Despite overall superior outcomes, transplant outcomes of patients with autosomal dominant polycystic kidney disease are heavily influenced by race. Access to living donor and preemptive transplantation partly explains these racial disparities. Favorable Expected Post-Transplant Survival scores suggest that promoting equity would result in improved survival for patients with ADPKD.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of ESKD and occurs without racial predilection. In general, non-White patients with ESKD have less access to transplantation, especially living donor transplantation. We examined long-term outcomes of patients with ADPKD-ESKD by self-reported race, with attention to the trajectory of Estimated Post-Transplant Survival (EPTS) scores over time.

United Network for Organ Sharing Standard Transplant Analysis and Research files were used to identify 32,611 ADPKD transplant recipients between January 2000 and December 2022. EPTS scores were calculated from the date of waitlisting until transplantation occurred. Cumulative incidences of living and deceased transplantation were calculated and plotted. Cox models were made for graft failure and death, and a subdistribution hazards model for graft failure accounted for death as a competing outcome, with adjustment for patient, donor, and transplant factors.

Compared with White patients with ADPKD, all other groups had more dialysis years, more delayed graft function, and fewer living and preemptive transplants; mean EPTS scores were lower in Black and Hispanic patients at each time point on the waitlist. However, EPTS scores at the time of transplant was less likely to be <20% in Black and Hispanic patients because of longer waiting time. Black patients had a significantly higher risk of graft failure with death as competing risk compared with White patients. Asian and Hispanic patients had similar graft survivals but better patient survival compared with White patients.

Waitlist experience, allograft quality, and post-transplant outcomes of patients with ADPKD are influenced by patient race.

• ESKD
• clinical nephrology
• ethnic minority
• health equity, diversity, and inclusion
• kidney transplantation
• polycystic kidney disease
• social health justice

Cardiac localized polycystin facilitates natriuretic peptide signaling pathways. Hypertension associated with autosomal dominant polycystic kidney disease may arise from impaired cardiac natriuretic peptide signaling.

Hypertension is seen in 70% of patients with autosomal dominant polycystic kidney disease by age of 30 years before decline in kidney function. However, cardiac origins of hypertension, such as the natriuretic peptide signaling pathway, have not been fully investigated. We hypothesized that cardiomyocyte localized polycystin proteins contribute to production of natriuretic peptides, and loss of this pathway would contribute to hypertension.

Telemetry, echocardiography, and a molecular analysis of the natriuretic peptide pathway from left ventricular tissue of cardiomyocyte specific knockout models of polycystin-2 (cPC2-KO) mice and Cre control littermates were conducted. Complementary studies were conducted in ex vivo murine hearts, engineered heart tissue with human iPSCs driven into cardiomyocytes with CRISPR/Cas9 knockout of PKD2 and in in vitro cell lines.

cPC2-KO mice demonstrated diurnal hypertension. Circulating atrial natriuretic peptide (ANP) and brain natriuretic peptide were unchanged between cPC2-KO and Cre mice. Analysis of the pathways involved in production, maturation, and activity of natriuretic peptides identified decreased transcription of chromogranin B, PCSK6, NPR1, and NFAT genes in cPC2-KOs. Human iPSC-derived cardiomyocytes with PC2-KO failed to produce ANP. Re-expression of polycystin-2 in a myoblast cell line, but not pathogenic forms of polycystin-2, restored ANP production.

Natriuretic peptide production required cardiac localized polycystin-2, and loss of this pathway may contribute to the development of hypertension in autosomal dominant polycystic kidney disease.

