Source:http://linkedlifedata.com/resource/pubmed/id/20971496
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
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pubmed:dateCreated |
2010-12-14
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pubmed:abstractText |
The objective of the study was to investigate the role of endothelin-1 in the pathogenesis of scleroderma renal crisis in patients with systemic sclerosis. We used immunohistochemical analysis with anti-endothelin-1 and anti-von Willebrand factor antibodies in comparing kidney biopsies from patients with systemic sclerosis and scleroderma renal crisis (n = 14); from normal kidneys (n = 5); and from patients with typical hemolytic uremic syndrome and thrombotic microangiopathy (n = 5), antiphospholipid syndrome (n = 6), diabetic nephropathy (n = 5), minimal change disease with cyclosporine toxicity (n = 5), or nephroangiosclerosis (n = 5). Kidney biopsies from all systemic sclerosis patients presented specific lesions: glomerular lesions with thickened capillary walls (n = 6, 42.8%), mesangiolysis (n = 3, 21.4%), fibrin thrombi (n = 3, 21.4%), hypertrophy of juxtaglomerular apparatus (n = 5, 35.7%), arteriolar lesions showing mucinous intimal thickening and lumen mucoid occlusions (n = 13, 92.8%), proliferation of intimal cells (ie, "onion-skin" lesions; n = 13, 92.8%), fibrinoid necrosis (n = 3, 21.4%), and fibrin thrombosis (n = 4, 28.6%). Chronic lesions in large arteries showed modifications such as fibrous intimal thickening (n = 13, 92.8%). The pattern of endothelial staining for endothelin-1 in both glomeruli and arteriolar lesions appears to be specific for scleroderma renal crisis. Glomerular endothelin-1 staining without arteriolar staining was seen in hemolytic uremic syndrome; and isolated arteriolar staining (without glomerular staining) was seen in a number of conditions including antiphospholipid nephropathy, cyclosporine toxicity, and diabetic nephropathy. Endothelin-1 is overexpressed in glomeruli and arterioles of patients with scleroderma renal crisis, which suggests that endothelin-1 might be a therapeutic target in this condition.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Jan
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pubmed:issn |
1532-8392
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pubmed:author | |
pubmed:copyrightInfo |
Copyright © 2011 Elsevier Inc. All rights reserved.
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pubmed:issnType |
Electronic
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pubmed:volume |
42
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
95-102
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pubmed:meshHeading |
pubmed-meshheading:20971496-Acute Kidney Injury,
pubmed-meshheading:20971496-Adult,
pubmed-meshheading:20971496-Aged,
pubmed-meshheading:20971496-Antiphospholipid Syndrome,
pubmed-meshheading:20971496-Endothelin-1,
pubmed-meshheading:20971496-Female,
pubmed-meshheading:20971496-Hemolytic-Uremic Syndrome,
pubmed-meshheading:20971496-Humans,
pubmed-meshheading:20971496-Kidney,
pubmed-meshheading:20971496-Male,
pubmed-meshheading:20971496-Middle Aged,
pubmed-meshheading:20971496-Scleroderma, Systemic,
pubmed-meshheading:20971496-von Willebrand Factor
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pubmed:year |
2011
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pubmed:articleTitle |
Endothelin-1 expression in scleroderma renal crisis.
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pubmed:affiliation |
Université Paris Descartes, faculté de Médecine Paris Descartes, pôle de Médecine Interne, hôpital Cochin, Centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, Assistance Publique-Hôpitaux de Paris (AP-HP), 75014 Paris, France. luc.mouthon@cch.aphp.fr
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pubmed:publicationType |
Journal Article
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