Lifetime Risk of Hepatorenal Events Assessed in Longitudinal Cohort of Renal Transplant Recipients with HBV Infection

BackgroundKidney transplantation is a treatment option for patients with end-stage renal disease (ESRD) who are infected with hepatitis B virus (HBV). However, the impact of nucleos(t)ide analogues usage on the clinical outcomes in HBV-infected ESRD patients undergoing kidney transplantation is not well understood. This study aimed to assess the outcomes of kidney transplant recipients with HBV infection using real-world data to provide insight into the disease course over time.MethodsA nationwide retrospective longitudinal population-level cohort study was conducted using the National Health Insurance Research Database. The study evaluated patient and allograft survival and kidney-related and liver-related events and identified factors contributing to these events.ResultsOf the 4838 renal transplant recipients in the study, there were no significant differences in graft survival between the HBV-infected and non-infected groups (P = .244). However, the HBV-infected group had suboptimal patient survival compared to the non-infected group (hazard ratio [HR] for overall survival, 1.80; 95% CI 1.40-2.30; P < .001). Diabetes mellitus was associated with a higher re-dialysis rate (HR, 1.71; 95% CI, 1.38-2.12; P < .001) regarding kidney-associated events. For liver-associated events, HBV-infected status (HR, 9.40; 95% CI, 5.66-15.63; P < .001), and age >60 years (HR, 6.90; 95% CI, 3.14-15.19; P < .001) were associated with increased incidence of liver cancer.ConclusionsHepatitis B-infected renal transplant recipients have comparable graft survival but inferior patient survival outcomes due to pre-existing diseases and increasing liver-related complications. The findings of this study can help optimize treatment strategies and improve long-term outcomes for this patient population.     

Association of Human Leukocyte Antigen Haplotypes With End-Stage Renal Disease in Vietnamese Patients Prior to First Transplantation

IntroductionThe prevalence of chronic kidney failure is significantly increasing in Vietnam, causing a burden for health care. This study assessed the relationship of HLA-A, -B, and –DRB1 alleles with end-stage renal disease (ESRD).MethodA retrospective, cross-sectional study and a comparative study using secondary data analysis were conducted on 196 ESRD patients and 187 controls from 2009 to 2017. The patient and donor profiles were collected from medical records, including age, sex, etiology of renal failure, and HLA phenotypes. HLA-A*, -B*, and -DRB1* typing were done by polymerase chain reaction-sequence specific primers.ResultThe most frequent HLA alleles in Vietnamese patients with ESRD were HLA-A*02, -A*11, -B*15, -B*46, -DRB1*04, -DRB1*09, and -DRB1*12. The haplotypes HLA-A*0233 (odds ratio [OR] = 0.40, 95% CI: 0.15–0.98) had a negative association for ESRD. The haplotypes HLA-B*1515 (OR = 4.14, 95% CI: 1.52–11.26) and HLA-DRB1*1212 (OR = 2.01, 95% CI: 1.06–3.81) had a positive association for ESRD. The haplotypes HLA-B*1515 (OR = 4.69, 95% CI: 1.69–13.03) and -DRB1*1212 (OR = 2.15, 95% CI: 1.10–4.21) had a positive association for ESRD related to glomerulonephritis. The HLA-B*1557 (OR = 17.34, 95% CI: 2.70–11.49) had a positive association for ESRD related to hypertension.ConclusionThe haplotypes of HLA class I and II had significant relationships with ESRD. The results of our study should be confirmed in further investigations.     

Pulmonary vascular resistance determines mortality in end‐stage renal disease patients with pulmonary hypertension

The multifactorial etiology of pulmonary hypertension (PH) in end‐stage renal disease (ESRD) includes patients with and without elevated pulmonary vascular resistance (PVR). We explored the prognostic implication of this distinction by evaluating pretransplant ESRD patients who underwent right heart catheterization and echocardiography. Demographics, clinical data, and test results were analyzed. All‐cause mortality data were obtained. Median follow‐up was 4 years. Of the 150 patients evaluated, echocardiography identified 99 patients (66%) with estimated pulmonary artery (PA) systolic pressure > 36 mm Hg, which correlated poorly with mortality (HR = 1.28, 95% CI 0.72‐2.27, P = .387). Right heart catheterization identified 88 (59%) patients with mean PA pressure ≥ 25 mm Hg. Of these, 70 had PVR ≤ 3 Wood units and 18 had PVR > 3 Wood units. Survival analysis demonstrated a significant prognostic effect of an elevated PVR in patients with high mean PA pressures (HR = 2.26, 95% CI 1.07‐4.77, P = .03), while patients with high mean PA pressure and normal PVR had equivalent survival to those with normal PA pressure. Despite the high prevalence of PH in ESRD patients, elevated PVR is uncommon and is a determinant of prognosis in patients with PH. Patients with normal PVR had survival equivalent to those with normal PA pressures.     

