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Clinical presentation, outcomes and risk of relapses of lupus podocytopathy in a multicentre Italian cohort.
J Nephrol
January 2025
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20072, Milan, Italy.
Background: In an Italian cohort of lupus podocytopathy patients, we aimed to characterize the presenting features, therapy, and outcomes, and explore differences between relapsing and non-relapsing patients.
Methods: We identified 29 patients with lupus podocytopathy from 1994 to 2023 in 11 Italian Nephrology/Rheumatology Units, and divided them into two groups: relapsing and non-relapsing. Given the limited sample size, a p-value ≤ 0.
The impact of a secondary, rare, non-pathogenic PKD1 variant on disease progression in autosomal dominant polycystic kidney disease.
J Nephrol
January 2025
Department of Nephrology, Beaumont Hospital, Dublin, Ireland.
Background: Autosomal dominant polycystic kidney disease (ADPKD) is caused primarily by pathogenic variants in the PKD1 and PKD2 genes. Although the type of ADPKD variant can influence disease severity, rare, hypomorphic PKD1 variants have also been reported to modify disease severity or cause biallelic ADPKD. This study examines whether rare, additional, potentially protein-altering, non-pathogenic PKD1 variants contribute to ADPKD phenotypic outcomes.
View Article and Find Full Text PDFPast and future in vitro and in vivo approaches toward circulating factors and biomarkers in idiopathic nephrotic syndrome.
Pediatr Nephrol
January 2025
Division of Molecular Medicine, University of São Paulo School of Medicine, São Paulo, Brazil.
Predicting the risks of progression to chronic kidney disease (CKD) stage 5 in idiopathic nephrotic syndrome (NS) and recurrence of the disease (rNS) following kidney transplantation (KT) is a key assessment to provide essential management information. NS has been categorized etiologically as genetic and immune-based. A genetic cause can be identified in ~ 30% of children with steroid-resistant NS (SRNS), a finding associated with a very low risk of rNS following KT.
View Article and Find Full Text PDFRecent progress in xenotransplantation and its application to pediatric kidney disease.
Pediatr Nephrol
January 2025
Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, 105-8461, Japan.
Patients with kidney failure require dialysis or kidney transplantation. Kidney transplantation offers great benefits, including reduced mortality; however, many patients who wish to undergo kidney transplantation are unable to do so due to a shortage of donor organs. This shortage is a global issue, and xenotransplantation has emerged as a potential solution.
View Article and Find Full Text PDFClinical applicability of 2023 International Pediatric Nephrology Association recommended limited therapeutic drug monitoring formulae to assess mycophenolic acid exposure.
Pediatr Nephrol
January 2025
University of Western Ontario, London, ON, Canada.
Background: The 2023 IPNA guidelines recommended a 12-h mycophenolic acid (MPA) area under the curve (AUC) estimation for managing pediatric nephrotic syndrome and MPA AUC > 50 mg * h/L for an optimal therapeutic response to mycophenolate mofetil (MMF). The IPNA guidelines endorsed two limited AUC formulae based on three-point MPA measurements to predict 12-h MPA AUC. The relative performance of these two limited AUC formulae has not been tested.
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