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Pathogenesis of Focal Segmental Glomerulosclerosis and Related Disorders.
Annu Rev Pathol
January 2025
Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas, USA; email:
Focal segmental glomerulosclerosis (FSGS) is the morphologic manifestation of a spectrum of kidney diseases that primarily impact podocytes, cells that create the filtration barrier of the glomerulus. As its name implies, only parts of the kidney and glomeruli are affected, and only a portion of the affected glomerulus may be sclerosed. Although the diagnosis is based primarily on microscopic features, patient stratification relies on clinical data such as proteinuria and etiological criteria.
View Article and Find Full Text PDF[Short-term efficacy of rituximab in children with calcineurin inhibitor resistant steroid resistant nephrotic syndrome].
Zhonghua Er Ke Za Zhi
January 2025
Department of Nephrology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai Kidney Development and Pediatric Kidney Disease Research Center State, Key Laboratory of Kidney Diseases, Shanghai201102, China.
To investigate the short-term efficacy and safety of rituximab (RTX) in children with calcineurin inhibitor (CNI) resistant steroid resistant nephrotic syndrome (SRNS). A retrospective case analysis was conducted. Thirteen children with CNI resistant SRNS who were regularly treated with RTX (375 mg/m per dose (maximum dose 500 mg), 1 dose per week, a total of 4 doses) in Department of Nephrology, Children's Hospital of Fudan University from January 2016 to December 2023 were enrolled.
View Article and Find Full Text PDFClinical significance of concomitant IgM and C3 deposition in patients with focal segmental glomerulosclerosis.
J Nephrol
January 2025
Laboratory of Renal Pathophysiology, Hospital das Clínicas, University of São Paulo School of Medicine, 455 Dr Arnaldo Av, São Paulo, 01246903, Brazil.
Novel mutations related to simultaneous congenital nephropathy and ovarian insufficiency.
Clin Kidney J
January 2025
Department of Nephrology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy, Beijing, China.
Nucleoporins, as major components of nuclear pore complex, have been recently discovered to participate in organ development. Here, we report a young female patient with nephrotic proteinuria resistant to immune suppressant treatment and congenital ovarian insufficiency. Renal pathology confirmed focal segmental glomerulosclerosis and whole-exome sequencing revealed compound heterozygous mutations in Nucleoporin 160 (), NM_015231.
View Article and Find Full Text PDFEtiological spectrum and short-term outcomes of acute kidney injury in hospitalised patients of uncertain aetiology who underwent percutaneous renal biopsy.
J Pak Med Assoc
January 2025
Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
Objectives: To analyse the aetiological spectrum, clinical presentation and short-term renal outcomes of patients with acute kidney injury of uncertain aetiology subjected to percutaneous renal biopsy.
Methods: The prospective, cohort study was conducted at the Sindh Institute of Urology and Transplantation, Karachi, from March 1 to October 30, 2024, and comprised hospitalised acute kidney injury patients of either gender aged 18- 75 years who had been subjected to percutaneous renal biopsy. The patients were followed up for at least three months from the time of the biopsy.
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