Reduced complication rate after implementation of a detailed treatment protocol for percutaneous endoscopic gastrostomy with T-fastener fixation in pediatric patients: A prospective study

Background/aimsPercutaneous endoscopic gastrostomy with push technique (PEG-T) is increasingly used in pediatric patients. In a retrospective study of PEG-T (cohort 1) we reported frequent complications related to T-fasteners and tube dislodgment. The aim of this study was to assess complications after implementation of a strict treatment protocol, and to compare these with the previous retrospective study.Materials and methodsThe study is a prospective study of PEG-T placement performed between 2017 and 2020 (cohort 2) in pediatric patients (0–18 years). Complications were recorded during hospital stay, fourteen days and three months postoperatively, graded according to the Clavien-Dindo classification and categorized as early (<30 days) or late (>30 days).ResultsIn total 82 patients were included, of which 52 (60%) had neurologic impairments. Median age and weight were 2.0 years [6 months-18.1 years] and 13.4 kg [3.5–51.5 kg], respectively. There was a significant reduction in median operating time from 28 min [10–65 min] in cohort 1 to 15 min [6–35 min] in cohort 2 (p<0.001), number of patients with early tube dislodgement (cohort 1: 9 (10%) vs cohort 2: 1 (1%), p = 0.012), and number of patients with late migrated T-fasteners (cohort 1: 11 (13%) vs cohort 2: 1 (1%), p = 0.004).ConclusionWe experienced less migrated T-fasteners and tube dislodgment after implementation of strict treatment protocol.Level of evidenceTreatment study level III.     

Cause of End-Stage Renal Disease Is Not a Risk Factor for Cytomegalovirus Infection After Kidney Transplant

BackgroundCytomegalovirus infection (CMV) after kidney transplantation leads to increased morbidity and mortality. Whether the cause of end-stage renal disease (ESRD) influences the risk of CMV infection post-transplant is not known.MethodsWe analyzed data from 2741 adult kidney transplant recipients from January 1993 through December 2014. The causes of ESRD included diabetes mellitus (n = 947), hypertension (n = 442), polycystic kidney disease (n = 549), and glomerulonephritis (GN) (n = 803). The primary outcome was incidence of CMV infection, defined as the first episode of detectable CMV DNA in the blood following transplant.ResultsThree hundred and thirty patients developed a CMV infection over a median follow-up of 4.5 years. Patients with diabetes mellitus (DM) as the cause of ESRD had a higher incidence of CMV infection post-transplant compared to patients with GN (2.37 vs 1.58/100 person-years, P < .005) whereas hypertension (HTN) and autosomal dominant polycystic kidney disease (PKD) were similar (2.17 and 2.07/100 person-years). DM was associated with a 35% higher risk of CMV infection compared to GN in unadjusted analyses [hazard ratio=1.35 [95% confidence interval 1.02–1.78], P = .04). However, after adjustment for age, the risk of CMV infection was similar in all groups (DM: age-adjusted hazard ratio 1.02 [0.78–1.39]; HTN: 0.96 (0.67–1.36); PKD: 1.08 [0.78–1.48]; compared to GN). The risk of CMV infection increased with age (adjusted hazard ratio=1.32 [1.18–1.47] for every decade of life, P < .001).ConclusionsOur study demonstrates that the cause of ESRD is not a significant risk factor for CMV infection in kidney transplant recipients once adjusted for age. Future studies are needed to identify risk factors for CMV infection to define patient-centered monitoring and prevention.     