This article contains a podcast at https://dts.podtrac.com/redirect.mp3/www.asn-online.org/media/podcast/JASN/2024_10_08_ASN0000000000000490.mp3

• calcium
• cardiovascular
• hypertension
• hypertension, and cardiorenal disorders

• 1021282 PKDF
• S10 OD028449 NIH HHS
• T35 HL120835 NHLBI NIH HHS
• T35HL120835 NHLBI NIH HHS
• R00DK101585 NIDDK NIH HHS
• 1S10OD028449 NIH Office of the Director
• R00 DK101585 NIDDK NIH HHS
• UL1 TR002389 NCATS NIH HHS
• UL1TR002389 Common Fund
• U2CDK129917 NIDDK NIH HHS
• TL1 DK132769 NIDDK NIH HHS
• U2C DK129917 NIDDK NIH HHS
• TL1DK132769 NIDDK NIH HHS
• R00141698 NHLBI NIH HHS
• U54 DK137307 NIDDK NIH HHS

• Autosomal dominant polycystic kidney disease
• Kidney transplantation
• Post-transplant diabetes mellitus
• Post-transplant infection

• Autosomal dominant polycystic kidney disease: quantitative susceptibility mapping to evaluate complex cysts
• Hemorrhage
• Renal calcifications

• Humans
• Female
• Polycystic Kidney, Autosomal Dominant/diagnostic imaging
• Polycystic Kidney, Autosomal Dominant/complications
• Male
• Magnetic Resonance Imaging/methods
• Middle Aged
• Adult
• Hemorrhage/diagnostic imaging
• Kidney Calculi/diagnostic imaging
• Tomography, X-Ray Computed/methods
• Feasibility Studies
• Diagnosis, Differential
• Image Interpretation, Computer-Assisted/methods
• Aged

• polycystic kidney, autosomal dominant
• renal insufficiency
• rate
• mortality
• risk factors
• rim policístico autossômico dominante
• insuficiência renal
• taxa
• mortalidade
• fatores de risco

• Humans
• Polycystic Kidney, Autosomal Dominant/complications
• Male
• Female
• Disease Progression
• Brazil/epidemiology
• Glomerular Filtration Rate
• Adult
• Middle Aged
• Risk Factors
• Cohort Studies
• Uric Acid/blood
• Retrospective Studies

More patients with autosomal dominant polycystic kidney disease received their first intervention to re-establish vascular access patency. Patients with autosomal dominant polycystic kidney disease do not require differential monitoring and treatment of hemodialysis vascular access.

Autosomal dominant polycystic kidney disease (ADPKD) is a leading hereditary cause of ESKD, often using hemodialysis as a form of RRT. Patients with ADPKD may also present with extrarenal manifestations, including arterial aneurysms. The gold standard for hemodialysis access is an arteriovenous vascular access (VA), such as arteriovenous fistulas (AVFs) or arteriovenous grafts (AVGs). However, limitations, such as low VA flow and inadequate AVF outward remodeling, affect VA utilization. This study aimed to explore whether ADPKD affects patency rates of AVFs/AVGs in comparison with other underlying ESKD causes.

We conducted a retrospective cohort study using data from the Swedish Renal Registry from 2011 to 2020, with follow-up until 2022. We included 496 patients with ADPKD and 4321 propensity score–matched controls. VA patency rates of patients with ADPKD were compared with those of non-ADPKD patients using Kaplan–Meier survival curves and Mantel–Cox log-rank test. Interventions to maintain or restore patency were also analyzed.

Patients with ADPKD constituted 8.0% of all patients, with a higher proportion in the pre-ESKD phase during VA creation (51.6% versus 40.6%). No significant differences were observed in primary, postcannulation primary, secondary, or functional patency between patients with ADPKD and non-ADPKD patients. However, more VAs were ligated in patients with ADPKD (10.5% versus 7.7%, P = 0.03), and they underwent more first interventions to re-establish flow (49.4% versus 41.9%, P = 0.02).

These findings suggest that AVF/AVG patency remains comparable in patients with ESKD with or without ADPKD, and VA monitoring and treatment strategies for patients with ADPKD should align with those for individuals with other ESKD causes.

