End-Stage Kidney Disease in Patients With Autosomal Dominant Polycystic Kidney Disease: A 12-Year Study Based on the Canadian Organ Replacement Registry

Abstract

Background: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.Objectives:To examine the association of kidney transplantation, dialysis modalities, and vascular access in ADPKD patients compared with a matched, non-ADPKD cohort.Setting:Canadian patients from 2001-2012 excluding Quebec.Patients:All adult incident ESKD patients who received dialysis or a kidney transplant.Measurements:ADPKD as defined by the treating physician.Methods: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.Results: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).Limitations:Conservative care patients were not captured; despite PS matching, the possibility of residual confounding remains.Conclusions: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.

Identification of the Factors Associated With Intraperitoneal Pressure in ADPKD Patients Treated With Peritoneal Dialysis

“…In addition, the PD technical survival rate is similar in ADPKD compared with patients without ADPKD. [7][8][9][10][11][12][13][14][15][16][17][18] Therefore, identification of unbiased criteria for selecting patients with ADPKD for PD treatment could reassure nephrologists and increase the use of PD in this population.…”

Surgical Management of Autosomal Dominant Polycystic Kidney Disease: Principles and Current Practice

“…Therefore, planning RT well in advance, preferably with a timely living kidney donor (LKD) work-up offers the best opportunity for the avoidance of dialysis, which does carry significant morbidity and morbidity commensurate with the duration of dialysis. 31 The surgical technique of RT, immunosuppressive drugs and the outcomes of RT in ADPKD patients are similar to those of non-ADPKD patients. 32 An early referral by the nephrology team for RT assessment when the estimated glomerular filtration rate (eGFR) is between 15-20 ml/min/1.73 m 2 gives plenty of time for undertaking an LKD work-up or creation of dialysis access.…”

High-Resolution Computed Tomography of Cystic Lung Disease

“…Autosomal dominant polycystic kidney disease is the most common genetic kidney disease and accounts for 5 to 10% of all patients requiring renal replacement therapy. 124 On chest CT, bronchiectasis is most common; however, cysts have been reported, varying in size and distribution 125 126 ( Fig. 18 ).…”