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Background and objectives Autosomal Dominant Polycystic Kidney Disease (ADPKD) presents with variable disease severity and progression. Advanced imaging biomarkers may provide insights into cystic and non-cystic processes leading to kidney failure in different age groups. Methods This pilot study included 39 ADPKD patients with kidney failure, stratified into three age groups (56 years old). Advanced imaging biomarkers were assessed using an automated instance cyst segmentation tool. The biomarkers were compared with an age- and sex-matched ADPKD cohort in early chronic kidney disease (CKD). Results Ht-total parenchymal volume correlated negatively with age at kidney failure. The median Ht-total parenchymal volume was significantly lower in patients older than 56 years. Cystic burden was significantly higher at time of kidney failure and especially in patients who reached it before age 46. The cyst index at kidney failure was comparable across age groups and Mayo Imaging Classes. Advanced imaging biomarkers showed higher correlation with Ht-total kidney volume in early CKD than at kidney failure. Cyst index and parenchymal index were relatively stable over five years prior to kidney failure, whereas Ht-total cyst volume and cyst parenchymal surface area increased significantly. Conclusion Age-related differences in advanced imaging biomarkers suggest variable pathophysiological mechanisms in ADPKD patients with kidney failure. Further studies are needed to validate the utility of these biomarkers in predicting disease progression and guiding treatment strategies.

Cardiovascular disease leads to high morbidity and mortality in patients with kidney failure. Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a systemic disease with various cardiac abnormalities. Details on the cardiovascular profile of patients with ADPKD who are undergoing kidney transplantation (KT) and its progression are limited.Echocardiographic data within 2 years before KT (1993-2020), and major adverse cardiovascular events (MACEs) after transplantation were retrieved. The primary outcome is to assess cardiovascular abnormalities on echocardiography at the time of transplantation in ADPKD as compared with patients without ADPKD matched by sex (male, 59.4%) and age at transplantation (57.2 ± 8.8 years).Compared with diabetic nephropathy (DN,ADPKD transplant recipients have the most favorable cardiac profile pretransplantation with better patient survival and MACE-free survival rates but worsening valvular function and increasing sinus of Valsalva diameter, as compared with patients with other kidney diseases.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) has phenotypic variability only partially explained by established biomarkers that do not readily assess pathologically important factors of inflammation and kidney fibrosis. We evaluated asymptomatic pyuria (AP), a surrogate marker of inflammation, as a biomarker for disease progression. METHODS: We performed a retrospective cohort study of adult patients with ADPKD. Patients were divided into AP and no pyuria (NP) groups. We evaluated the effect of pyuria on kidney function and kidney volume. Longitudinal models evaluating kidney function and kidney volume rate of change with respect to incidences of AP were created. RESULTS: There were 687 included patients (347 AP, 340 NP). The AP group had more women (65% versus 49%). Median ages at kidney failure were 86 and 80 years in the NP and AP groups (log rank, P=0.49), respectively, for patients in Mayo Imaging Class (MIC) 1A–1B as compared with 59 and 55 years for patients in MIC 1C–1D–1E (log rank, P=0.02), respectively. Compared with the NP group, the rate of kidney function (ml/min per 1.73 m(2) per year) decline shifted significantly after detection of AP in the models, including all patients (−1.48; P

Computed tomography coronary angiography (CTCA) has a higher negative predictive value (NPV) for coronary artery disease (CAD) than stress echocardiography (SE). CT calcium scoring (CTCS) allows detection and quantification of coronary artery calcification (CAC). The NPV of combined SE and CTCS for CAD is not well defined. Consecutive patients from the executive screening program who underwent exercise SE and concomitant CTCA were retrospectively identified between January 2010 and December 2014. Patients with normal SE and CAC score of zero were determined, and the presence or absence of any CAD (obstructive or non-obstructive plaques) on CTCA was confirmed. The NPV of combined SE and CTCS was then re-tested using a validation cohort of subsequent consecutive patients enrolled between January 2015 and July 2018. The initial cohort consisted of 173 patients (19% age > 65 years, 19% diabetic); 40% had normal CTCA, 48% with non-obstructive CTCA (77 with CAC score > 0), and 12% with obstructive CTCA (all with CAC score > 0). There were 16 (9.2%) patients with inducible ischemia on SE. A normal SE had a 93% NPV to exclude obstructive CAD but only 42% NPV to exclude any CAD. A combined normal SE and CTCS had a 100% NPV for obstructive CAD, and 92% for any CAD. In a validation cohort of 111 patients, a normal SE and CAC score of zero had NPV of 100% for obstructive CAD and 92% for any CAD. The combined cohort consisted of predominately low Framingham risk patients; more than 40% (70/181) had CAC score > 0 and 5/70 had obstructive CAD, with the remaining non-obstructive. A concomitant normal SE and CAC score of zero excluded obstructive CAD (NPV 100%) and any CAD in 92% of the testing and validation cohorts. CTCS seems to add incremental risk stratification, particularly for patients with low Framingham score.