We describe the case of a 67 year-old female with nephrotic syndrome and rapidly progressive renal failure. The nephropathy was characterized by deposits of randomly oriented fibrils with a diameter of about 18-20 nm on electron microscopy. Immunofluorescence microscopy was performed and there was no staining for immunoglobulins and complement. We diagnosed atypical fibrillary glomerulopathy with absence of immune deposits. The patient developed end-stage renal failure rapidly. We review in the literature new clinical and pathogenetic features related to fibrillary and immunotactoid glomerulopathy.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.4321/s0212-71992005000100009 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Aquaporin-2 in the early stages of the adenine-induced chronic kidney disease model.
PLoS One
January 2025
Facultad de Farmacia y Bioquímica, Departamento de Ciencias Biológicas, Cátedra de Biología Celular y Molecular, Universidad de Buenos Aires, Buenos Aires, Argentina.
Chronic kidney disease (CKD) is one of the leading health problems in the world. It is silent in the early stages and gradually progresses, inducing renal physiological and structural alterations. Moreover, CKD is associated with impaired life quality, increased risk for cardiovascular diseases, and reduced life expectancy.
View Article and Find Full Text PDFBackground: Regionally anticoagulated continuous renal replacement therapy with citrate is the first choice for critically ill patients with acute kidney injury. If citrate that reaches the patient exceeds the metabolic capacity, metabolic alkalosis will follow. Bicarbonate from the treatment fluids will also reach the patient and add to the bicarbonate load.
View Article and Find Full Text PDFPlasma proteomics for risk prediction and identification of novel drug targets in systemic lupus erythematosus.
Rheumatology (Oxford)
January 2025
Division of Nephrology, Nanfang Hospital, Southern Medical University; National Clinical Research Center for Kidney Disease; State Key Laboratory of Organ Failure Research; Guangdong Provincial Institute of Nephrology, Guangdong Provincial Key Laboratory of Renal Failure Research, Guangdong Laboratory, Guangzhou, 510515, China.
Objectives: The relationship between proteomic profiles and incident systemic lupus erythematosus (SLE) remains unclear. We aimed to identify candidate plasma proteins for SLE risk in women, discover potential treatment targets for SLE, and develop and validate a protein-based prediction model for SLE risk.
Methods: 28 220 women from the UK Biobank were randomly split into training (70%) and testing (30%) sets.
The impact of a secondary, rare, non-pathogenic PKD1 variant on disease progression in autosomal dominant polycystic kidney disease.
J Nephrol
January 2025
Department of Nephrology, Beaumont Hospital, Dublin, Ireland.
Background: Autosomal dominant polycystic kidney disease (ADPKD) is caused primarily by pathogenic variants in the PKD1 and PKD2 genes. Although the type of ADPKD variant can influence disease severity, rare, hypomorphic PKD1 variants have also been reported to modify disease severity or cause biallelic ADPKD. This study examines whether rare, additional, potentially protein-altering, non-pathogenic PKD1 variants contribute to ADPKD phenotypic outcomes.
View Article and Find Full Text PDFAdenine base editor corrected ADPKD point mutations in hiPSCs and kidney organoids.
Adv Biotechnol (Singap)
June 2024
MOE Key Laboratory of Gene Function and Regulation, State Key Laboratory of Biocontrol, School of Life Sciences, Sun Yat-Sen University, Guangzhou, Guangdong, 510275, China.
Autosomal dominant polycystic kidney disease (ADPKD) is a dominant genetic disorder caused primarily by mutations in the PKD1 gene, resulting in the formation of numerous cysts and eventually kidney failure. However, there are currently no gene therapy studies aimed at correcting PKD1 gene mutations. In this study, we identified two mutation sites associated with ADPKD, c.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!