An Electronic Health Record-Integrated Application for Standardizing Care and Monitoring Patients With Autosomal Dominant Polycystic Kidney Disease Enrolled in a Tolvaptan Clinic: Design and Implementation Study.

Background: Tolvaptan is the only US Food and Drug Administration-approved drug to slow the progression of autosomal dominant polycystic kidney disease (ADPKD), but it requires strict clinical monitoring due to potential serious adverse events.

Objective: We aimed to share our experience in developing and implementing an electronic health record (EHR)-based application to monitor patients with ADPKD who were initiated on tolvaptan.

Methods: The application was developed in collaboration with clinical informatics professionals based on our clinical protocol with frequent laboratory test monitoring to detect early drug-related toxicity.

Development and Implementation of an Acute Kidney Injury Remote Patient Monitoring Program: Research Letter.

Unlabelled: Acute kidney injury (AKI) survivors have a dynamic posthospital course which warrants close monitoring. Remote patient monitoring (RPM) could be used to improve quality and efficiency of AKI survivor care.

Objective: The objective of this report was to describe the development and preliminary feasibility of an AKI RPM program launched in October 2021.

Effects of SARS-CoV-2 vaccination on the severity of COVID-19 infection in patients on chronic dialysis.

Background: COVID-19 is associated with increased morbidity and mortality in patients with end-stage kidney disease on dialysis. Efficacy of SARS-CoV-2 vaccination to prevent severe COVID-19 disease in end-stage kidney disease patients remains limited. We compared the incidence of COVID-19-related hospitalization and death in dialysis patients based on SARS-CoV-2 vaccine status.

Bone health in autosomal dominant polycystic kidney disease (ADPKD) patients after kidney transplantation.

ADPKD is caused by pathogenic variants in or , encoding polycystin-1 and -2 proteins. Polycystins are expressed in osteoblasts and chondrocytes in animal models, and loss of function is associated with low bone mineral density (BMD) and volume. However, it is unclear whether these variants impact bone strength in ADPKD patients.

Cardiovascular Outcomes in Kidney Transplant Recipients With ADPKD.

Introduction: 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.

Asymptomatic Pyuria as a Prognostic Biomarker in Autosomal Dominant Polycystic Kidney Disease.

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.

Provider Perspectives and Clinical Outcomes with Inpatient Telenephrology.

Background And Objectives: Despite the dramatic increase in the provision of virtual nephrology care, only anecdotal reports of outcomes without comparators to usual care exist in the literature. This study aimed to provide objective determination of clinical noninferiority of hybrid (telenephrology plus face-to-face) versus standard (face-to-face) inpatient nephrology care.

Design, Setting, Participants, & Measurements: This retrospective study compares objective outcomes in patients who received inpatient hybrid care versus standard nephrology care at two Mayo Clinic Health System community hospitals.

Congenital Heart Disease in Adults with Autosomal Dominant Polycystic Kidney Disease.

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is caused mainly by pathogenic variants in PKD1 or PKD2 encoding the polycystin-1 and -2 proteins. Polycystins have shown to have an essential role in cardiac development and function in animal models. In the current study, we describe the clinical association between ADPKD and congenital heart disease (CHD).

Telehealth versus face-to-face visits: A comprehensive outpatient perspective-based cohort study of patients with kidney disease.

Background: Telenephrology has become an important health care delivery modality during the COVID-19 pandemic. However, little is known about patient perspectives on the quality of care provided via telenephrology compared to face-to-face visits. We aimed to use objective data to study patients' perspectives on outpatient nephrology care received via telenephrology (phone and video) versus face-to-face visits.

Rituximab-Associated Flare of Cryoglobulinemic Vasculitis.

Background: Patients with cryoglobulinemic vasculitis (CV) can develop disease flare after rituximab administration. The objective of our study was to describe the prevalence, clinical characteristics, predisposing factors, and outcomes of patients with rituximab-associated flare of CV.

Methods: We conducted a retrospective study in a tertiary referral center until March 25, 2020.

COVID-19 Vaccination and Glomerulonephritis.

Introduction: mRNA COVID-19 vaccine is more effective than traditional vaccines owing to superior immune activation. Nevertheless, the impact of mRNA COVID-19 vaccine on triggering /relapsing glomerulonephritis (GN) is limited. We report a case series of patients who developed new or relapsing GN postvaccination.

MARS: Should I Use It?

Severe liver failure, including acute liver failure and acute-on-chronic liver failure, is associated with high mortality, and many patients die despite aggressive medical therapy. While liver transplantation is a viable treatment option for liver failure patients, a large proportion of these patients die given the shortage in the liver donation and the severity of illness, leading to death while waiting for a liver transplant. Extracorporeal liver support devices, including molecular adsorbent recirculating system (MARS), have been developed as bridge to transplantation (bridge for patients who are decompensating while waiting for liver transplantation) and bridge to recovery (for whom recovery is deemed reasonable).

