relative to AusDiab participants. RESULTS: The SMRs in patients with ESKD were, in non-diabetic patients and in those with type 2 diabetes, respectively: 14.2 (95% CI 13.9-14.6) and 10.8 (95% CI 10.4-11.2) (p < 0.01); in people aged <60 years, 28.7 (95% CI 27.2-30.4) and 18.6 (95% CI 17.1-20.4) (p < 0.01); in people aged > or =60 years, 12.5 (95% CI 12.1-12.9) vs 9.7 (95% CI 9.3-10.1) (p < 0.01); in men, 11.0 (95% CI 10.7-11.4) vs 8.9 (95% CI 8.4-9.3) (p < 0.01); and in women, 23.4 (95% CI 22.5-24.3) vs 16.2 (95% CI 15.2-17.3) (p < 0.01). CONCLUSIONS/INTERPRETATION: ESKD was associated with a greater relative increase in mortality in the non-diabetic study populations than in the type 2 diabetes population. Excess mortality was greater among younger people and women.