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Título: | Renal involvement in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): report of a case with a six-year follow-up |
Fecha de publicación: | 2012 |
Editorial: | F. Hernández y Juan F. Madrid. Universidad de Murcia. Departamento de Biología Celular e Histología |
Cita bibliográfica: | Histology and histopathology, Vol. 27, nº 10 (2012) |
ISSN: | 0213-3911 1699-5848 |
Materias relacionadas: | CDU::5 - Ciencias puras y naturales::57 - Biología |
Palabras clave: | CADASIL Kidney |
Resumen: | Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a disorder of the cerebral small blood vessels caused by a mutation in the NOTCH3 gene, which encodes a large transmembrane receptor NOTCH3. It is associated with systemic arteriopathy involving small arteries, besides the brain, in skin, spleen, liver, muscle, aorta and in the kidney. The key pathological finding is the accumulation of granular osmiophilic material (GOM) on degenerating vascular smooth muscle cells. In the kidney GOMs have been described only in a very limited number of CADASIL patients. We describe a genetically confirmed CADASIL patient with mild renal dysfunction and GOMs in the interlobular and juxtaglomerular arteries and, for the first time, also within the glomerulus, whose nephrology conditions remained stable, whereas the neurological manifestations markedly worsened over a six-year follow-up period. The reasons for this discrepancy are probably related to differences in the structure and function of brain and kidney blood vessels. |
Autor/es principal/es: | Ragno, Michele Trojano, Luigi Pianese, Luigi Boni, Maria Virginia Silvestri, Serena Mambelli, Vladimiro Lorenzi, Teresa Scarpelli, Marina Morroni, Manrico |
URI: | http://hdl.handle.net/10201/54457 |
Tipo de documento: | info:eu-repo/semantics/article |
Número páginas / Extensión: | 8 |
Derechos: | info:eu-repo/semantics/openAccess |
Aparece en las colecciones: | Vol.27, nº10 (2012) |
Ficheros en este ítem:
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Ragno-27-1307-1314-2012.pdf | 22,68 MB | Adobe PDF | Visualizar/Abrir |
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