Mycophenolate Mofetil in Children with Steroid/cyclophosphamide-resistant Nephrotic Syndrome
Overview
Pediatrics
Authors
Affiliations
The purpose of this study was to assess the results of therapy with mycophenolate mofetil (MMF) in children with idiopathic nephrotic syndrome (INS) who were both steroid- and cyclophosphamide-resistant. Treatment lasted a minimum of 6 months, and follow-up data were collected over a 2-year period. The children were divided into two groups: Group 1 (n=34) comprised patients who had received cyclosporine A (CsA) before the initiation of MMF therapy; Group 2 (n=18) comprised patients who received only MMF. Among the 34 patients of Group 1, complete and partial remission were achieved in seven (20.6%) and 13 patients (38.6%), respectively; there was no response in 14 patients (41.2%). Among the 18 patients in Group 2, complete and partial remission occurred in five (27.8%) and six (33.3%) patients, respectively; there was no response in seven patients (38.9%). Eight patients developed chronic kidney disease. The main side-effects were gastrointestinal complaints (n=11, 21%), recurring severe infections (n=1, 1.9%), and mild thrombocytopenia/leucopenia (n=1, 1.9%). MMF proved to be therapeutically effective in 59.5% of the cases. These beneficial effects need to be confirmed in studies with a long-term follow-up after discontinuation of the treatment. Our statistical analysis of the results of therapy with MMF did not reveal any significant difference between its use alone or following CsA administration.
Current understandings in treating children with steroid-resistant nephrotic syndrome.
Lee J, Kronbichler A, Shin J, Oh J Pediatr Nephrol. 2020; 36(4):747-761.
PMID: 32086590 PMC: 7910243. DOI: 10.1007/s00467-020-04476-9.
Treatment of nephrotic syndrome: going beyond immunosuppressive therapy.
Zhao J, Liu Z Pediatr Nephrol. 2019; 35(4):569-579.
PMID: 30904930 DOI: 10.1007/s00467-019-04225-7.
Treatment of steroid-resistant nephrotic syndrome in the genomic era.
Bensimhon A, Williams A, Gbadegesin R Pediatr Nephrol. 2018; 34(11):2279-2293.
PMID: 30280213 PMC: 6445770. DOI: 10.1007/s00467-018-4093-1.
Mycophenolate mofetil for sustained remission in nephrotic syndrome.
Querfeld U, Weber L Pediatr Nephrol. 2018; 33(12):2253-2265.
PMID: 29750317 DOI: 10.1007/s00467-018-3970-y.
Iorember F, Aviles D, Kallash M, Bamgbola O Pediatr Nephrol. 2017; 33(2):261-267.
PMID: 28864927 DOI: 10.1007/s00467-017-3789-y.