• adpkd
• arteriovenous access
• arteriovenous fistula
• arteriovenous graft
• arteriovenous shunt
• dialysis
• dialysis access
• vascular access

• Humans
• Polycystic Kidney, Autosomal Dominant
• Female
• Male
• Renal Dialysis
• Treatment Outcome
• Middle Aged
• Adult
• Arteriovenous Shunt, Surgical

• Leids Universitair Medisch Centrum, the Swedish Association of Local Authorities and Regions

• R56 HL165137 NHLBI NIH HHS

• Autosomal dominant polycystic kidney disease
• Checkpoint inhibitors
• Hypertension
• T cell subsets

• Humans
• Polycystic Kidney, Autosomal Dominant/complications
• Polycystic Kidney, Autosomal Dominant/metabolism
• CTLA-4 Antigen
• T-Lymphocytes, Regulatory/metabolism
• Programmed Cell Death 1 Receptor
• Kidney
• Renal Insufficiency, Chronic/complications
• Hypertension
• Disease Progression
• Cell Count
• Glomerular Filtration Rate

• TTU-2020-9771 Erciyes University BAP

• ADPKD
• cardiac hypertrophy
• cardiac remodeling
• hypertension
• vascular damages

• Male
• Female
• Adolescent
• Humans
• Child
• Polycystic Kidney, Autosomal Dominant/complications
• Pilot Projects
• Carotid Intima-Media Thickness
• Blood Pressure Monitoring, Ambulatory
• Pulse Wave Analysis
• Ventricular Remodeling
• Hypertension/complications
• Blood Pressure
• Hypertrophy, Left Ventricular/etiology

• autosomal dominant polycystic kidney disease
• dapagliflozin
• total kidney volume

• ADPKD
• Hemodialysis
• Kidney transplantation
• Living donor
• Nephrectomy
• Peritoneal dialysis

• Humans
• Renal Dialysis
• Polycystic Kidney, Autosomal Dominant/complications
• Peritoneal Dialysis/adverse effects
• Kidney Transplantation/adverse effects

• ADPKD
• Bone
• Mineral metabolism
• Transplantation

• BIN1
• T-tubule
• dilated cardiomyopathy
• heart failure
• polycystin-1

Cardiovascular abnormalities, such as left ventricular hypertrophy and valvular disorders, particularly mitral valve prolapse, have been described as highly prevalent among adult patients with autosomal dominant polycystic kidney disease (ADPKD). The present study aimed to assess echocardiographic parameters in a large sample of both normotensive and hypertensive ADPKD patients, regardless of kidney function level, and evaluate their association with clinical and laboratorial parameters. A retrospective study consisted of the analysis of clinical, laboratorial, and transthoracic echocardiograms data retrieved from the medical records of young adult ADPKD outpatients. A total of 294 patients (120 M/174 F, 41.0 ± 13.8 years old, 199 hypertensive and 95 normotensive) with a median estimated glomerular filtration rate (eGFR) of 75.5 mL/min/1.73 m² were included. The hypertensive group (67.6%) was significantly older and exhibited significantly lower eGFR than the normotensive one. Increased left ventricular mass index (LVMI) was seen in 2.0%, mitral valve prolapse was observed in 3.4%, mitral valve regurgitation in 15.3%, tricuspid valve regurgitation in 16.0%, and aortic valve regurgitation in 4.8% of the whole sample. The present study suggested that the prevalence of mitral valve prolapse was much lower than previously reported, and increased LVMI was not seen in most adult ADPKD patients.

• ADPKD
• Autosomal dominant polycystic kidney disease
• Consenso
• Consensus
• Management
• Manejo
• PQRAD
• Poliquistosis renal autosómica dominante
• Progresión
• Progression
• Recomendaciones
• Recommendations

• Child
• Humans
• Consensus
• Polycystic Kidney, Autosomal Dominant/diagnosis
• Polycystic Kidney, Autosomal Dominant/genetics
• Polycystic Kidney, Autosomal Dominant/therapy
• Prospective Studies

• horseshoe
• nephrectomy
• polycystic kidney

• Autosomal dominant polycystic kidney disease
• Cardiovascular disease
• Chronic kidney disease
• Mortality

• Autosomal dominant polycystic kidney disease
• Cardiac dysfunction
• Lipid derangement
• Metabolic rewiring
• Mitochondria
• Polycystin-1 cleavage at GPS

