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10.1186/s12882-018-0880-y http://scihub22266oqcxt.onion/10.1186/s12882-018-0880-y C5875003!5875003!29592796     free     free     free       BMC+Nephrol 2018 ; 19 (ä): ä Nephropedia Template TP {{tp|p=29592796|t=2018. C3 glomerulopathy in cystic fibrosis: a case report |pdf=|usr=}}{{29592796}} gab.com Text C3 glomerulopathy in cystic fibrosis: a case report JO: BMC Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=29592796 #FOAvip Background: C3 glomerulonephritis is a rare glomerulopathy characterized at renal biopsy by C3 deposition, alone or with scanty immunoglobulins, as well as by an electron-dense material in mesangium, subendothelial and subepithelial space. An abnormal systemic activation of the alternative pathway of the complement cascade is responsible for the development of the disease if triggered by several possible environmental conditions. We report the first case in literature of a patient affected by cystic fibrosis and C3GN. Case presentation: Our case involves a young woman with cystic fibrosis, who had persistent microscopic hematuria, proteinuria and hypocomplementemia C3 for over three months. Renal biopsy confirmed the diagnosis of C3 glomerulopathy. Complement system dysregulation was tested and resulted in a strong terminal pathway activation proved by high levels of sC5b-9 complex, amounting to 1588 ng/ml (normal value <?400 ng/ml). Next generation sequencing (NGS) showed polymorphism in CFH (p.V62I in SCR1) and THBD (p.A473V), already known as pathogenic for C3GN, as well as a mutation in C3 (p.R102G) associated only with age-related macular degeneration (AMD) so far. Treatment was based on ACE inhibitors and kidney function is currently stable (GFR 50 ml/min, serum creatinine 1.7). Conclusions: The co-existence of C3 glomerulopathy in a patient with CF, which is characterized by chronic infection/inflammation, makes this case an interesting model of chronic altered systemic activation of the alternative pathway of the complement cascade. Twit Text FOAVip C3 glomerulopathy in cystic fibrosis: a case report ????BMC Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=29592796 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=29592796 #FOAvip English Wikipedia <ref>{{Cite pmid|29592796}}</ref> C3 glomerulopathy in cystic fibrosis: a case report #MMPMID29592796 Santoro D; Siligato R; Vadalà C; Lucanto M; Cristadoro S; Conti G; Buemi M; Costa S; Sabadini E; Magazzù G BMC Nephrol 2018[]; 19 (ä): ä PMID29592796show ga Background: C3 glomerulonephritis is a rare glomerulopathy characterized at renal biopsy by C3 deposition, alone or with scanty immunoglobulins, as well as by an electron-dense material in mesangium, subendothelial and subepithelial space. An abnormal systemic activation of the alternative pathway of the complement cascade is responsible for the development of the disease if triggered by several possible environmental conditions. We report the first case in literature of a patient affected by cystic fibrosis and C3GN. Case presentation: Our case involves a young woman with cystic fibrosis, who had persistent microscopic hematuria, proteinuria and hypocomplementemia C3 for over three months. Renal biopsy confirmed the diagnosis of C3 glomerulopathy. Complement system dysregulation was tested and resulted in a strong terminal pathway activation proved by high levels of sC5b-9 complex, amounting to 1588 ng/ml (normal value äC3 glomerulopathy in cystic fibrosis: a case report (epmc).pdf DeepDyve Pubget Overpricing