Unlabelled: Resistant nephrotic syndrome is a group of disorders with diverse histological findings, which are by definition resistant to corticosteroids given in adequate dose for adequate duration and many are resistant to other therapy as well. In many patients progression to end-stage renal disease is the ultimate outcome. The role of B cells has not been fully explained in man, agents that specifically interfere with B cells would ideally represent the first step toward selective therapy. We studied short term and long term effects of rituximab in patients with resistent primary nephrotic syndrome.
Material: Study was conducted at SMS-medical college and Hospital Jaipur, four doses of rituximab were given weakly, in fixed dose of 500 mg per dose and proteinuria was evaluated before start of therapy and at 3 months, 6 months and 12 months of therapy. Patients with resistant primary nephrotic syndrome who failed to respond to other therapies, with stable eGFR >30, and controlled BP were included in study. Patients with Active infection, Uncontrolled hypertension, pregnancy were excluded from study.
Observation: 10 patients were enrolled in study out of which 7 FSGS (focal segmental glomeruloscllerosis) and 3 were IMN (idiopathic membranous nephropathy), 5 were female and 5 were male, age 17-61years (average 34.6), weight were 48-70 kg (avg 57.9), BMI 19.4-23 (AVG 21.18), all patients have normal renal function (average creatinine value of 0.8, range= 0.5 to 1.1). At 3 months 1 patient had partial response and 9 had no response. At 6 months of treatment 2 patients had partial response, 3 had complete response and 5 no response. At 12 months of treatment 4 had partial response, 5 had complete response and 1 no response. Out of 10 patients no one had relapse of Nephrotic syndrome at 12 month of therapy. Renal function remain normal in all patients over 12 months followup.
Conclusion: This prospective, observational study evaluated 3 month, 6 month, and 12 month outcome of 3 IMN and 7 FSGS patients, with persistent nephrotic range proteinuria and showed that rituximab promoted sustained remission in proteinuria in resistent nephrotic syndrome with normal renal function.
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JAMA Pediatr
January 2025
Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Importance: Cyclophosphamide and calcineurin inhibitors are the most used nonsteroid immunosuppressive medications globally for children with various chronic inflammatory conditions. Their comparative effectiveness remains uncertain, leading to worldwide practice variation. Nephrotic syndrome is the most common kidney disease managed by pediatricians globally and suboptimal treatment is associated with high morbidity.
View Article and Find Full Text PDFPak J Med Sci
January 2025
Khemchand N Moorani, MBBS, MCPS, FCPS, IPNA Pediatr Nephrol fellowship Professor, Department of Pediatric Nephrology, The Kidney Centre Post Graduate Training Institute, Karachi. 197/9, Rafiqui Shaheed Road, Karachi-75530, Pakistan.
Objectives: To determine the effectiveness of Levamisole (Leva) in maintaining short-term and long-term remission in early steroid responders (ESRs) and late steroid responders (LSRs).
Methods: This retrospective study on 106 cohorts, aged 2-14 years with frequent-relapsing (FR) and steroid-dependent nephrotic syndrome (SDNS) who received Leva over 10-years (2012-2023), was carried out at tertiary care centre, Karachi from January-August 2023. Patients were categorized based on steroid response during first episode of NS as ESRs if complete remission (CR) was achieved within two weeks of daily steroid and LSRs if CR achieved between two-four weeks.
Introduction Nephrotic syndrome, an unusual clinical presentation of IgA nephropathy (IgAN), occurs only in a few cases. The data regarding its clinical characteristics and treatment outcomes are lacking. Material and methods In this retrospective analysis, we reviewed kidney biopsies conducted between January 2007 and December 2018.
View Article and Find Full Text PDFInt Med Case Rep J
January 2025
Department of Neurology, The Lishui Hospital of Wenzhou Medical University, The First Affiliated Hospital of Lishui University, Lishui People's Hospital, Lishui, Zhejiang, 323000, People's Republic of China.
Primary amyloidosis (AL type) is a systemic disease that can lead to structural and functional damage to organs and tissues such as the kidney, heart and liver with non-specific symptoms. Most of the affected patients develop thyroid infiltration and thus diffuse enlargement of the thyroid gland, while cases leading to hypothyroidism are exceedingly rare. Some researchers have analyzed thyroid function in newly diagnosed patients with AL amyloidosis, and found that the incidence of overt hypothyroidism is only 7%.
View Article and Find Full Text PDFBiomedicines
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.
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