A5088796897- Genetic and Kidney Cyst Diseases
- Renal Diseases and Glomerulopathies
- Renal and related cancers
- Chronic Kidney Disease and Diabetes
- Biomedical Research and Pathophysiology
- Genetic Syndromes and Imprinting
- Pediatric Urology and Nephrology Studies
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Hedgehog Signaling Pathway Studies
- Cystic Fibrosis Research Advances
- Liver Disease Diagnosis and Treatment
- Genomics and Rare Diseases
- Vasculitis and related conditions
- Genomic variations and chromosomal abnormalities
- Renal cell carcinoma treatment
- Dialysis and Renal Disease Management
- Pancreatic function and diabetes
- Metabolism and Genetic Disorders
- Pregnancy and preeclampsia studies
- Central Venous Catheters and Hemodialysis
- Autoimmune Bullous Skin Diseases
- Renal and Vascular Pathologies
- Fetal and Pediatric Neurological Disorders
- Venous Thromboembolism Diagnosis and Management
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
Hypertension Institute
1997-2025
Mayo Clinic
2015-2024
WinnMed
2012-2024
Mayo Clinic in Florida
2009-2024
Children's Hospital of Philadelphia
2024
The Ohio State University Wexner Medical Center
2024
The Bronx Defenders
2023
Albert Einstein College of Medicine
2023
Mayo Clinic in Arizona
2001-2023
Children's Hospital at Montefiore
2023
The rate of renal disease progression varies widely among patients with autosomal dominant polycystic kidney (ADPKD), necessitating optimal patient selection for enrollment into clinical trials. Patients from the Mayo Clinic Translational PKD Center ADPKD (n=590) computed tomography/magnetic resonance images and three or more eGFR measurements over ≥6 months were classified radiologically as typical (n=538) atypical (n=52). Total volume (TKV) was measured using stereology (TKVs) ellipsoid...
Hypertension is common in autosomal dominant polycystic kidney disease (ADPKD) and associated with increased total volume, activation of the renin-angiotensin-aldosterone system, progression disease.In this double-blind, placebo-controlled trial, we randomly assigned 558 hypertensive participants ADPKD (15 to 49 years age, an estimated glomerular filtration rate [GFR] >60 ml per minute 1.73 m(2) body-surface area) either a standard blood-pressure target (120/70 130/80 mm Hg) or low (95/60...
Proteins associated with autosomal dominant and recessive polycystic kidney disease (polycystin-1, polycystin-2, fibrocystin) localize to various subcellular compartments, but their functional site is thought be on primary cilia. PC1+ vesicles surround cilia in Pkhd1del2/del2 mice, which led us analyze these structures detail. We subfractionated urinary exosome-like (ELVs) isolated a subpopulation abundant polycystin-1, fibrocystin (in cleaved forms), polycystin-2. This removed Tamm-Horsfall...
There are no proven, effective therapies for polycystic kidney disease (PKD) or liver (PLD). We enrolled 42 patients with severe PLD resulting from autosomal dominant PKD (ADPKD) (ADPLD) in a randomized, double-blind, placebo-controlled trial of octreotide, long-acting somatostatin analogue. randomly assigned 2:1 ratio to octreotide LAR depot (up 40 mg every 28+/-5 days) placebo 1 year. The primary end point was percent change volume baseline year, measured by MRI. Secondary points were...
Background and objectives: It was postulated that in patients with membranous nephropathy (MN), four weekly doses of Rituximab (RTX) would result more effective B cell depletion, a higher remission rate, maintaining the same safety profile compared treated RTX dosed at 1 g every 2 weeks. This hypothesis supported by previous pharmacokinetic (PK) analysis showing levels two-dose regimen were 50% lower nonproteinuric patients, which could potentially undertreatment. Design, setting,...
Hypertension develops early in patients with autosomal dominant polycystic kidney disease (ADPKD) and is associated progression. The renin–angiotensin–aldosterone system (RAAS) implicated the pathogenesis of hypertension ADPKD. Dual blockade RAAS may circumvent compensatory mechanisms that limit efficacy monotherapy an angiotensin-converting–enzyme (ACE) inhibitor or angiotensin II–receptor blocker (ARB).
IgA nephropathy frequently leads to progressive CKD. Although interest surrounds use of immunosuppressive agents added standard therapy, several recent studies have questioned efficacy these agents. Depleting antibody-producing B cells potentially offers a new therapy. In this open label, multicenter study conducted over 1-year follow-up, we randomized 34 adult patients with biopsy-proven and proteinuria >1 g/d, maintained on angiotensin-converting enzyme inhibitors or angiotensin receptor...
mRNA COVID-19 vaccine is more effective than traditional vaccines owing to superior immune activation. Nevertheless, the impact of on triggering de novo/relapsing glomerulonephritis (GN) limited. We report a case series patients who developed new or relapsing GN postvaccination.We evaluated baseline characteristics, type, and clinical outcomes 13 from our institution had diagnosis relapse their post-mRNA vaccination.Of patients, 8 were newly diagnosed with having 5 relapse. Median age was 62...
