23 research outputs found

    Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

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    Introduction: The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients. Methods: Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment. Results: A total of 667 patients (506 IgAN, 161 IgAV) constitute the IgAN/IgAV cohort (382 adults, 285 children). At biopsy, those with IgAV were younger (13.0 years vs. 29.6 years, P < 0.001), more frequently white (89.7% vs. 78.9%, P = 0.003), had a higher estimated glomerular filtration rate (103.5 vs. 70.6 ml/min per 1.73 m2, P < 0.001), and lower serum albumin (3.4 vs. 3.8 g/dl, P < 0.001) than those with IgAN. Adult and pediatric individuals with IgAV were more likely than those with IgAN to have been treated with immunosuppressive therapy at or prior to enrollment (79.5% vs. 54.0%, P < 0.001). Conclusion: This report highlights clinical differences between IgAV and IgAN and between children and adults with these diagnoses. We identified differences in treatment with immunosuppressive therapies by disease type. This description of baseline characteristics will serve as a foundation for future CureGN studies

    Improving Primary Care Delivery for Patients Receiving Maintenance Hemodialysis

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    The beneficial impact of primary care, focused on all aspects of a patients\u27 health (rather than a disease-specific focus) is well established. Recognized benefits include greater receipt of preventive care and counseling, lower utilization of emergency care and hospitalization for ambulatory care sensitive conditions, and decreased early mortality. While the importance of primary care and care coordination at the primary care-specialty interface is well recognized, the role of primary care within traditional and emerging care models for patients receiving maintenance in-center hemodialysis remains ill-defined. In this perspective article, we will describe: 1) the role of primary care for patients receiving maintenance hemodialysis and the current evidence regarding the receipt of primary care among those patients; 2) the key challenges to delivery of primary care for these complex patients, including suboptimal care coordination between nephrology and primary care providers (PCPs), the intensity of dialysis care, and the limited capacity of nephrologists and PCPs to meet the broad health needs of hemodialysis patients; 3) the potential strategies for improving the delivery of primary care for patients receiving hemodialysis; and 4) future research needs to improve primary care delivery for this high-risk population

    Predictors of Complication after Percutaneous Ultrasound-Guided Kidney Biopsy in HIV-Infected Individuals: Possible Role of Hepatitis C and HIV Co-infection

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    Background and objectives: HIV-infected patients often undergo kidney biopsy. The risks of percutaneous ultrasound-guided kidney biopsy in this population are not well established

    Inhibiting calpain 1 and 2 in cyclin G associated kinase–knockout mice mitigates podocyte injury

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    Evidence for reduced expression of cyclin G associated kinase (GAK) in glomeruli of patients with chronic kidney disease was observed in the Nephroseq human database, and GAK was found to be associated with the decline in kidney function. To examine the role of GAK, a protein that functions to uncoat clathrin during endocytosis, we generated podocyte-specific Gak-knockout mice (Gak-KO), which developed progressive proteinuria and kidney failure with global glomerulosclerosis. We isolated glomeruli from the mice carrying the mutation to perform messenger RNA profiling and unearthed evidence for dysregulated podocyte calpain protease activity as an important contributor to progressive podocyte damage. Treatment with calpain inhibitor III specifically inhibited calpain-1/-2 activities, mitigated the degree of proteinuria and glomerulosclerosis, and led to a striking increase in survival in the Gak-KO mice. Podocyte-specific deletion of Capns1, essential for calpain-1 and calpain-2 activities, also improved proteinuria and glomerulosclerosis in Gak-KO mice. Increased podocyte calpain activity–mediated proteolysis of IκBα resulted in increased NF-κB p65–induced expression of growth arrest and DNA-damage-inducible 45 beta in the Gak-KO mice. Our results suggest that loss of podocyte-associated Gak induces glomerular injury secondary to calcium dysregulation and aberrant calpain activation, which when inhibited, can provide a protective role
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