Pediatric Nephrotic Syndrome
Conditions
Interventions
Group 1 (retrospective section):none
Group 2:Standard-dose rituximab induction in month 1, followed by standard-dose rituximab maintenance every 6 months
Group 3:After standard-dose rituximab induction in the first month, maintenance therapy with standard-dose rituximab is administered every 3 months within the next half year, followed by maintenance e
Group 4:Following standard-dose rituximab induction in the first month, standard-dose rituximab maintenance is administered monthly for the subsequent 2 months, then every 6 months thereafter.
Sponsors
Xiangya Hospital, Central South University
Eligibility
Sex/Gender
All
Age
1.4 Years to 18 Years
Inclusion criteria
Inclusion criteria: Subjects must meet one of the following criteria: 1. Patients exhibiting Frequently Relapsing Nephrotic Syndrome (FRNS) or Steroid-Dependent Nephrotic Syndrome (SDNS) either during or after combined treatment with steroids and Tacrolimus or Mycophenolate Mofetil. 2. Patients with initial-onset nephrotic syndrome who exhibit Steroid-Resistant Nephrotic Syndrome (SRNS) after 6 weeks of standard Prednisolone treatment. 3. Patients exhibiting Late-Onset Steroid-Resistant Nephrotic Syndrome or Complex Relapse of Nephrotic Syndrome, showing no remission after 4–6 weeks of standard Prednisone treatment. 4. Patients with Secondary Nephrotic Syndrome excluded, diagnosed with Nephritic Syndrome (Nephritic-type NS) and exhibiting Steroid-Dependence, Frequent Relapses, or Steroid-Resistance. This includes: Primary IgA Nephropathy, Primary Membranous Nephropathy, Mesangial Proliferation with IgA and IgM deposits, and Mesangial Proliferation with "Full House" (Mantle) immune complex deposits. Complete Remission: All patients must have achieved Complete Remission through any of the following treatments: (1) Extended Standard Prednisone Therapy: Full-dose Prednisone for 6 weeks. (2) Combination Therapy: Standard Prednisone treatment combined with Tacrolimus or Mycophenolate Mofetil. (3) Methylprednisolone Pulse Therapy or, if necessary, combined with Cyclophosphamide Pulse Therapy.
Exclusion criteria
Exclusion criteria: 1.Secondary Nephrotic Syndrome: Patients diagnosed with secondary nephrotic syndrome. 2. Steroid-Nonresponsive Nephrotic Syndrome: Failure to achieve complete remission of proteinuria despite salvage therapy with methylprednisolone pulse therapy, combined methylprednisolone and cyclophosphamide pulse therapy, or combined standard prednisone therapy with tacrolimus or mycophenolate mofetil. 3. Severe Infections: Patients with severe infections, such as sepsis, severe pneumonia, Pneumocystis jirovecii pneumonia, pulmonary tuberculosis, and invasive fungal diseases. 4. Uncontrolled Active Viral Infections: Patients with active viral infections, such as human immunodeficiency virus (HIV) and hepatitis B virus (HBV) infections. 5. Live Vaccine Vaccination: Administration of live vaccines within 4 weeks prior to enrollment. 6. Patients with Renal Insufficiency. 7. Heart Disease: Patients with a history of heart failure, myocardial infarction, or severe arrhythmias. 8. Autoimmune Diseases: Patients with a history of autoimmune diseases,including Hashimoto's disease (chronic thyroiditis), Crohn's disease, ulcerative colitis, rheumatoid arthritis, idiopathic thrombocytopenic purpura, systemic lupus erythematosus, autoimmune hemolytic anemia, scleroderma, or Henoch-Schönlein purpura, among others. 9. Malignant tumors: Patients with malignant tumors or a history of malignant tumors. 10. Other diseases: Patients with other diseases that may cause secondary kidney disease. 11. Laboratory abnormalities: Patients with the following abnormal clinical laboratory values:white blood cells < 2000/µL,neutrophils < 1500/µL,platelets < 50000/µL.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Duration of complete or sustained remission;Reasons for recurrence;Mean dose of Rituximab;Tapering and discontinuation time of steroids / Tacrolimus / Mycophenolate Mofetil; | — |
Secondary
| Measure | Time frame |
|---|---|
| serum IgG level;peripheral blood B lymphocyte count; | — |
Countries
China
Contacts
Public ContactWeixi Zhang
Department of Pediatrics, Xiangya Hospital, Central South University
Outcome results
None listed