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Artikel ; Online: Remission of proteinuria in multidrug-resistant idiopathic nephrotic syndrome following immunoglobulin immunoadsorption.

Nattes, Elodie / Karaa, Danièle / Dehoux, Laurène / Peuchmaur, Michel / Kwon, Theresa / Deschênes, Georges

Acta paediatrica (Oslo, Norway : 1992)

2018  Band 108, Heft 4, Seite(n) 757–762

Abstract: Aim: Complete or partial resistance to prednisone and calcineurin inhibitors in children with idiopathic nephrotic syndrome often leads to end-stage renal disease. The aim of the study was to report the outcome of patients with multidrug-resistant ... ...

Abstract Aim: Complete or partial resistance to prednisone and calcineurin inhibitors in children with idiopathic nephrotic syndrome often leads to end-stage renal disease. The aim of the study was to report the outcome of patients with multidrug-resistant nephrotic syndrome treated with an association of immunoglobulin immunoadsorption, intravenous immunoglobulins and B-cell depletion.
Methods: At treatment initiation, patients received ten sessions of immunoglobulin immunoadsorption and intravenous immunoglobulins in two weeks followed by one rituximab in case of remission.
Results: A remission of proteinuria was obtained in nine out of 14 patients at the end of the initial phase and in two additional patients after an extended period. The remission was stable in three patients and after additional IgIA and heavy immunosuppression in six. Two patients that initially responded relapsed after IgIA withdrawal and remained with an uncontrolled disease at last follow-up. Three patients did not respond to the treatment including two who were found to have a genetic podocytopathy.
Conclusion: Patients with multidrug-resistant idiopathic nephrotic syndrome can be successfully led into remission by IgIA prior to reaching end-stage renal disease. However, IgIA does not suppress the need for heavy additional immunosuppression to control INS in most cases.
Mesh-Begriff(e) Adolescent ; Child ; Child, Preschool ; Drug Resistance, Multiple ; Female ; Humans ; Immunoglobulins ; Infant ; Male ; Nephrotic Syndrome/drug therapy ; Nephrotic Syndrome/therapy ; Plasmapheresis ; Proteinuria/etiology ; Proteinuria/therapy ; Remission Induction ; Retrospective Studies
Chemische Substanzen Immunoglobulins
Sprache Englisch
Erscheinungsdatum 2018-10-15
Erscheinungsland Norway
Dokumenttyp Journal Article
ZDB-ID 203487-6
ISSN 1651-2227 ; 0365-1436 ; 0803-5253
ISSN (online) 1651-2227
ISSN 0365-1436 ; 0803-5253
DOI 10.1111/apa.14582
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