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Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion
Hereditary nephritis (HN) and thin glomerular basement membrane (GBM) lesion share a common clinical presentation of persistent hematuria, thin GBM by kidney biopsy electron microscopic examination, and a mutation in type IV collagen. However, the clinical course and treatment for these entities are...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9677744/ https://www.ncbi.nlm.nih.gov/pubmed/36751492 http://dx.doi.org/10.1159/000516744 |
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author | Lusco, Mark A. Fogo, Agnes B. |
author_facet | Lusco, Mark A. Fogo, Agnes B. |
author_sort | Lusco, Mark A. |
collection | PubMed |
description | Hereditary nephritis (HN) and thin glomerular basement membrane (GBM) lesion share a common clinical presentation of persistent hematuria, thin GBM by kidney biopsy electron microscopic examination, and a mutation in type IV collagen. However, the clinical course and treatment for these entities are different with varying patterns of heredity. Ultrastructural examination of a renal biopsy specimen is essential for the morphologic diagnosis of HN and thin GBM lesion, whereas light microscopy may only give limited diagnostic clues. Additional workup including immunostaining for subtypes of type IV collagen may provide further information on underlying genetic mutations. The diagnosis of HN may lead to treatment with renin-angiotensin system blockade in patients at risk of early-onset renal failure to delay progression to end-stage renal disease. Additionally, patients with isolated microscopic hematuria and thin GBM lesion are at increased risk for chronic kidney disease when associated with other comorbidities; those patients should receive regular clinical assessment to prevent renal function decline. |
format | Online Article Text |
id | pubmed-9677744 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
spelling | pubmed-96777442023-02-06 Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion Lusco, Mark A. Fogo, Agnes B. Glomerular Dis Review Article Hereditary nephritis (HN) and thin glomerular basement membrane (GBM) lesion share a common clinical presentation of persistent hematuria, thin GBM by kidney biopsy electron microscopic examination, and a mutation in type IV collagen. However, the clinical course and treatment for these entities are different with varying patterns of heredity. Ultrastructural examination of a renal biopsy specimen is essential for the morphologic diagnosis of HN and thin GBM lesion, whereas light microscopy may only give limited diagnostic clues. Additional workup including immunostaining for subtypes of type IV collagen may provide further information on underlying genetic mutations. The diagnosis of HN may lead to treatment with renin-angiotensin system blockade in patients at risk of early-onset renal failure to delay progression to end-stage renal disease. Additionally, patients with isolated microscopic hematuria and thin GBM lesion are at increased risk for chronic kidney disease when associated with other comorbidities; those patients should receive regular clinical assessment to prevent renal function decline. S. Karger AG 2021-06-15 /pmc/articles/PMC9677744/ /pubmed/36751492 http://dx.doi.org/10.1159/000516744 Text en Copyright © 2021 by The Author(s) Published by S. Karger AG, Basel https://creativecommons.org/licenses/by-nc/4.0/This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. |
spellingShingle | Review Article Lusco, Mark A. Fogo, Agnes B. Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion |
title | Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion |
title_full | Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion |
title_fullStr | Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion |
title_full_unstemmed | Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion |
title_short | Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion |
title_sort | hereditary nephritis and thin glomerular basement membrane lesion |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9677744/ https://www.ncbi.nlm.nih.gov/pubmed/36751492 http://dx.doi.org/10.1159/000516744 |
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