Ultrasonographic Assessment of Extravascular Lung Water in Hospitalized Patients Requiring Hemodialysis: A Prospective Observational Study.

Introduction: Sonographic technologies can estimate extravascular lung water (EVLW) in hemodialysis (HD) patients. This study investigated the suitability of a handheld scanner in contrast to a portable scanner for quantifying EVLW in hospitalized patients requiring HD.

Methods: In this prospective study, 54 hospitalized HD patients were enrolled.

Characteristics of Patients with End-Stage Kidney Disease in ADPKD.

Introduction: Cystic expansion damaging the parenchyma is thought to lead to end-stage kidney disease (ESKD) in autosomal dominant polycystic kidney disease (ADPKD). Here we characterized genotypic and phenotypic attributes of ADPKD at time of ESKD.

Methods: This is a retrospective cross-sectional study of patients with ADPKD with ESKD evaluated at Mayo Clinic with available abdominal computed tomography (CT) or magnetic resonance imaging (MRI).

Membranous nephropathy in a patient with coronavirus disease 2019 (COVID-19): A case report.

Introduction: Though respiratory, immune, and coagulation systems are major targets of coronavirus disease 2019 (COVID-19), kidney dysfunction, presenting with acute kidney injury (AKI), is also common. Most AKI cases in COVID-19 manifest as acute tubular injury (ATI) in conjunction with multiorgan failure. While initial renal pathological findings were limited to acute tubular necrosis and collapsing glomerulopathy, a recent case series reported a larger spectrum of findings.

Presymptomatic Screening for Intracranial Aneurysms in Patients with Autosomal Dominant Polycystic Kidney Disease.

Background And Objectives: Intracranial aneurysm rupture is the most devastating complication of autosomal dominant polycystic kidney disease. Whether selective or widespread intracranial aneurysm screening is indicated remains controversial.

Design, Setting, Participants & Measurements: Records of 3010 patients with autosomal dominant polycystic kidney disease evaluated at the Mayo Clinic between 1989 and 2017 were reviewed.

Bacterial Cholangitis in Autosomal Dominant Polycystic Kidney and Liver Disease.

Objective: To describe first episodes of bacterial cholangitis complicating autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (ADPLD) and to identify risk factors for cholangitis episodes among patients with ADPKD-associated polycystic liver disease (PLD).

Patients And Methods: We searched the electronic medical records at our tertiary referral center for episodes of cholangitis in patients with ADPKD or ADPLD from January 1, 1996, through June 30, 2017. Cases were categorized as suspected or definite cholangitis by expert review.

Long-Term Administration of Tolvaptan in Autosomal Dominant Polycystic Kidney Disease.

Background And Objectives: In the 3-year Tolvaptan Efficacy and Safety in Management of ADPKD and Its Outcomes (TEMPO) 3:4 and 1-year Replicating Evidence of Preserved Renal Function: an Investigation of Tolvaptan Safety and Efficacy in ADPKD (REPRISE) trials, tolvaptan slowed the decline of eGFR in patients with autosomal dominant polycystic kidney disease at early and later stages of CKD, respectively. Our objective was to ascertain whether the reduction associated with the administration of tolvaptan is sustained, cumulative, and likely to delay the need for kidney replacement therapy.

Design, Setting, Participants, & Measurements: One hundred and twenty-eight patients with autosomal dominant polycystic kidney disease participated in clinical trials of tolvaptan at the Mayo Clinic.

Cystatin C Predicts Renal Recovery Earlier Than Creatinine Among Patients With Acute Kidney Injury.

Introduction: Serum cystatin C increases earlier than creatinine during acute kidney injury. However, whether cystatin C decreases earlier during recovery is unknown. This retrospective study aimed to determine the temporal trend between creatinine and cystatin C in acute kidney injury.

Younger Adults Initiating Hemodialysis: Antidepressant Use for Depression Associated With Higher Health Care Utilization.

Objective: To examine associations between antidepressant use and health care utilization in young adults beginning maintenance hemodialysis (HD) therapy.

Patients And Methods: Antidepressant use, hospitalizations, and emergency department (ED) visits were examined in young adults (N=130; age, 18-44 years) initiating HD (from January 1, 2001, through December 31, 2013) at a midwestern US institution. Primary outcomes included hospitalizations and ED visits during the first year.

Autosomal Dominant Polycystic Kidney Patients May Be Predisposed to Various Cardiomyopathies.

Introduction: Mutations in and cause autosomal dominant polycystic kidney disease (ADPKD). Experimental evidence suggests an important role of the polycystins in cardiac development and myocardial function. To determine whether ADPKD may predispose to the development of cardiomyopathy, we have evaluated the coexistence of diagnoses of ADPKD and primary cardiomyopathy in our patients.