Outcomes of Older Patients in the Recent Era of Heart Kidney Transplantation

Background and ObjectiveVariable age thresholds are often used at transplant centers for simultaneous heart and kidney transplantation (HKT). We hypothesize that selected older recipients enjoy comparable outcome to younger recipients in the current era of HKT.MethodsWe performed a retrospective analysis of HKT outcomes in the United Network for Organ Sharing (UNOS) registry from 2006 to 2018, classifying patients by age at transplant as ≥ 65 or < 65 years. The primary outcome was patient death. Secondary outcomes included all-cause kidney graft failure and death-censored kidney allograft failure.ResultsOf 973 patients, 774 (80%) were younger than 65 years (mean 52 ± 10 years) and 199 (20%) were 65 years or older (mean 67 ± 2 years). The older HKT cohort had fewer blacks (22% vs 35%, P = .01) and women (12 vs 18%, P = .04). Fewer older patients received dialysis (30% vs 54%, P < .001) and mechanical support (36% vs 45%, P = .03) before HKT. Older recipients received organs from slightly older donors. The median follow-up time was shorter for patients 65 years or older than for the younger group (2.3 vs 3.3 years, P < .001). Patient survival was similar between the groups (mean 8.8 vs 9.8 years, P = .3), with the most common causes of death being cardiovascular (29%) and infectious complications (28%). There was no difference in all-cause kidney graft survival (mean 8.7 vs 9.3 years, P = .8). Most commonly, recipients died with a functional renal allograft (59.8%), and this occurred more commonly in older patients (81.4% vs 54.8%, P = .001). Cox proportional hazard modeling showed that higher donor age (hazard ratio [HR] 1.015, P = .01; HR 1.022, P = .02) and use of pre-transplant dialysis (HR 1.5, P = .004; HR 1.8, P = .006) increased the risk for both all-cause and death-censored kidney allograft failure, respectively.ConclusionsOur study showed that carefully selected older patients have outcomes similar to those of a younger cohort and argues for comprehensive evaluation of the recipients with age as part of comorbidity assessment rather than use of an arbitrary age threshold for candidacy.     

Primary hyperoxaluria type 1 in The Netherlands: prevalence and outcome.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is a phenotypically heterogeneous disease. To date the relationship between biochemical parameters and outcome is unclear. We therefore undertook a national cohort study on biochemical and clinical parameters and outcome in PH1. METHODS: Review of medical charts of all Dutch PH1 patients, who were identified by sending questionnaires to all Dutch nephrologists for children and adults. RESULTS: Fifty-seven patients were identified. The prevalence and incidence rates were 2.9/10(6) and 0.15/10(6)/year, respectively. Median age at diagnosis was 7.3 years (range 0-57). Seventeen (30%) patients were older than 18 years at time of diagnosis, of whom 10 (59%) presented with end-stage renal disease (ESRD), in contrast to only nine (23%) of those aged under 18 years. Median age at initial symptoms was 6.0 years (range 0-50). In four of nine patients with infantile PH1, normal renal function was preserved after a median follow-up of 7.7 years (range 0.1-16). Progression to renal insufficiency was associated with the presence of nephrocalcinosis, as assessed by ultrasound (relative risk=1.8; 95% CI, 1.0-3.4) and with pyridoxine-unresponsiveness (relative risk=2.2; 95% CI, 1.1-4.2) but not with age at presentation, the extent of hyperoxaluria, or AGT activity. No apparent nephrocalcinosis was found in five of the 19 patients who presented with ESRD. CONCLUSIONS: Although more than one-half of the PH1 patients have symptoms under the age of 10 years, PH1 can present at any age. In adults, PH1 presents predominantly with ESRD, which may be due to misinterpretation of early symptoms. Although nephrocalcinosis is correlated with development of renal insufficiency, the latter can occur even in the absence of nephrocalcinosis. Pyridoxine sensitivity is associated with better outcome in PH1.     

Endoscopic thoracic sympathectomy for primary hyperhidrosis: A 16-year follow up in a single UK centre

IntroductionSince the introduction of Endoscopic Thoracic Sympathectomy ( ETS ) by Kux in 1951, the procedure has been performed for treatment of primary hyperhidrosis (PH) of the upper limb. Despite its initial success and minimally invasive nature, the long-term results are yet to be established. The aim of this study is to evaluate the outcome of patients after ETS with particular emphasis on patient satisfaction and its effectiveness over a 16-year period.MethodsA patient survey of fifty-one (n = 51) patients who had ETS for PH of palms from 1995 to 2011 was conducted. The data on age, sex, site of the PH, family history, trigger, hospital stay, relief from symptoms, complications, refractory sweating and overall satisfaction with the procedure was analysed with SAS software version 9.1.3.ConclusionThe mean follow-up was 77 months (range, 6–189 months) with equal gender distribution (n = 24 males Vs n = 27 females) and mean age of 19 (range, 13–64 years). The hereditary prevalence was 55%. Forty-six patients (90.2%) reported an immediate (≤24 h) and four patients (7.8%) reported a delay (>24 h) in relief of symptoms. To the best of our knowledge this is longest duration of follow-up reported in the literature.     