• Animals
• Heart
• Mice
• Mitochondria/metabolism
• Mutation
• Polycystic Kidney, Autosomal Dominant/genetics
• Polycystic Kidney, Autosomal Dominant/metabolism
• TRPP Cation Channels/genetics
• TRPP Cation Channels/metabolism

• P30 DK090868 NIDDK NIH HHS
• U54 DK126114 NIDDK NIH HHS
• R01 DK111611 NIDDK NIH HHS

• cardiovascular disease
• coronary artery disease
• diabetes
• heart failure
• hypertension
• immunosuppression
• kidney transplantation
• kidney transplantation series
• obesity

• angiotensin ii receptor blocker
• angiotensin-converting enzymes inhibitor
• autosomal dominant polycystic kidney disease
• cardiovascular disease

• Angiotensin Receptor Antagonists/therapeutic use
• Angiotensin-Converting Enzyme Inhibitors/therapeutic use
• Cardiovascular Diseases/epidemiology
• Cardiovascular Diseases/prevention & control
• Female
• Heart Disease Risk Factors
• Humans
• Incidence
• Male
• Middle Aged
• Outcome and Process Assessment, Health Care
• Polycystic Kidney, Autosomal Dominant/epidemiology
• Polycystic Kidney, Autosomal Dominant/therapy
• Preventive Health Services/methods
• Preventive Health Services/statistics & numerical data
• Protective Factors
• Registries/statistics & numerical data
• Renal Dialysis/methods
• Renal Dialysis/statistics & numerical data
• Taiwan/epidemiology
• Time

• Autosomal dominant polycystic kidney disease
• Cardiovascular disease
• Chronic kidney disease
• Nephropathy

• Ankle Brachial Index
• Cardiovascular Diseases/etiology
• Carotid Intima-Media Thickness
• Comorbidity
• Disease Progression
• Female
• Heart Disease Risk Factors
• Humans
• Male
• Middle Aged
• Plaque, Atherosclerotic/complications
• Polycystic Kidney, Autosomal Dominant/complications
• Prognosis
• Renal Insufficiency, Chronic/etiology

• Aneurysmal rupture
• Autosomal dominant polycystic kidney disease
• Dolichoectasia
• Hypercholesterolemia
• Intracranial aneurysm
• Mitral inflow deceleration time

• Autosomal dominant polycystic kidney disease
• Diverticular bleeding
• Diverticulitis
• Diverticulosis
• Nationwide Inpatient Sample

• calcineurin inhibitor
• coronary artery disease
• death
• graft survival
• human leukocyte antigen (HLA)
• kidney transplant
• nephrectomy
• obesity
• polycystic kidney disease
• post-transplant diabetes mellitus
• transplantation

Patients with autosomal dominant polycystic kidney disease (ADPKD) have an increased risk of cardiovascular morbidity and mortality. Impaired left ventricular (LV) global longitudinal strain (GLS) can be a sign of subclinical cardiac dysfunction even in patients with otherwise preserved ejection fraction (EF). Transmitral early filling velocity to early diastolic strain rate (E/SRe) is a novel measure of LV filling pressure, which is often affected early in cardiac disease.

A total of 110 ADPKD patients not on dialysis were included in this prospective study. All patients underwent an extensive echocardiographic examination including two-dimensional speckle tracking. GLS and strain rates were measured. The distribution of GLS and E/SRe was determined and patient characteristics were compared by median levels of GLS (− 17.8%) and E/SRe (91.4 cm). Twenty healthy participants were included as control group.