The application of deep learning for automated segmentation (delineation boundaries) histologic primitives (structures) from whole slide images can facilitate the establishment novel protocols kidney biopsy assessment. Here, we developed and validated networks structures on biopsies nephrectomies. For development, examined 125 Minimal Change Disease collected across 29 NEPTUNE enrolling centers along with 459 stained Hematoxylin & Eosin (125), Periodic Acid Schiff Silver (102), Trichrome...
We report on the successful, compassionate use of anti-CD20 chimeric monoclonal antibody rituximab in a patient with chronic, relapsing cytoplasmic antineutrophil (cANCA)-associated Wegener's granulomatosis (WG). The initially responded to treatment glucocorticoids and cyclophosphamide. However, bone marrow toxicity during cyclophosphamide relapse precluded its further use. Azathioprine mycophenolate mofetil had failed maintain remission WG, methotrexate was contraindicated. Because...
Renal pathology and clinical outcomes in patients with primary Sjögren's syndrome (pSS) who underwent kidney biopsy (KB) because of renal impairment are reported.Twenty-four 7276 pSS KB over 40 years. Patient cases were reviewed by a pathologist, nephrologist, rheumatologist. Presentation, laboratory findings, pathology, initial treatment, therapeutic response noted.Seventeen (17 24; 71%) had acute or chronic tubulointerstitial nephritis (TIN) as the lesion, TIN (11 17; 65%) most common...
<h3>Objectives</h3> Sustained disease-modifying antirheumatic drug (DMARD)-free status, the sustained absence of synovitis after cessation DMARD therapy, is infrequent in autoantibody-positive rheumatoid arthritis (RA), but approximates cure (ie, disappearance signs and symptoms). It was recently suggested that immunological remission, defined as anti-citrullinated protein antibodies (ACPA) factor (RF), underlies this outcome. Therefore, long-term observational study determined if...
The association of overweight/obesity with disease progression in patients autosomal dominant polycystic kidney (ADPKD) remains untested. We hypothesized that associates faster early-stage ADPKD. Overall, 441 nondiabetic participants ADPKD and an eGFR>60 ml/min per 1.73 m2 who participated the Halt Progression Polycystic Kidney Disease Study A were categorized on basis body mass index (BMI; calculated using nonkidney nonliver weight) as normal weight (18.5-24.9 kg/m2; reference; n=192),...
Fibrillary glomerulonephritis (FGN) is a rare disease with unknown pathogenesis and poor prognosis. Until now, the diagnosis of this has required demonstration glomerular deposition randomly oriented fibrils by electron microscopy that are Congo red negative stain antisera to Igs. We recently discovered novel proteomic tissue biomarker for FGN, namely, DNAJB9.In work, we developed DNAJB9 immunohistochemistry tested its sensitivity specificity FGN. This testing was performed on renal biopsy...
We showed in a randomized double-blinded placebo-controlled clinical trial that octreotide long-acting repeatable depot.® (OctLAR®) for 12 months reduces kidney and liver growth autosomal dominant polycystic patients with severe disease (PLD) isolated PLD. have now completed an open-label extension one additional year to assess safety benefits of continued use OctLAR 2 years (O→O) examined drug effect the placebo group who crossed over Year (P→O). The primary end point was change total...
Summary Background and objectives Autosomal dominant polycystic kidney disease (ADPKD) patients have an increased risk for intracranial aneurysms (IAs). The importance of screening unruptured IAs (UIAs) depends on their risks growth rupture. Design, setting, participants, & measurements ADPKD with UIAs found by presymptomatic magnetic resonance angiography (MRA) during 1989 to 2009 were followed initially at 6 months annually, less frequently after demonstration stability. Results...
Autosomal dominant polycystic kidney disease (ADPKD) is a common cause of ESRD. Affected individuals inherit defective copy either PKD1 or PKD2, which encode polycystin-1 (PC1) polycystin-2 (PC2), respectively. PC1 and PC2 are secreted on urinary exosome-like vesicles (ELVs) (100-nm diameter vesicles), in present cleaved form may be complexed with PC2. Here, label-free quantitative proteomic studies urine ELVs an initial discovery cohort (13 mutations 18 normal controls) revealed that 2008...