Results 41 to 50 of about 4,208 (171)

Effectiveness of Cyclosporine in a 10-year-old Girl with C3 Glomerulopathy [PDF]

Childhood Kidney Diseases, 2017
C3 glomerulopathy (C3G) is a recently defined pathological entity characterized by C3 accumulation with absent or scant immunoglobulin deposition, leading to variable glomerular inflammation.
Kyung Mi Jang, Yong Hoon Park
doaj   +1 more source

Factor B Inhibition with Iptacopan in Recurrent C3 Glomerulopathy Following Kidney Transplant: A Report of Two Cases

Kidney Medicine
C3 glomerulopathy is a rare disease caused by fluid phase dysregulation of the alternative complement pathway. Currently, treatment depends on clinical and histological severity and includes nephroprotection, unspecific immunosuppression, and terminal ...
Víctor J. Escudero-Saiz, Ángela Gonzalez, Adriana García-Herrera, Ana B. Larque, Andrew S. Bomback, Laura Morantes, Marta Martínez-Chillarón, Júlia Ollé, Elena Guillén, Marc Xipell, Alicia Molina-Andújar, Diana Rodríguez, Elena Cuadrado, Judit Cacho, Carolt Arana, Núria Esforzado, Carla Bastida, Esteban Poch, Fritz Diekman, David Cucchiari, Luis F. Quintana, Miquel Blasco   +21 more
doaj   +1 more source

Lipodistrofia parcial adquirida y glomerulopatía C3: la desregulación del sistema del complemento como mecanismo común

Nefrología, 2018
Resumen: La activación de la vía alternativa del complemento interviene en el desarrollo de varias enfermedades renales, como el síndrome hemolítico urémico atípico o la glomerulopatía C3.
Fernando Corvillo, Margarita López-Trascasa   +1 more
doaj   +1 more source

Pediatric Non‐Lupus Full House Nephropathy: Case Report and Review of Literature

Clinical Case Reports, Volume 14, Issue 3, March 2026.
ABSTRACT Lupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) typically characterized by glomerular “full‐house” immunofluorescence. However, non‐lupus nephropathies may occasionally exhibit similar patterns, creating diagnostic uncertainty.
Mohammad Firoz Anjum, Suraj Sah, Inesh Khanal, Sharmila Gainju, Sri Ram K.C.   +4 more
wiley   +1 more source

Treatment options for C3 glomerulopathy [PDF]

Current Opinion in Nephrology and Hypertension, 2013
The purpose of this review is to discuss emerging nomenclature, review the salient clinicopathological features and describe the therapeutic options available for the treatment of C3 glomerulopathy (C3G).C3G is minimally responsive to traditional immune suppression and randomized controlled trials to support therapy are absent.
Carla M, Nester, Richard J, Smith
openaire   +2 more sources

Evaluation of the children with C3 glomerulopathy

Saudi Journal of Kidney Diseases and Transplantation, 2020
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.
Ayse Seda Pinarbasi, Ismail Dursun, Muammer Hakan Poyrazoglu, Hulya Akgun, Adil Bozpolat, Ruhan Dusunsel   +5 more
doaj   +1 more source

Iptacopan for Immune Thrombocytopenia and Cold Agglutinin Disease: A Global Phase 2 Basket Clinical Trial

American Journal of Hematology, Volume 101, Issue 2, Page 242-254, February 2026.
ABSTRACT Iptacopan is a first‐in‐class, oral, selective inhibitor of complement factor B that has demonstrated positive efficacy across several complement‐driven diseases. Here we evaluate the efficacy and safety of iptacopan monotherapy in adult patients with primary immune thrombocytopenia (ITP) and primary cold agglutinin disease (CAD). We performed
Alexander Röth, Wilma Barcellini, Christine Ademokun, Junho Jang, Maria Luisa Lozano, David Valcarcel Ferreiras, Cristina Pascual‐Izquierdo, Shripad Chitnis, Sofiya Matviykiv, Alessandra Vitaliti, Chi Chen, Vasiliki Katsanou, Raghav Chawla, Hanny Al‐Samkari   +13 more
wiley   +1 more source

Meningococcal Serogroup Y Meningitis Reveals Inborn Factor B Deficiency

European Journal of Immunology, Volume 56, Issue 2, February 2026.
We report a novel case of complete complement Factor B deficiency revealed by invasive meningococcal disease. Using combined functional and genetic analyses, we demonstrate that loss of Factor B abolishes alternative pathway activity despite detectable protein levels. These findings provide a mechanistic framework relevant to complement diagnostics and
Camille Bougeard, Eléonore Eskander, Paula Vieira Martins, Joan Bitan, Julia Roquigny, Christèle Kyheng, Tamazoust Guiddir, Véronique Frémeaux‐Bacchi, Cécile Gonnin, Carine ElSissy   +9 more
wiley   +1 more source

Complement Inhibitors in Clinical Trials for Glomerular Diseases

Frontiers in Immunology, 2019
Defective complement action is a cause of several human glomerular diseases including atypical hemolytic uremic syndrome (aHUS), anti-neutrophil cytoplasmic antibody mediated vasculitis (ANCA), C3 glomerulopathy, IgA nephropathy, immune complex ...
Peter F. Zipfel, Peter F. Zipfel, Thorsten Wiech, Ramona Rudnick, Sara Afonso, Fermin Person, Christine Skerka   +6 more
doaj   +1 more source

The Complement System in ANCA‐Associated Vasculitis: Mechanistic Insights, Therapeutic Horizons, and Unmet Clinical Needs

Immunity, Inflammation and Disease, Volume 14, Issue 2, February 2026.
Complement activation, particularly via the alternative pathway, drives inflammation and organ damage in ANCA‐associated vasculitis. This review highlights mechanistic insights, tissue and biomarker evidence, and clinical implications of targeting the C5a–C5aR1 axis.
Kehinde Sunmboye, Pauline Millan
wiley   +1 more source