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Association of monoclonal gammopathy of undetermined significance and C3 glomerulopathy.
Intern Med J
September 2023
Department of Renal Medicine, Woodlands Health, Singapore, Singapore.
Monoclonal gammopathy of undetermined significance (MGUS) is usually an asymptomatic pre-malignant condition caused by the proliferation of clonal plasma cells. Often considered a benign condition, it has the potential to progress to malignant plasma cell or lymphoproliferative disorders. Moreover, MGUS can rarely cause glomerular disease by activating the alternative complement pathway resulting in immunoglobulin-negative C3-positive glomerulonephritis called C3 glomerulopathy.
View Article and Find Full Text PDFA 78-Year-Old Man with Chronic Kidney Disease and Monoclonal Gammopathy Who Developed Post-Transplant C3 Glomerulopathy - Recurrence or De Novo? A Case Report and Literature Review.
Am J Case Rep
June 2023
Department of Nephrology, Virgen de la Nieves University Hospital, Granada, Spain.
BACKGROUND The incidence of glomerular disease recurrence in kidney transplant patients varies according to type of glomerulopathy; therefore, it is important to know the primary chronic kidney disease etiology. C3 glomerulopathy (C3G) is characterized by deposits of C3 in immunofluorescence and its pathogeny is based on the dysregulation of the alternative complement pathway. C3G has a high recurrence rate and, given its low prevalence, only case series have been published.
View Article and Find Full Text PDFAlternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial.
Kidney Int Rep
October 2022
Molecular Otolaryngology and Renal Research Laboratories, University of Iowa, Iowa City, Iowa, USA.
Article Synopsis
- Complement 3 glomerulopathy (C3G) is a rare kidney disease linked to issues with the alternative pathway of the complement system, often leading to kidney failure within a decade of diagnosis, and there are currently no approved treatments.
- Iptacopan is a new oral medication that inhibits factor B in the complement system and showed promise in reducing proteinuria in initial studies.
- The ongoing Phase III APPEAR-C3G study is evaluating the safety and effectiveness of iptacopan by comparing it to a placebo in 68 adults over a 12-month period, with a focus on reducing protein levels in urine and improving kidney function.
Rare Case of C3 Glomerulopathy in a Patient of Type 1 Diabetes Mellitus.
Saudi J Kidney Dis Transpl
February 2022
Department of Nephrology, Mahatma Gandhi Hospital, Jaipur, Rajasthan, India.
Complement component 3 glomerulopathy (C3G) is a recently defined entity comprising dense deposit disease and C3 glomerulonephritis. It is associated with nonrenal diseases such as diabetes mellitus (DM) type 1, ocular drusen, acquired partial lipodystrophy, and monoclonal gammopathy of undetermined significance. We describe a case of 13-year-old boy of the known case of type 1 DM, who developed proteinuria, and swelling over his face, and lower limbs, which on renal biopsy, was diagnosed as a case of C3G.
View Article and Find Full Text PDFC3 glomerulopathy associated with monoclonal gammopathy: impact of chronic histologic lesions and beneficial effects of clone-targeted therapies.
Nephrol Dial Transplant
October 2022
Instituto de Investigación Hospital 12 de Octubre, Madrid, Spain.
Article Synopsis
- C3 glomerulopathy associated with monoclonal gammopathy (C3G-MIg) is a rare kidney condition, and this study examined the clinical and histological characteristics of patients diagnosed with it between 1995 and 2021.
- The study involved 23 patients, with a median age of 63 years, and found that a significant number reached kidney failure (39%) during an average follow-up of 40 months, with transplant recipients faring particularly poorly.
- The researchers concluded that the C3G histologic index is useful for predicting kidney prognosis, noting that clone-targeted therapies improved survival rates in patients who responded to treatment.
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