There was a significantly worse GLS in the ADPKD patients (mean: − 17.8 ± 2.5%) compared to the healthy controls (mean: − 21.9 ± 1.9%), p < 0.001. The same was true for E/SRe (mean: 10.0 ± 0.3 cm) compared to the control group (mean: 6.5 ± 0.3 cm), p < 0.001. In simple logistic regression, male gender (OR: 4.74 [2.10–10.71], p < 0.001), fasting glucose (odds ratio (OR) 1.05 [1.01–1.10], p = 0.024), htTKV (OR: 1.07 [1.01–1.13], p = 0.013), HDL cholesterol (OR: 0.97 [0.94, 0.996], p = 0.025), triglycerides (OR: 1.01 [1.00–1.02], p = 0.039), hemoglobin (OR: 1.50 [1.11–2.04], p = 0.009), and β-blocker use (OR: 1.07 [1.01, 1.13], p = 0.013) were all associated with higher GLS. After multivariate logistic regression with backward model selection, only male gender (OR: 5.78 [2.27–14.71], p < 0.001) and β-blocker use (OR: 14.00 [1.60, 122.51], p = 0.017) remained significant. In simple logistic regression models, BMI (OR: 1.11 [1.02–1.20], p = 0.015), systolic blood pressure (OR: 1.03 [1.00–1.06], p = 0.027) and β-blocker use (OR: 17.12 [2.15–136.20], p = 0.007) were associated with higher E/SRe - a novel measure of left ventricular filling pressure. After backward elimination, only β-blocker use (OR: 17.22 [2.16, 137.14], p = 0.007) remained significant.

Higher GLS and E/SRe are common in ADPKD patients, even in patients with preserved eGFR and normal left ventricular EF. GLS and E/SRe may aid in cardiovascular risk stratification in patients with ADPKD as they represent early markers of cardiac dysfunction.

The online version of this article (10.1186/s12882-019-1500-1) contains supplementary material, which is available to authorized users.

• Autosomal dominant polycystic kidney disease
• Global longitudinal Strain
• Two-dimensional speckle tracking echocardiography

• Diuresis
• Female
• Humans
• Kidney Transplantation
• Male
• Middle Aged
• Nephrectomy
• Peritoneal Dialysis
• Polycystic Kidney, Autosomal Dominant/physiopathology
• Polycystic Kidney, Autosomal Dominant/therapy
• Polycystic Kidney, Autosomal Dominant/urine

Mutations in PKD1 or PKD2 gene lead to autosomal dominant polycystic kidney disease (ADPKD). The mechanism of ADPKD progression and its link to increased cardiovascular mortality is still elusive.

We differentiated ADPKD patient induced pluripotent stem cells (iPSCs) to cardiomyocytes (CMs). The electrophysiological properties at the cellular level were analyzed by calcium imaging and whole cell patch clamping.

The ADPKD patient iPSC-CMs had decreased sarcoplasmic reticulum calcium content compared with Control-CMs. Spontaneous action potential of the PKD2 mutation line-derived CMs demonstrated slower beating rate and longer action potential duration. The PKD1 mutation line-derived CMs showed a comparable dose-dependent shortening of phase II repolarization with the Control-CMs, but a significant increase in beating frequency in response to L-type calcium channel blocker. The PKD1-mutant iPSC-CMs also showed a relatively unstable baseline as a greater percentage of cells exhibited delayed afterdepolarizations (DADs). Both the ADPKD patient iPSC-CMs showed more β-adrenergic agonist-elicited DADs compared with Control-CMs.

Characterization of ADPKD patient iPSC-CMs provides new insights into the increased clinical risk of arrhythmias, and the results enable disease modeling and drug screening for cardiac manifestations of ADPKD.

Ministry of Science and Technology, National Health Research Institutes, Academia Sinica Program for Technology Supporting Platform Axis Scheme, Thematic Research Program and Summit Research Program, and Kaohsiung Medical University Hospital, Taiwan.

• Arrhythmia
• Autosomal dominant polycystic kidney disease
• Cardiomyocyte
• Human iPS cell

• CD8(+) T cells
• Pkd1 RC mouse model
• adaptive immunity
• autosomal dominant polycystic kidney disease

• Adaptive Immunity
• Animals
• Antineoplastic Agents, Immunological/therapeutic use
• CD8-Positive T-Lymphocytes/microbiology
• Cell Line
• Disease Models, Animal
• Disease Progression
• Epithelial Cells
• Female
• Humans
• Immunotherapy/methods
• Kidney/cytology
• Kidney/immunology
• Kidney/pathology
• Male
• Mice
• Mice, Inbred C57BL
• Mice, Transgenic
• Mutation
• Polycystic Kidney, Autosomal Dominant/genetics
• Polycystic Kidney, Autosomal Dominant/immunology
• Polycystic Kidney, Autosomal Dominant/pathology
• Polycystic Kidney, Autosomal Dominant/therapy
• Signal Transduction/immunology
• TRPP Cation Channels/genetics