Phenotype-Genotype Correlations and Estimated Carrier Frequencies of Primary Hyperoxaluria

Primary hyperoxaluria (PH) is a rare autosomal recessive disease characterized by oxalate accumulation in the kidneys and other organs. Three loci have been identified: AGXT (PH1), GRHPR (PH2), and HOGA1 (PH3). Here, we compared genotype to phenotype in 355 patients in the Rare Kidney Stone Consortium PH registry and calculated prevalence using publicly available whole-exome data. PH1 (68.4% of families) was the most severe PH type, whereas PH3 (11.0% of families) showed the slowest decline in renal function but the earliest symptoms. A group of patients with disease progression similar to that of PH3, but for whom no mutation was detected (11.3% of families), suggested further genetic heterogeneity. We confirmed that the AGXT p.G170R mistargeting allele resulted in a milder PH1 phenotype; however, other potential AGXT mistargeting alleles caused more severe (fully penetrant) disease. We identified the first PH3 patient with ESRD; a homozygote for two linked, novel missense mutations. Population analysis suggested that PH is an order of magnitude more common than determined from clinical cohorts (prevalence, approximately 1:58,000; carrier frequency, approximately 1:70). We estimated PH to be approximately three times less prevalent among African Americans than among European Americans because of a limited number of common European origin alleles. PH3 was predicted to be as prevalent as PH1 and twice as common as PH2, indicating that PH3 (and PH2) cases are underdiagnosed and/or incompletely penetrant. These results highlight a role for molecular analyses in PH diagnostics and prognostics and suggest that wider analysis of the idiopathic stone-forming population may be beneficial.     

Comparison of Outcomes in Patients Undergoing Renal Transplantation With Impaired vs Normal Left Ventricular Ejection Fraction

BackgroundRenal transplantation improves long-term outcomes in patients with end-stage renal disease (ESRD); however, patients with impaired left ventricular ejection fraction (LVEF) are less likely to be selected for renal transplantation. We sought to evaluate the effect of renal transplantation in this population.MethodsWe retrospectively evaluated 181 patients who underwent renal transplantation between 2011 and 2016. For patients with pretransplant LVEF <50% (cohort 1) and ≥50% (cohort 2), we evaluated the effect of renal transplantation on LVEF, graft failure, and mortality.ResultsCohort 1 comprised 24 patients (mean age, 47 years; pretransplant LVEF 38%). Cohort 2 comprised 157 patients (mean age, 53 years; pretransplant LVEF 64%). Forty-six percent of cohort 1 experienced significant improvement in LVEF posttransplant, with mean LVEF improvement from 38% to 66%. There was no significant association between pretransplant LVEF and graft failure (hazard ratio [HR] = 2.7; 95% confidence interval [CI], 0.6-11.4; P = .1) or mortality (HR = 1.02; 95% CI, 0.3-3.6; P = .9). Coronary artery disease predicted mortality (HR = 3.12; 95% CI, 1.2-8.4; P = .02). Older age trended toward higher mortality (HR = 1.04; 95% CI, 1.0-1.1; P = .05). Younger age predicted graft failure (HR = 0.96; 95% CI, 0.8-0.9; P = .02).ConclusionsIn patients with ESRD undergoing renal transplantation, there was no significant association between pretransplant LVEF and mortality or graft failure, suggesting that patients with ESRD with impaired LVEF can experience positive posttransplant outcomes.     

Late diagnosis of primary hyperoxaluria after failed kidney transplantation

Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive inborn error of the glyoxylate metabolism that is based on absence, deficiency or mislocalization of the liver-specific peroxisomal enzyme alanine:glyoxylate aminotransferase. Hyperoxaluria leads to recurrent formation of calculi and/or nephrocalcinosis and often early end-stage renal disease (ESRD) accompanied by systemic calcium oxalate crystal deposition. In this report, we describe an adult female patient with only one stone passage before development of ESRD. With unknown diagnosis of PH, the patient received an isolated kidney graft and developed an early onset of graft failure. Although initially presumed as an acute rejection, the biopsy revealed calcium oxalate crystals, which then raised a suspicion of primary hyperoxaluria. The diagnosis was later confirmed by hyperoxaluria, elevated plasma oxalate levels and mutation of the AGXT gene, showing the patient to be compound heterozygous for the c.33_34InsC and c.508G > A mutations. Plasma oxalate levels did not decrease after high-dose pyridoxine treatment. Based on this case report, we would recommend in all patients even with a minor history of nephrolithiasis but progression to chronic renal failure to exclude primary hyperoxaluria before isolated kidney transplantation is considered.     

