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Atypical Anti-GBM with ANCA Vasculitis- A Rarest of the Rare Entity: Index Case from Eastern India

Published on: 13th September, 2024

Anti-glomerular basement membrane (GBM) antibody glomerulonephritis is an extremely rare glomerular disease. Around 90% of the patients are positive for serum anti-GBM antibodies while up to 10% can be negative. In such patients, only a kidney biopsy can reveal the anti-GBM disease it is then labeled as an atypical anti-GBM disease. Though anti-GBM disease can be associated with Anti Neutrophil Cytoplasmic Antibodies (ANCA) positivity, it is extremely rare to find atypical anti-GBM with ANCA positivity so much so that till now there are very few such cases reported from across the world.The case presented here is one such case where the patient presented with adult-onset nephrotic syndrome features with active urinary sediments and mildly deranged renal function. Myeloperoxidase (MPO) ANCA was positive and it was considered ANCA-associated crescentic glomerulonephritis (GN) but after the renal biopsy the picture was of anti-GBM disease. She was treated with pulse methylprednisolone but her creatinine increased in the meantime and considering anti-GBM she was put on Plasma Exchange (PLEX). She received 5 sessions of PLEX after which her renal function improved. She was also planned for Rituximab which could not be given due to local infection. As there are no protocols for treating such cases because of the extremely rare nature of the presentation, this case will increase the understanding of such presentations for the clinicians. This will help to plan for building the approach for such cases.
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Anatomo-clinical and Etiological Profile of Nephropathies Biopsied in the Nephrology Department of the Thies Regional Hospital (Senegal)

Published on: 17th September, 2024

Introduction: In many developing countries, particularly in Africa, the use of renal biopsy (RB) in clinical nephrology is severely lacking. The objectives were to describe the anatomoclinical and etiological profile of these biopsied nephropathies, as well as the factors associated with the etiology of the nephropathies.Patients and method: This was a retrospective descriptive and analytical study from 1 April 2020 to 30 October 2022. The patients were selected from the renal biopsy register of the nephrology department of the Thiès Regional Hospital. Sociodemographic, clinical, biological, and histological parameters were studied.Results: 75 renal biopsies were included. The mean age was 33.3 ± 14.8 years, with a male predominance (65.3%). The main indications were nephrotic syndrome in 50.67% of cases. RB was adequate in 82.7%, inadequate in 13.3%, and borderline in 4%. Glomerular nephropathies predominated, with focal segmental glomerulosclerosis (FSGS) in 34.7%, membranous nephropathy (MN) in 17.3%, minimal change disease (MCD) in 10.67%, extracapillary glomerulonephritis (ECGN) in 5.3% and lupus nephritis (LN) in 3.9%. Thrombotic microangiopathy (TMA) was found in 9.3%. Chronic tubulointerstitial nephropathy (CTIN) accounted for 5.3% of all RB and acute tubular necrosis (ATN) for 4%. The etiologies were primary in 48%, secondary in 28%, and undetermined in 24%. In the bivariate analysis, the etiology was correlated with the mean SBP (p = 0.023), the mean level of hemoglobin (p = 0.028), the levels of GFR (p = 0.017), and the type of kidney disease (p = 0.000).Conclusion: Glomerular nephropathy was more frequent and FSGS was the most common histological lesion found. Primary causes predominated. Associated factors were identified to improve patient management.
Cite this ArticleCrossMarkPublonsHarvard Library HOLLISGrowKudosResearchGateBase SearchOAI PMHAcademic MicrosoftScilitSemantic ScholarUniversite de ParisUW LibrariesSJSU King LibrarySJSU King LibraryNUS LibraryMcGillDET KGL BIBLiOTEKJCU DiscoveryUniversidad De LimaWorldCatVU on WorldCat
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