• P30 DK090728 NIDDK NIH HHS
• P30 CA168524 NCI NIH HHS
• R01 HL133545 NHLBI NIH HHS
• R01 HL117062 NHLBI NIH HHS
• R01 DK096030 NIDDK NIH HHS
• P30 DK106912 NIDDK NIH HHS
• R01 HL132318 NHLBI NIH HHS
• T32 GM007635 NIGMS NIH HHS
• T32 DK007135 NIDDK NIH HHS

• Acute myocardial infarction (AMI)
• Autosomal dominant polycystic kidney disease (ADPKD)
• Cardiovascular complications

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, with afflicted patients often progressing to end-stage kidney disease (ESKD) requiring renal replacement therapy (RRT). As the timelines to ESKD are predictable over decades, it follows that ADPKD patients should be optimized regarding kidney transplantation, home dialysis therapies, and vascular access.

To examine the association of kidney transplantation, dialysis modalities, and vascular access in ADPKD patients compared with a matched, non-ADPKD cohort.

Canadian patients from 2001-2012 excluding Quebec.

All adult incident ESKD patients who received dialysis or a kidney transplant.

ADPKD as defined by the treating physician.

ADPKD and non-ADPKD patients were propensity score (PS) matched (1:4) using demographics, comorbidities, and lab values. Conditional logistic regression and Cox proportional hazards models were used to examine associations with kidney transplantation (preemptive or any), dialysis modality (peritoneal, short daily, home, or in-center hemodialysis [HD]), vascular access (arteriovenous fistula [AVF], permanent or temporary central venous catheter [CVC]), and dialysis survival.

We matched 2120 ADPKD (99.9%) with 8283 non-ADPKD with no significant imbalances between the groups. ADPKD was significantly associated with preemptive kidney transplantation (odds ratio [OR] = 7.13, 95% confidence interval [CI] = 5.74-8.87), any kidney transplant (OR = 2.37, 95% CI = 2.14-2.63), and initial therapy of nocturnal daily HD (OR = 2.74, 95% CI = 1.38-5.44), whereas in-center intermittent HD was significantly less likely in the ADPKD population (OR = 0.59, 95% CI = 0.54-0.65). There was no difference in peritoneal dialysis (PD) as initial RRT but lower use of any PD among the ADPKD group (OR = 0.85, 95% CI = 0.77-0.95). ADPKD patients were significantly more likely to have an AVF (OR = 3.25, 95% CI = 2.79-3.79) and less likely to have either a permanent (OR 0.68, 95% CI 0.59-0.78) or temporary (OR = 0.49, 95% CI = 0.41-0.59) CVC as compared with the non-ADPKD cohort. Survival on either in-center HD or PD was better for ADPKD patients (HD: hazard ratio [HR] 0.48, 95% CI 0.44-0.53; PD: HR 0.73, 95% CI 0.60-0.88).

Conservative care patients were not captured; despite PS matching, the possibility of residual confounding remains.

ADPKD patients were more likely to receive a kidney transplant, use home HD, dialyze with an AVF, and have better survival relative to non-ADPKD patients. Conversely, they were less likely to receive PD either as initial therapy or anytime during ESKD. This may be attributed to higher transplantation or clinical decision-making processes susceptible to education and intervention.