Novel mutations in response to vitamin B6 in primary hyperoxaluria type 1 after only kidney transplantation: a case report

Recently, the mainstream curative treatment for primary hyperoxaluria type 1 (PH1) is combined liver and kidney transplantation, and only kidney transplantation is considered ineffective for most PH1 patients. Furthermore, vitamin B6 (B6) is the only permitted drug available for treatment. However, except for specific mutations such as G170R and F152I in gene AGXT, data of B6 effect on other mutations are lacking. Insufficient research has evaluated the efficacy of the combination of kidney transplantation and B6 treatment in the therapeutic strategy in PH1 patients. Here, we report a case of a 52-year-old male with frequent stone events and end-stage renal diseases (ESRD), and subsequently undergone kidney transplantation. Sudden rising of serum creatinine within two months after the transplantation. After gene sequencing, the mutations of A186V, R197Q, and I340M were presented in gene AGXT. Therefore, the patient was diagnosed with PH1. B6 administration was attempted during the period of waiting for liver transplantation. Four-week oral B6 therapy (50 mg tid) reduced the serum creatinine of the patient from 194 to 145 µmol/L, which revealed that the patient probably responded to B6 treatment. At the almost three-year follow-up, the patient’s serum creatinine remained reduced (130 µmol/L), without urinary oxalate excretion. In this case, we established a positive effect, even a beneficial result, of the use of B6 as a retrospective therapeutic choice in PH1 treatment after kidney transplantation.     

Oxalate retinopathy is irreversible despite early combined liver‐kidney transplantation in primary hyperoxaluria type 1

In primary hyperoxaluria type 1 (PH1), systemic oxalate deposition (oxalosis) in end‐stage renal disease (ESRD) is associated with high morbidity and mortality, particularly in children with infantile oxalosis (IO). Combined liver and kidney transplantation (CLKT) is the only curative treatment option in these patients. After CLKT, systemic oxalosis decreases continuously, although only insufficient data are available regarding oxalate retinopathy (ROx), leading to severe visual impairment. We analyzed long‐term follow‐up data of ROx in 13 patients undergoing CLKT for PH1 at our center between 1998 and 2018. Age at transplantation was 1.3‐14.2 years, including nine patients with IO. We performed visual acuity testing, slit lamp investigation, funduscopy, fundus photography, and spectral‐domain optical coherence tomography (SD‐OCT) imaging. Severe (grade 2‐4) ROx was present in all nine children with IO but not in the four patients developing ESRD in adolescence. A significant negative correlation was found between age at onset of ESRD and grade of ROx (r = ?0.66; P < .001). Notably, follow‐up assessment after CLKT demonstrated no regression of ROx after a median of 5.3 years (range 0.6‐14). The data show that despite early CLKT in IO, ROx is irreversible and the concomitant visual deterioration occurs prior to transplantation.     

Occurrence,outcomes and predictors of portal hypertension in cystic fibrosis: A longitudinal prospective birth cohort study

BackgroundThe reported prevalence of portal hypertension (PH) in Cystic Fibrosis is variable, incidence rates rarely provided and the utility of liver function tests (LFT's) early in life to predict PH is questionable. The aims were to (1) determine PH prevalence (P) and incidence rate (IR) and combined mortality transplant (MTX) data in PH vs non-PH patients and (2) to assess association of LFTs in early life with liver disease and PH.Method(1) A double centre longitudinal cohort study of 577 CF patients diagnosed by newborn screening (NBS) with annual examinations for PH up to 18.5 years of age (max) was performed over 28 years for P, IR, and MTX data; (2) Cox proportional hazard models were used to assess the association of elevated LFTs on 2 or more occasions over 0–6.5 years and PH.Results51/577(8.8%) developed PH with an average IR of near 3/1000 patient years per 5 year interval representing young, mid and late childhood respectively in patients 3–18 years of age. Combined mortality/liver transplant occurred in 12/51 (23.5%) PH and 25/526 (4.8%) non-PH (p < 0.001). Elevated enzymes particularly GGT (HR:5.71, 95% CI 3.11–10.47); ALT/GGT (HR: 5.56, 95% CI 2.82–10.98); and ALP/GGT (HR: 5.74, 95% CI 2.78–11.86) were associated with the onset of PH.ConclusionThis birth cohort with annual examination for PH provides an accurate assessment of the prevalence, and IR of PH and MTX of PH vs non-PH. Early elevated LFTs are associated with onset of MBC/PH.     

