Objective: To investigate the efficacy and adverse effect of mycophenolate mofetil (MMF) in the treatment of frequently relapsing nephrotic syndrome in children.
Methods: The study population consisted of 37 children (24 simple nephrotic syndrome and 13 nephritis-type syndrome) suffering from frequently relapsing nephrotic syndrome. Patients received 20-30 mg/(kg d) of MMF in conjunction with 1 mg/(kg d) prednisone for 3-6 months.
Results: Out of 24 patients suffered from simple nephrotic syndrome, 17 patients (70.8%) with complete relief, 4 patients (16.7%) with partial relief and 3 patients (12.5%) with non-relief, whereas out of 13 patients suffered from nephritis-type syndrome 6 patients (46.2%) with complete relief, 3 patients (23.1%) with partial relief and 4 patients (30.7%) with non-relief. Eight patients with Minimal Change Disease (MCD) achieved complete relief. Of 23 patients with Mesangial Proliferative Glomerulonephritis (MsPGN) or Membranoproliferative Glomerulonephritis (MPGN), complete relief was observed in 17 patients (73.9%), partial relief in 4 patients (17.4%) and non-relief in 2 patients.
Conclusion: These Results suggest that MMF has better efficacy against simple renal disease than against nephritis-type syndrome, and MMF may be more suitable for the treatment of frequently relapsing nephrotic syndrome characterized by proliferative lesions.
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Biomedicines
January 2025
Second Department of Internal Medicine, Division of Nephrology, Kansai Medical University, Hirakata 573-1010, Japan.
: Charcot-Marie-Tooth (CMT) disease is an inherited peripheral neuropathy primarily involving motor and sensory neurons. Mutations in INF2, an actin assembly factor, cause two diseases: peripheral neuropathy CMT-DIE (MIM614455) and/or focal segmental glomerulosclerosis (FSGS). These two phenotypes arise from the progressive degeneration affecting podocytes and Schwann cells.
View Article and Find Full Text PDFDiagnostics (Basel)
January 2025
Laboratory of Renal Pathophysiology, Hospital das Clínicas, Faculty of Medicine, University of São Paulo, São Paulo 05508-220, Brazil.
: Focal segmental glomerulosclerosis (FSGS) is a pattern of kidney injury with diverse causes and pathogeneses, resulting in podocyte injury and depletion. It can be classified as primary, genetic, or secondary. Because FSGS classically has a worse prognosis in patients with nephrotic syndrome, most studies have focused on the treatment and evolution of these patients, resulting in a lack of data related to patients without nephrotic syndrome.
View Article and Find Full Text PDFPediatr Nephrol
January 2025
Department of Pediatrics, University of Cologne, Faculty of Medicine and University Hospital Cologne, Kerpener Street 62, 50937, Cologne, Germany.
The integrity of the filtration barrier of the kidney relies on the proper composition of podocyte interdigitating foot processes. Their architecture is supported by a complex actin-cytoskeleton. Following podocyte stress or injury, podocytes encounter structural changes, including rearrangement of the actin network and subsequent effacement of the foot processes.
View Article and Find Full Text PDFIndian J Nephrol
July 2024
Departments of Nephrology, Christian Medical College Vellore, Ranipet Campus, Ranipet, India.
Background: Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a rare entity classified under the umbrella of monoclonal gammopathy of renal significance. The clinical implications of circulating monoclonal immunoglobulin (MIg), light chain restriction on immunofluorescence (IF) microscopy, histopathological pattern, and type of therapy on renal outcomes are not clearly defined.
Materials And Methods: Sixteen patients of PGNMID diagnosed between 2013 and 2020 were included from a biopsy registry of 11,459 patients at a single center.
Iran J Basic Med Sci
January 2025
Department of Nephrology, Affiliated Hospital of Jiangnan University, Wuxi, China.
Objectives: To investigate the natural product sulforaphane (SFN) in protection of membranous nephropathy (MN) by inhibiting oxidative stress-associated podocyte pyroptosis.
Materials And Methods: A passive Heymann nephritis (PHN) model was established and treated with SFN. Clinical manifestations were examined by testing 24-hr urine protein, albumin, total cholesterol, triglyceride, high-density and low-density lipoprotein levels.
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