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The top of pageAbstractH deficiency by 50 % brothe
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The top of pageAbstractH deficiency into two brothers with atypical dense intramembranous deposit disease. We benefit by an H deficiency by 50 % Algerian brothers who had early glomerulonephritis. Furthermore, one suffered with serious lung infections. H deficiency was defined by undetectable CH50 and AP50, and low quantities of H, C3 and B (less than 10% of ordinary levels). I and classical pathway components, including C4bp were normal. CR1 was present for patients' erythrocytes. No nephritic factor or any other circulating alternative pathway activator was detected. The parents, that [url=http://www.eurocontrol.es/uggs.asp]Botas Ugg Espana[/url] happen to be first cousins, as well as a healthy brother and sister had half amounts of H. These bits of information favor an autosomal [url=http://thalia1.dk/canadagoose/]Canada Goose Jackets[/url] recessive transmission on the H defect. Although by electron microscopy renal biopsies from both patients were typical for dense intramembranous deposit disease, immunofluorescence microscopy showed an atypical pattern with abundant granular C3 deposits inside mesangium and down the capillary walls. Alternative [url=http://www.jaunay-clan.fr/longchampascher.asp]Sac Longchamp[/url] pathway activators, possibly regarding dense deposits, may enable the formation of membrane C3/C5 convertases, unusually stable without H, since C5, C6, C7, C8 and C9 levels were decreased inside patients. This observation may represent an interesting clue on the relationship between nephritic factor, alternative pathway activation, and dense intramembranous deposit disease.Haiti prez to improve term if election not held
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