scholarly journals Immunosuppressive therapy in children with steroid-resistant, frequently-relapsing, and steroid-dependent idiopathic nephrotic syndrome: a single center experience

10.19082/2039 ◽  
2016 ◽  
Vol 8 (2) ◽  
pp. 2039-2047 ◽  
Author(s):  
Bahia Hassan Moustafa ◽  
Omar Atef Tolba
Keyword(s):  
Nephrotic Syndrome ◽  
Immunosuppressive Therapy ◽  
Idiopathic Nephrotic Syndrome ◽  
Single Center ◽  
Steroid Resistant ◽  
Single Center Experience ◽  
Steroid Dependent

scholarly journals Immunosupressive therapy in children with steroid-resistant nephrotic syndrome: single center experience

2013 ◽  
Vol 35 (3) ◽  
pp. 200-205 ◽  
Author(s):  
Catalina Velez Echeverri ◽  
Gustavo Adolfo Zuluaga Valencia ◽  
Lina Maria Serna Higuita ◽  
Ana Katherina Serrano Gayubo ◽  
Carolina Lucia Ochoa ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Single Center ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Single Center Experience ◽  
Immunosupressive Therapy

MO291RITUXIMAB IS EFFECTIVE AND SAFE IN ADULTS WITH STEROID-DEPENDENT NEPHROTIC SYNDROME: A LONG-TERM, SINGLE-CENTER EXPERIENCE

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Gemma Patella ◽  
Alessandro Comi ◽  
Giuseppe Coppolino ◽  
Nicolino Comi ◽  
Giorgio Fuiano ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Case Series ◽  
Adult Patients ◽  
Single Center ◽  
Single Center Experience ◽  
Steroid Dependent ◽  
The Mean ◽  
Steroid Dependent Nephrotic Syndrome

Abstract Background and Aims Steroid-dependent nephrotic syndrome (SDNS) may require a prolonged multi-drug therapy with risk of drug toxicity and renal failure. Rituximab (RTX) treatment has been found to be helpful in reducing the steroid dosage and the need for immunosuppressants (ISs), but little data are currently available regarding very long-term outcomes in adults. We herein describe a long-term, single-center experience of RTX use in a large series of adults with SDNS. Method We studied 23 adult patients with SDNS (mean age 54.2±17.1 y; 65% male; BMI 28.5±4.7), mostly consequent to membranous (47.8%) or focal glomerulonephritis (30.2 %) who were eligible to start a RTX regimen. Before entering the RTX protocol, proteinuria and eGFR were 7.06±3.87 g/24h and 65.9±28.2 ml/min/1.73 m2, respectively; albumin and CD19/CD20 ratio were 2.9±0.9 g/L and 0.99±0.01 respectively; the mean number of ISs was 2.39±0.89 and the mean annual rate of relapses was 2.2±0.9. Results Patients were followed over a mean follow-up of 64 months (range: 12-144). After RTX (mean dose: 1202.1±372.4 mg) the rate of relapses was virtually nullified (p<0.001). eGFR remained roughly stable (62.1±19.8 ml/min/1.73 m2, p=NS), while proteinuria, albumin, CD19/CD20 and BMI all significantly improved (p ranging from 0.01 to 0.001). The mean number of additional ISs was also reduced (0.44±0.12; p<0.001) and RTX enabled discontinuation of steroids in 13/23 (56.5%) patients. No major adverse events related to therapy were recorded. Conclusion Findings from this large case-series with a remarkable very long follow-up reinforce the role of RTX as an efficient and safe weapon to improve outcomes in adult patients suffering from SDNS.

scholarly journals Management of idiopathic nephrotic syndrome in childhood

2004 ◽  
Vol 132 (9-10) ◽  
pp. 352-359 ◽  
Author(s):  
Amira Peco-Antic
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Alkylating Agents ◽  
Oral Prednisone ◽  
Symptomatic Treatment ◽  
Dietary Regimen ◽  
Steroid Resistant ◽  
Steroid Dependent ◽  
Frequent Relapses ◽  
Steroid Sensitive

The management of idiopathic nephrotic syndrome (INS) in children includes immunosuppressive and symptomatic treatment. The response to corticosteroid therapy is the best prognostic marker of the disease. The majority of children with INS (about 85%) are steroid-sensitive as they normalize proteinuria within 4 weeks of daily, oral prednisone administration. The most of steroid-sensitive patients (94%) has minimal change of nephrotic syndrome, while the majority (80.5%-94.4%) of those who are steroid-resistant has focal segmental glomerulosderosis or mesangioproliferative glomerulonephritis. Initial therapy of INS consists of 60 mg/m2/day prednisone daily for 4 weeks followed by 40 mg/m2 on alternate days for 4 weeks, thereafter decreasing alternate day therapy every month by 25% over the next 4 months. Thus, the overall duration of the initial cortico-steroids course is 6 months that may be significantly protective against the future development of frequent relapses. Approximately 30% of patients experience only one attack and are cured after the first course of therapy; 10-20% have only 3 or 4 steroid-responsive episodes before permanent cure; the remaining 40-50% of patients are frequent relapsers, or steroid-dependent. Standard relapse therapy consists of 60 mg/m2/ day prednisone until urine is protein free for at least 3 days, followed by 40 mg/m2 on alternate days for 4 weeks. The treatment of frequent-relapses and steroid-dependent INS includes several different regimens: maintenance (6 months) alternate steroid therapy just above steroid threshold (0.1-0.5 mg/kg/ 48h), levamisole, alkylating agents (cyclophosphamide or chlorambucil) or cyclosporine. The worse prognosis is expected in steroid-resistant patients who are the most difficult to treat. Renal biopsy should be performed in them. At present, there is no consensus on therapeutic regimen for steroid-resistant patients. The following immunosuppressive drugs have been used with varying success: cyclosporine, intravenous methyl prednisone pulses alone or combined with or followed by alkylating agents, plasma-exchange, and angiotensin-converting enzyme (ACE) inhibitors. Symptomatic treatment includes: 1) dietary regimen with normal protein intake and salt restriction, 2) calcium and vitamin D are prescribed with steroids, 3) diuretics should be used in case of severe edema, 4) infusion of albumin in case of severe hypovolemia, 5) treatment of hypertension, 6) anticoagulant therapy, and 7) prophylactic antibiotics in high-risk patients.

scholarly journals Immunosuppressive therapy in children with primary nephrotic syndrome: single center experience, Karachi, Pakistan

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Khemchand Netaram Moorani ◽  
Harnam Moolchand Hotchandani ◽  
Aasia Mohammad Zubair ◽  
Neelesh Chander Lohana ◽  
Nanga Ram Veerwani
Keyword(s):  
Nephrotic Syndrome ◽  
Immunosuppressive Therapy ◽  
Single Center ◽  
Primary Nephrotic Syndrome ◽  
Single Center Experience

Cyclosporine in the treatment of steroid-dependent and steroid-resistant idiopathic nephrotic syndrome in children

1998 ◽  
Vol 30 (8) ◽  
pp. 4156-4157 ◽  
Author(s):  
C Garcia ◽  
T Michelon ◽  
V Barros ◽  
D Mota ◽  
A Uhlmann ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Steroid Resistant ◽  
Steroid Dependent
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