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.

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).

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.

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.

The Rate of Acute Kidney Injury After Total Hip Arthroplasty Is Low but Increases Significantly in Patients with Specific Comorbidities.

Background: The purpose of this study was to investigate the rate and risk factors associated with the development of acute kidney injury after total hip arthroplasty, including the perioperative use of nonsteroidal anti-inflammatory drugs (NSAIDs).

Methods: We retrospectively collected the demographic and comorbidity data of all patients who underwent total hip arthroplasty between 2004 and 2014 at our institution (n = 8,949). We conducted analyses of the entire cohort and a nested case-control subset.

Renal Outcomes in Patients With IgA Nephropathy Undergoing Liver Transplant: A Retrospective Cohort Study.

Background: End-stage liver disease (ESLD) is the most common cause of secondary immunoglobulin A nephropathy (IgAN). Multiple mechanisms have been proposed to explain the association between liver disease and IgAN. Although some mechanisms are expected to reverse in patients after liver transplant, the long-term renal prognosis is unclear for these patients.

Charcoal hemoperfusion in the treatment of medically refractory pruritus in cholestatic liver disease.

Background: Pruritus is a distressing symptom in a considerable proportion of cholestatic patients and a few of them do not respond to conventional treatment. Charcoal hemoperfusion (CH) is an extracorporeal technique that is effective in eliminating protein-bound substances which may have accumulated during cholestasis. Several case reports have shown significant reduction of bilirubin in mechanical jaundice and neonatal hemolytic jaundice.

Calciphylaxis: A Disease of Pannicular Thrombosis.

Objective: To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival.

Patients And Methods: Study subjects were 101 patients with calciphylaxis seen at Mayo Clinic from 1999 to September 2014. Data including thrombophilia profiles were extracted from the medical records of each patient.

Survival, Risk Factors, and Effect of Treatment in 101 Patients With Calciphylaxis.

Objective: To report on the survival and the associations of treatments upon survival of patients with calciphylaxis seen at a single center.

Patients And Methods: Using the International Classification of Diseases, Ninth Revision diagnosis code of 275.49 and the keyword "calciphylaxis" in the dismissal narrative, we retrospectively identified 101 patients with calciphylaxis seen at our institution between January 1, 1999, through September 20, 2014, using a predefined, consensus-developed classification scheme.

Native Nephrectomy in Renal Transplant Recipients with Autosomal Dominant Polycystic Kidney Disease.

Background: Native nephrectomy (NNx) is often done in patients with autosomal dominant polycystic kidney disease (ADPKD). Controversy exists concerning the need and timing of nephrectomy in transplant candidates. We hypothesize that post-transplant NNx does not negatively impact patient and graft survival.

Effect of genotype on the severity and volume progression of polycystic liver disease in autosomal dominant polycystic kidney disease.

Background: The autosomal dominant polycystic kidney disease (APDKD) genotype influences renal phenotype severity but its effect on polycystic liver disease (PLD) is unknown. Here we analyzed the influence of genotype on liver phenotype severity.

Methods: Clinical data were retrieved from electronic records of patients who were mutation screened with the available liver imaging (n = 434).

Extracorporeal Elimination of Piperacillin/Tazobactam during Molecular Adsorbent Recirculating System Therapy.

Use of the Molecular Adsorbent Recirculating System (MARS) as a liver support device continues to grow worldwide. Various components of the MARS circuit remove both protein-bound and water-soluble molecules. Little is known about the extent of the enhanced clearance mechanisms used in MARS therapy on drug elimination.

Volume regression of native polycystic kidneys after renal transplantation.

Background: The natural course of native kidneys after renal transplantation (RT) or dialysis in patients with autosomal dominant polycystic kidney disease (ADPKD) remains poorly understood.

Methods: We measured the total volumes of native kidneys and liver in 78 and 68 ADPKD patients, respectively, who had pre-transplant (within 2 years) and at least one post-transplant computed tomography (CT)/magnetic resonance imaging (MRI); in 40 patients with at least two post-transplant but no pre-transplant CT/MRIs; in 9 patients on chronic hemodialysis with at least one CT/MRI before and after beginning dialysis; and in 5 patients who had no image before and more than one image after dialysis. The last imaging was used in patients with multiple studies.

Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials.

The rate of renal disease progression varies widely among patients with autosomal dominant polycystic kidney disease (ADPKD), necessitating optimal patient selection for enrollment into clinical trials. Patients from the Mayo Clinic Translational PKD Center with ADPKD (n=590) with computed tomography/magnetic resonance images and three or more eGFR measurements over ≥6 months were classified radiologically as typical (n=538) or atypical (n=52). Total kidney volume (TKV) was measured using stereology (TKVs) and ellipsoid equation (TKVe).

Cardiovascular disease burden and risk factors before and after kidney transplant.

Cardiovascular (CV) disease is the most common cause of mortality among kidney transplant candidates on the waiting-list and after kidney transplantation. The mechanisms of cardiovascular disease burden after transplant are multifactorial and the risk is largely determined by pre-transplant factors including CV disease and dialysis duration. Current pre-transplant cardiac evaluation protocols have proven to be inconsistent in predicting adverse cardiovascular outcome post-transplant.

Rapid self-infusion of tap water.

Intravenous self-infusion of tap water has never been reported in the literature.  We present a 24-year-old healthy man who self-administered 2.5 L of tap water over 2 hours and developed acute illness including fever, change of mental status, acute hemolysis, low-grade disseminated intravascular coagulation, and acute kidney injury.

Severe anion gap metabolic acidosis from acetaminophen use secondary to 5-oxoproline (pyroglutamic acid) accumulation.

Anion gap metabolic acidosis (AGMA) is commonly encountered in medical practice. Acetaminophen-induced AGMA is, however, not widely recognized. We report 2 cases of high anion gap metabolic acidosis secondary to 5-oxoproline accumulation resulting from acetaminophen consumption: the first case caused by acute one-time ingestion of large quantities of acetaminophen and the second case caused by chronic repeated ingestion in a patient with chronic liver disease.

Urolithiasis and the risk of ESRD.

Background And Objectives: The contribution of urolithiasis, if any, to the development of ESRD is unclear.

Design, Setting, Participants, & Measurements: All stone formers in Olmsted County, Minnesota, first diagnosed between 1984 and 2008 were identified by diagnostic codes with up to four controls matched on age and sex. Charts were reviewed to validate symptomatic stone formers in a random subset.