Male Sexual Function in Patients Receiving Different Types of Renal Replacement Therapy

BackgroundPatients with end-stage renal disease (ESRD) experience erectile dysfunction (ED). Although it is a benign disorder, ED is related to physical and psychosocial health, and it has a significant impact on the quality of life (QOL). The objective of the present study was to investigate the effects of different renal replacement therapies on ED.MethodsA total of 100 ESRD patients and 50 healthy men were recruited to the present cross-sectional study. The study was consisted of 53 renal transplantation (RT; group I; mean age, 39.01 ± 7.68 years; mean duration of follow-up, 97.72 ± 10.35 months) and 47 hemodialysis (HD) patients (group II; mean age, 38.72 ± 9.12 years; mean duration of follow-up, 89.13 ± 8.65 months). The control group consisted of 50 healthy men (group III; mean age 39.77 ± 8.51 years). Demographic data and laboratory values were obtained. All groups were evaluated with the following scales: International Index of Erectile Function (IIEF)-5 and Short Form (SF)-36 questionnaires, and Beck Depression Inventory (BDI). The patients whose IIEF score were ≤21 were accepted as having ED.ResultsThe mean age of these groups were similar (P > .05). Total IIEF-5 scores of men in groups I, II, and III were 19.5 ± 4.5, 16.4 ± 5.9, and 22.5 ± 3.4, respectively. The mean total IIEF-5 score of control group was higher than those of groups I and II (P < .001). Posttransplant group mean total IIEF-5 score was also higher than the HD group (P < .05). Groups I and II significantly differed from control group in terms of presence of ED (IIEF score ≤21: Group I, n = 28 [52.8%]; group II, n = 29 [61.7%]; and group III, n = 12 [%24], respectively [P < .001]), whereas there was no difference between groups I and II. In the logistic regression analysis (variables included age, BDI, and renal replacement therapy [HD and transplantation]), ED was independently associated with age (odds ratio [OR], 1.1; 95% confidence interval [CI], 1.05–1.2), BDI (OR, 1.1; 95% CI, 1.01–1.13). Additionally, ED was not associated with renal replacement therapy (OR, 1.46; 95% CI, 0.60–3.57). Physiologic health domain of SF-36 was significantly better in healthy controls (P < .001). Patient groups were similar in terms of BDI score (P > .05). ED score was negatively correlated with BDI (r = ?0.368; P < .001), and positively correlated with SF-36 (r = 0.495; P < .001) in all patient groups.ConclusionPatients with ESRD had significantly lower sexual function and lower QOL scores than the healthy control men. Notably, the mode of renal replacement therapy had no impact on male sexual function.     

The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease

The course of autosomal dominant polycystic kidney disease (ADPKD) varies among individuals, with some reaching ESRD before 40 years of age and others never requiring RRT. In this study, we developed a prognostic model to predict renal outcomes in patients with ADPKD on the basis of genetic and clinical data. We conducted a cross-sectional study of 1341 patients from the Genkyst cohort and evaluated the influence of clinical and genetic factors on renal survival. Multivariate survival analysis identified four variables that were significantly associated with age at ESRD onset, and a scoring system from 0 to 9 was developed as follows: being male: 1 point; hypertension before 35 years of age: 2 points; first urologic event before 35 years of age: 2 points; PKD2 mutation: 0 points; nontruncating PKD1 mutation: 2 points; and truncating PKD1 mutation: 4 points. Three risk categories were subsequently defined as low risk (0–3 points), intermediate risk (4–6 points), and high risk (7–9 points) of progression to ESRD, with corresponding median ages for ESRD onset of 70.6, 56.9, and 49 years, respectively. Whereas a score ≤3 eliminates evolution to ESRD before 60 years of age with a negative predictive value of 81.4%, a score >6 forecasts ESRD onset before 60 years of age with a positive predictive value of 90.9%. This new prognostic score accurately predicts renal outcomes in patients with ADPKD and may enable the personalization of therapeutic management of ADPKD.