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Evaluation of the children with C3 glomerulopathy.

Treatment Used: Complement-Targeting Therapy with Cculizumab
Number of Patients: 69
MediFind Summary

Overview: This study described the response to immunosuppressive and supportive treatment in children with C3 glomerulopathy (kidney disease; C3G) and their prognosis.

Conclusion: Treatment for children with C3 glomerulopathy (kidney disease) may vary, and the authors recommend adding eculizumab to targeting therapy for early stage refractory cases that do not response to immunosuppression.

Abstract

C3 glomerulopathy (C3G) is a clinical spectrum that presents with a variety of symptoms, ranging from a mild disease with asymptomatic microhematuria and/or proteinuria to severe disease with nephritic or nephrotic syndrome and renal impairment. Herein, we aim to document the clinical and laboratory findings, response to immunosuppressive and supportive treatment and prognosis of the children with C3G. We retrospectively reviewed the medical records of patients diagnosed with membranoproliferative glomerulonephritis (MPGN). Kidney biopsy materials were reexamined for the diagnosis of C3G. The inclusion criteria for C3G are the dominant C3 staining with or without scanty immunoglobulins (Ig) deposition on immuno- fluorescence (IF) and MPGN patterns on light microscope. Twelve of 69 patients with MPGN were included in the study based on the definition criteria of C3G. Ten of them had only C3 staining and the rest of the patients had both C3 staining and a small amount of IgG/M staining on IF microscopy. One patient was on remission with only ACEI. The rest of the patients used immunosuppressive treatment and two of them needed eculizumab therapy. One of them did not respond to the treatment of eculizumab and progressed to end-stage renal failure. C3G is a disease characterized by a heterogeneous clinical presentation and outcome. Because of this broad spectrum of disease, treatment may vary widely. We think that complement-targeting therapy with eculizumab should be an alternative option for refractory cases, especially in the early stage of disease, if they did not respond to immunosuppressive treatment.

Authors
Ayse Pinarbasi, Ismail Dursun, Muammer Poyrazoglu, Hulya Akgun, Adil Bozpolat, Ruhan Dusunsel

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