Mycophenolate mofetil in children with steroid/cyclophosphamide-resistant nephrotic syndrome

Mello, Valderez Raposo de; Rodrigues, Maira Tinte; Mastrocinque, Tais Helena; Martins, Suelen Bianca Stopa; Andrade, Olberes Vitor Braga de; Guidoni, Eliana Biondi Medeiros; Scheffer, Débora da Luz; Filho, Dino Martini; Toporovski, Júlio; Benini, Vanda

doi:10.1007/s00467-009-1356-x

Cited by 41 publications

(19 citation statements)

References 25 publications

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“…Preliminary data raise the question of whether underexposure may result in relapses [35]. To date, the side effects of mycophenolate may be underreported: for example, de Mello et al [36] report severe gastrointestinal complications in some of their patients and the death of one of these patients, but they do not mention this side effect in the abstract.…”

Section: Mycophenolic Acidmentioning

confidence: 96%

New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome

2011

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Although many children with idiopathic nephrotic syndrome (INS) respond initially to steroid therapy, repeated courses for patients with relapses often cause significant steroid toxicity. Patients with frequent relapses who develop steroid dependency thus require alternative treatment. The first such options have been considered to be cyclophosphamide or levamisole, although the latter is no longer available in many countries. There is also an increasing body of data indicating that mycophenolic acid (MPA) may be an alternative for these patients. Calcineurin inhibitors (cyclosporine A or tacrolimus) are usually effective and often used after cytotoxic treatment, but long-term treatment with these agents is necessary, raising concerns of a possible accumulation of side effects. Some patients show a tendency to relapse even on such maintenance regimens, and some even have a refractory course that creates a medical dilemma. For this situation, recent data indicate that monoclonal antibodies directed to B-cells (e.g. rituximab) may have some effect and that such drugs may also prove to be a therapeutic option in less complicated cases. Patients that do not respond to steroid treatment need genetic testing and a renal biopsy since focal segmental glomerulosclerosis (FSGS) may be present. Treatment options include pulse methylprednisolone, often in addition to calcineurin inhibitors, mainly in the form of cyclosporine, but tacrolimus has also come into recent favor. Some studies have found cytotoxic treatment, especially intravenous cyclophosphamide, to be effective in steroid resistant nephrotic syndrome, but it seems to be inferior to calcineurin inhibitors. MPA and rituximab have also been used in children with primary FSGS, but the response seems to be inferior to that in patients with steroid sensitive nephrotic syndrome. Taken together, INS in both steroid-sensitive and steroid-resistant patients is a potentially complicated disorder, and despite a wide arsenal of immunological interventions, some patients have a treatment refractory course. Prospective studies or at least standardized treatment for complicated cases is urgently needed.

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Section: Mycophenolic Acidmentioning

confidence: 96%

New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome

2011

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“…Recently, MMF has been a commonly used steroid-sparing agent in childhood NS. In patients with SRNS, MMF gave rise to complete remission in 23-62% of patients and partial remission in 25-37% of patients (21)(22)(23). In our study, we used MMF with a mean duration of 26 months (10-44 months) and complete remission was achieved in 50% of patients.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Nephrotic Syndrome in Childhood: A Retrospective Analysis of Two Hundred and Eighty Nine Patients

Yılmaz¹,

Düşünsel²,

Dursun³

et al. 2018

Haseki

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“…Dilaporkan pada pemberian MMF selama minimal 6 bulan menghasilkan remisi komplit 23%-62%, remisi partial 25%-37%, dan tanpa remisi 8%-40%. 31 Apabila pasien sindrom nefrotik steroid resisten mengalami relaps kembali setelah pengobatan maka dianjurkan diberikan preparat kortikosteroid oral (2D) seperti pengobatan relaps, pada umumnya remisi cepat tercapai. Dapat juga kembali ke imunosupresan yang sebelumnya (2D), kecuali jika dipergunakan CPA untuk menghindari efek kumulatif (2D), atau menggantikan dengan obat imunupresan yang lain.…”

