4 results on '"Liapis H"'
Search Results
2. Electron microscopy in kidney research: seeing is believing.
- Author
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Liapis H
- Subjects
- Animals, Autoantigens genetics, Collagen Type IV deficiency, Collagen Type IV genetics, Disease Models, Animal, Freeze Fracturing, Genetic Predisposition to Disease, Humans, Hyperoxaluria genetics, Hyperoxaluria metabolism, Hyperoxaluria pathology, Kidney metabolism, Kidney Diseases genetics, Kidney Diseases metabolism, Mice, Mice, Knockout, Nephritis, Hereditary genetics, Nephritis, Hereditary metabolism, Nephritis, Hereditary pathology, Phenotype, Biomedical Research methods, Kidney ultrastructure, Kidney Diseases pathology, Microscopy, Electron, Nephrology
- Abstract
Electron microscopy (EM) has been an indispensable tool for kidney research since its inception more than half a century ago. Much of the substantial advances were propelled by the need to find methods to best visualize and analyze the kidney's structure deduced from the fundamental principle that has structure and function intimately related. The result of 3 decades of experimental kidney work between 1950 and 1980 coincided with remarkable advances in nephrology that marked a renaissance era for renal pathology and resulted in the morphologic classification of medical kidney diseases. In the era of genetics and molecular medicine TEM continues to contribute significant clinical and pathogenetic insights in kidney disease. The basic principles as applied to kidney disease experimental models are discussed with emphasis on crescent formation in Col4A3-deficient mice and a mouse model of experimental oxalosis (CaOx).
- Published
- 2013
- Full Text
- View/download PDF
3. Morphologic heterogeneity of renal light-chain deposition disease.
- Author
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Gokden N, Barlogie B, and Liapis H
- Subjects
- Basement Membrane ultrastructure, Female, Fluorescent Antibody Technique, Direct, Glomerular Mesangium ultrastructure, Humans, Immune Complex Diseases immunology, Kidney Diseases immunology, Kidney Glomerulus immunology, Male, Microscopy, Electron, Transmission, Middle Aged, Multiple Myeloma immunology, Prospective Studies, Retrospective Studies, Immune Complex Diseases pathology, Immunoglobulin Light Chains, Kidney Diseases pathology, Kidney Glomerulus ultrastructure, Multiple Myeloma pathology
- Abstract
Light-chain deposition disease (LCDD) of the kidney is defined as deposition of monotypic light chains (LC) within glomerular (GBM) and tubular (TBM) basement membranes. The morphologic features of pure renal LCDD have been presented only in case reports or small series. The aim of this study was to perform a comprehensive evaluation of the light (LM), immunofluorescence (IF), and electron microscopic (EM) features of pure renal LCDD in a large series of biopsies. Out of 46 cases assembled, 42 had multiple myeloma, 2 had monoclonal gammopathy of unknown significance, and in 2 patients no lymphoproliferative disease was identified. The most common LM lesion of LCDD, nodular glomerulosclerosis, was present in only 14 (30%) cases. GBM and/or TBM thickening was found in 3 (6%), mild to moderate mesangial matrix increase in 12 (23%), and unremarkable glomeruli and tubules were seen in 15 (32%) cases. Forty-two had IF and 40 (92%) showed characteristic linear LC immunoreactivity (24 kappa, 16 lambda) along GBM and/or TBM. Among 39 cases in which IF and EM was available, 25 (64%) were positive by both. Two (6%) were negative by IF, but had deposits by EM. In 12 (30%) with immunoreactivity to LC (4 kappa, 8 lambda), no deposits were identified ultrastructurally. This study shows heterogeneous LM lesions in pure LCDD cases. LM alone may be suspicious but not diagnostic of LCDD. Immunofluorescence is more sensitive than EM for detection of LC for the definitive diagnosis of LCDD. This study supports the importance of utilizing kappa and lambda stains in the routine IF panel for diagnosis of LCDD.
- Published
- 2008
- Full Text
- View/download PDF
4. Morphologic manifestations of combined light-chain deposition disease and light-chain cast nephropathy.
- Author
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Gokden N, Cetin N, Colakoglu N, Kumar J, Abul-Ezz S, Barlogie B, Liapis H, and Walker PD
- Subjects
- Adult, Aged, Female, Glomerular Basement Membrane immunology, Glomerular Basement Membrane metabolism, Glomerular Basement Membrane ultrastructure, Humans, Immune Complex Diseases immunology, Immune Complex Diseases metabolism, Kidney Diseases immunology, Kidney Diseases metabolism, Kidney Tubules immunology, Kidney Tubules metabolism, Male, Microscopy, Electron, Transmission, Microscopy, Fluorescence, Middle Aged, Retrospective Studies, Immune Complex Diseases pathology, Immunoglobulin Light Chains, Kidney Diseases pathology, Kidney Tubules ultrastructure
- Abstract
There are few data on morphology of light-chain deposition disease (LCDD) of kidney with coexistent light-chain cast nephropathy (LCCN). Here, the authors report the morphology in 23 cases of LCDD and LCCN. They retrospectively evaluated 23 renal biopsies with light (LM), immunofluorescence (IF), and electron microscopy (EM). Twenty-one patients had myeloma, 1 had a monoclonal gammopathy, and in 1 no illness was found. Nodular glomerulosclerosis, the LM lesion suggestive of LCDD, was noted in only 3 of 23 cases. Glomeruli were unremarkable in 16 (69%) cases. The diagnostic casts of LCCN were seen in all biopsies. Linear light chain (LC) immunoreactivity was observed in 23 (100%) cases (18 kappa, 5 lambda); GBM + TBM in 13, TBM only in 7, GBM only in 1, TBM and interstitium in 1, GBM, TBM and mesangium in 1. Casts were positive with same LC in all cases (100%). Fifteen cases (65%) showed granular electron-dense deposits; GBM only in 5, TBM only in 5, GBM and TBM in 4, mesangium in 1. In 8 patients without EM deposits, the diagnosis of LCDD was rendered by IF. Fifteen (65%) had deposits detectable by IF and EM, 8 (37%) had deposits with IF only. LCCN dominated the LM findings in all patients. There were minimal or no glomerular changes by LM. This study shows the lack of characteristic LM findings of LCDD in combined cases of LCDD and LCCN and emphasizes the difficulty for-definitive diagnosis-without IF and EM.
- Published
- 2007
- Full Text
- View/download PDF
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