ArticleVirchows Archiv : an international journal of pathology2008
Kidney biopsy findings in heterozygous Fabry disease females with early nephropathy.
Article in Virchows Archiv : an international journal of pathology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 24 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 46 citations in OpenAlex.
- Fabry disease cardiomyopathy: A state-of-the-art review.Progress in cardiovascular diseasesPooled it
- Evaluation of corneal morphology, pupillometry, and ocular surface parameters in Fabry disease patients: correlation with plasma Lyso-Gb3 levels.International ophthalmology · 2026Article
- Zebra bodies recognition by artificial intelligence (ZEBRA): a computational tool for Fabry nephropathy.Scientific reports · 2026Article
- The Role of Kidney Biopsy in Fabry Disease.Biomedicines · 2025Review
- Females with Fabry disease: an expert opinion on diagnosis, clinical management, current challenges and unmet needs.Frontiers in cardiovascular medicine · 2025Review
- Molecular Pathogenesis of Central and Peripheral Nervous System Complications in Anderson-Fabry Disease.International journal of molecular sciences · 2023Review
- A novel unbiased method reveals progressive podocyte globotriaosylceramide accumulation and loss with age in females with Fabry disease.Kidney international · 2022Article
- The Impact of Kidney Biopsy for Fabry Nephropathy Evaluation on Patients' Management and Long-Term Outcomes: Experience of a Single Center.Biomedicines · 2022Article
- Pathology and pathogenic pathways in fabry nephropathy.Clinical and experimental nephrology · 2021Review
- Gastrointestinal Involvement in Anderson-Fabry Disease: A Narrative Review.International journal of environmental research and public health · 2021Review
- Review
- Variables Associated with a Urinary MicroRNAs Excretion Profile Indicative of Renal Fibrosis in Fabry Disease Patients.International journal of chronic diseases · 2019Article
- Understanding the gastrointestinal manifestations of Fabry disease: promoting prompt diagnosis.Therapeutic advances in gastroenterology · 2016Review
- Epithelial-Mesenchymal Transition in Kidney Tubular Epithelial Cells Induced by Globotriaosylsphingosine and Globotriaosylceramide.PloS one · 2015Article
- Lysosome dysfunction in the pathogenesis of kidney diseases.Pediatric nephrology (Berlin, Germany) · 2014Review
- Fabry nephropathy: a review - how can we optimize the management of Fabry nephropathy?BMC nephrology · 2014Review
- Mosaicism of podocyte involvement is related to podocyte injury in females with Fabry disease.PloS one · 2014Article
- Fibrosis: a key feature of Fabry disease with potential therapeutic implications.Orphanet journal of rare diseases · 2013Review
- Renal outcomes of agalsidase beta treatment for Fabry disease: role of proteinuria and timing of treatment initiation.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2012Article
- Immunohistochemical diagnosis of Fabry nephropathy and localisation of globotriaosylceramide deposits in paraffin-embedded kidney tissue sections.Virchows Archiv : an international journal of pathology · 2012Article
Corrections and comments
- Erratum issued
Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Fabry disease is an X-linked glycosphingolipidosis caused by deficiency of alpha-galactosidase. Progressive chronic kidney disease (CKD) is a major cause of morbidity and mortality in males. Although 40% of heterozygous females may develop renal involvement, pathologic data on Fabry nephropathy in heterozygotes are scarce. We reviewed the kidney biopsies of four affected females who had normal to slightly sub-normal renal function, two of them with overt proteinuria. Chronic non-specific degenerative lesions and glycosphingolipid accumulation per cell type were semi-quantitatively assessed by light and electron microscopy. Cellular distribution of glycosphingolipid deposits was best assessed on semithin sections. Podocyte effacement was seen only in proteinuric patients. Combined analysis of our data with those of two earlier series showed that glomerular sclerosis and tubulointerstitial fibrosis are predictors of proteinuria and CKD stage. There was no histopathological evidence supporting a major role of vascular damage in the early pathogenesis of Fabry nephropathy in females.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.