Section: Pengobatan Steroid Resisten Primer/ Sekunderunclassified

Pengobatan Terkini Sindrom Nefrotik (SN) pada Anak

Alatas

Trihono

2016

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Pengobatan sindrom nefrotik pada anak mengalami perubahan dari masa ke masa. Sudah sejak lama kita mengadopsi rekomendasi International study of kidney disease in children (SKDC) yaitu pemberian kortikosteroid 8 minggu, 4 minggu dosis penuh dilanjutkan dengan 4 minggu dosis alternating. Dengan skema pengobatan ini 80% sindrom nefrotik idiopatik pada anak mengalami remisi, tetapi 70%-80% di antaranya mengalami relaps dan separuhnya relaps berulang/frekuen. Pengobatan hanya 8 minggu dirasakan kurang adekuat (Kidney Disease Improving Global Outcome= KDIGO), maka KDIGO membuat rekomendasi baru pada tahun 2013. Pengobatan inisial dapat dipilih dengan pemberian kortikosteroid 12 minggu atau tetap 8 minggu dan dilanjutkan dengan penurunan dosis selama 2-3 bulan (tapering-off). Namun usulan KDIGO dibantah oleh Japanese Study Group of Kidney Disease in Children (JSKDC) tahun 2015 dengan melakukan penelitian untuk membandingkan prednisolon 4-4 minggu selama 6 bulan yaitu 4-4 minggu + tapering off. Hasil penelitian tersebut membuktikan bahwa pemberian prednison 4-4 minggu tidak lebih inferior dan pemberian 6 bulan tidak mengurangi relaps. Rekomendasi lain dari KDIGO 2013 diterima dengan sedikit modifikasi yang akan dikemukakan pada makalah ini secara rinci, baik untuk pengobatan sindrom nefrotik sensitif steroid (SNSS) maupun sindrom nefrotik resisten steroid (SNRS). Juga diajukan pemberian beberapa preparat kortikosteroid "sparing agent" yang dapat dipakai pada sindrom nefrotik relaps frekuen/dependen steroid. Sari Pediatri 2015;17(2):155-62. Kata kunci: rekomendasi ISKDC, KDIGO, kortikosteroid sparing agent Review article Current Updates in Management of Nephrotic Syndrome in ChildrenHusein Alatas, Partini P. Trihono, Taralan Tambunan, Sudung O.Pardede, Eka L. Hidayati Management of nephrotic syndrome (NS) in children has been evolving time to time. We have been adopted ISKDC (International study of kidney disease in children) recommendation which were the administration of 8 weeks course of steroid (4 weeks of full dose corticosteroid, followed by 4 weeks of alternate dose). Remission was achieved by 80% of idiopathic NS children, although 80% of them were relapsed and half of the relapser were the frequent one. Since the 8 weeks course of treatment was considered inadequate, KDIGO (Kidney Disease Improving Global Outcome) released new recommendation in 2013. Initial treatment was proposed to be 12 weeks or remain 8 weeks which was continued by 2-3 months of tappering off. Another publication comparing the 6 months course of prednisolon administration (4-4 weeks) to the previous recommendation (4-4 weeks followed by tappering off of prednisolon) by JSKDC in 2015 refuted the KDIGO recommendation. The publication reported that prednisolone administration of 4-4 weeks followed by tappering off was not inferior to 6 months course and was not reducing the relapse rate. Another recommendation from KDIGO 2013 was adopted with some modifications which are going to be presented in this article, for steroid sensitive NS and stero...

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Mycophenolate mofetil in children with steroid/cyclophosphamide-resistant nephrotic syndrome

Cited by 41 publications

References 25 publications

New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome

New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome

Idiopathic Nephrotic Syndrome in Childhood: A Retrospective Analysis of Two Hundred and Eighty Nine Patients

Pengobatan Terkini Sindrom Nefrotik (SN) pada Anak

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