• CORR
• end-stage kidney disease
• home dialysis
• peritoneal dialysis
• polycystic kidney disease
• vascular access

• Atrium
• Autosomal dominant polycystic kidney disease
• Congenital left ventricular diverticulum
• Polycystin

• bleeding
• cholangitis
• cirrhosis
• pancreatitis
• polycystic kidney disease

Cardiovascular complications are the leading causes of death in patients with autosomal-dominant polycystic kidney disease (ADPKD) in the Western countries. However, theprevalence and risk of acute myocardial infarction (AMI) in patients with ADPKD remain unknown, especially in Asian population. We utilized the data from Taiwan National Health Insurance Research Database (NHIRD) to perform a population-based cohort study (1997-2008). A total of 2062 patients with ADPKD were selected from one million of general population after excluding those patients with age less than 18 years old, receiving renal replacement therapy, and concomitant diagnoses of AMI. Additionally, we set up those patients without ADPKD as comparison group by matching study cohort with age, gender, income and urbanization with 1:10 ratio (n=20620). The results showed that although the prevalence of AMI in ADPKD patients in Taiwan was lower than those in the United States (2.91% v.s. 6%, p=0.0567), the Taiwanese ADPKD group had significantly higher prevalence of AMI as compared with the non-ADPKD group (2.91% v.s. 0.97%, p<0.0001). In addition, Kaplan-Meier analysis demonstrated that cumulative incidence of AMI was significantly higher in ADPKD than in the non-ADPKD group (all p<0.001). After adjusting for age, gender and comorbidities by multivariate and sensitivity analysis, ADPKD patients had 2.43-fold greater risk for developing AMI as compared with non-ADPKD patients (95% CI 1.8 to 3.29, p<0.0001). In conclusion, Taiwanese patients with ADPKD have lower prevalence of AMI as compared to Americans, whereas ADPKD per se remains independently predictive of AMI in Asian population.

• Asian
• Taiwanese
• acute myocardial infarction
• autosomal-dominant polycystic kidney disease
• population-based cohort study

• apelin/APJ system
• fibrosis
• kidney
• kidney disease
• transforming growth factor-β

Fairly limited data reported the incidence and risk of cerebrovascular accident (CVA) in autosomal dominant polycystic kidney disease (ADPKD). Additionally, little is known regarding the therapeutic impact of renin-angiotensin-aldosterone system (RAAS) blockade and statin on reducing the occurrence of CVA in ADPKD. We utilized the data from Taiwan National Health Insurance Research Database (NHIRD) to perform a population-based cohort study (1997-2013). A total of 2,647 patients with ADPKD were selected from 1,000,000 general population after excluding patients with age<18, renal replacement therapy and concomitant diagnosis of CVA. Additionally, non-ADPKD subjects were assigned as comparison group by matching study cohort with age, gender, income and urbanization in 1:10 ratio (n=26,470). The results showed that ADPKD group had significantly higher frequency rate and cumulative incidence of CVA as compared with the non-ADPKD group (8.73% v.s. 3.93%, p<0.0001). Furthermore, the frequencies of both hemorrhagic and ischemic strokes were also significantly higher in the ADPKD than non-ADPKD group (all p-values <0.0001). After adjusting for age, gender and atherosclerotic risk factors with multivariate analysis, ADPKD independently carried 2.34- and 5.12-fold risk for occurrence of CVA and hemorrhagic stroke (95% CI: 2.02-2.72 and 4.01-6.54), respectively. Combination therapy [adjusted (a) HR=0.19, 95% CI: 0.11-0.31] was superior to either RAAS blockade (aHR=0.37, 95% CI, 0.28-0.5) or statin (aHR=0.44, 95% CI, 0.24-0.79) alone for reducing the CVA occurrence in the ADPKD population. In conclusion, ADPKD was associated with an increased risk of CVA occurrence. Combined RAAS blockade and statin therapy effectively reduces the risk of CVA in ADPKD.

• autosomal-dominant polycystic kidney disease
• cerebrovascular accident
• population-based cohort study
• renin-angiotensin-aldosterone system blockade
• statin

• Anévrisme intracrânien
• Autosomal dominant polycystic kidney disease
• Dépistage
• Hémorragie méningée
• Intracranial aneurism
• Polykystose rénale autosomique dominante
• Screening
• Subarachnoidal hemorrhage

• aortic aneurysm
• aortic dissection
• autosomal-dominant polycystic kidney disease
• population-based cohort study