Valérie Guérin

Assistance Publique – Hôpitaux de Paris, Lutetia Parisorum, Île-de-France, France

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Publications (3)9.25 Total impact

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    ABSTRACT: BACKGROUND: We report a 7-year-old boy with high-degree steroid-dependent idiopathic nephrotic syndrome (SDNS) who went into remission with rituximab (RTX) maintenance therapy. CASE-DIAGNOSIS/TREATMENT: Four months after this patient received his first RTX infusion, there was a progressive and sustained decrease of immunoglobulin (Ig)G and IgM levels. Thirteen months after the initiation of RTX therapy he was in sustained remission without any steroid or oral immunosuppressive therapy; however, B cell depletion was still present. At this time he developed a fulminant myocarditis due to enterovirus. Despite aggressive treatment and the administration of intravenous polyvalent immunoglobulins there was no clinical improvement. He successfully underwent heart transplant surgery. CONCLUSIONS: We conclude that B cell depletion with RTX is efficacious in the treatment of paediatric SDNS but that it may be associated with severe infectious complications. Therefore, we recommend a close monitoring of Ig levels in children who have received RTX therapy and a supplementation with intravenous Ig as soon as the Ig levels fall below the lower limit of the normal range.
    Pediatric Nephrology 05/2013; · 2.94 Impact Factor
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    ABSTRACT: Rituximab (RTX) is a new treatment strategy in high-degree steroid-dependent idiopathic nephrotic syndrome (SDNS) in childhood. Thirty patients (nine girls) with SDNS with steroid side effects and previously treated with immunosuppressive drugs, mostly calcineurin inhibitors, were treated with RTX and included in this non-controlled single-centre study. Patient age at first RTX infusion was 12.9 ± 0.7 years. Our aim was to evaluate disease outcome after a minimum CD19 depletion period of 15 months obtained by repeated RTX infusion. Minimum follow-up after initial CD19 depletion was 24 months. During the RTX treatment period, seven patients had nephrotic syndrome relapses, six among them at the time of an intermittent CD19 recovery and one patient relapsed under CD19 depletion. The risk for these patients to relapse after the RTX treatment period was higher than in those without intermittent relapses. After definitive CD19 recovery over a follow-up of 17.4 ± 1.9 months, 19 patients (63%) did not relapse and 11 (37%) relapsed 4.3 ± 1 months after defininitive CD19 recovery. Among these 11 patients, 6 already had intermittent relapses during the RTX treatment period. Steroid and immunosuppressive treatment could be discontinued in all patients during CD19 depletion and was re-introduced in two after CD19 recovery. Fourteen patients had mostly benign and transitory side effects, which did not require RTX discontinuation. In conclusion, RTX treatment with a 15-month CD19 depletion period induced long-term remission after definitive CD 19 recovery in almost two-thirds the of patients without oral immunosuppressive drugs.
    Nephrology Dialysis Transplantation 08/2011; 27(3):1083-9. · 3.37 Impact Factor
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    ABSTRACT: Although most patients with idiopathic nephrotic syndrome (NS) respond to steroid treatment, development of steroid dependency may require a long-term multidrug therapy including steroid and calcineurin inhibitor. Rituximab was shown to allow a reduction of the doses of steroid and immunosuppressive drugs in those patients. In the present series, 22 patients with steroid-sensitive, but steroid-dependent nephrotic syndrome were treated with rituximab. Rituximab reduced B cell count down to an undetectable level in all patients. A second treatment was necessary in 18 patients in order to maintain B cell depletion for up to 18 months. B cell depletion lasted 4.9 to 26 months (mean 17.2 months). At last follow-up, 9 patients were in remission without oral steroid or calcineurin inhibitor, although B cell count had recovered for 2.9 to 17 months (mean 9.5 months). A remission under ongoing B cell depletion was observed in 10 other patients in the absence of oral steroid or calcineurin inhibitor. Rituximab failed in 2 patients and 1 refused any additional treatment, despite B cell recovery and relapse. Toxicity of rituximab was limited to reversible cytokine shock in 2 patients and reversible neutropenia in 1 patient. No severe infection was observed.
    Pediatric Nephrology 03/2010; 25(6):1109-15. · 2.94 Impact Factor