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10.1136/bcr-2017-222036 http://scihub22266oqcxt.onion/10.1136/bcr-2017-222036 C5878378!5878378!29592975     free     free     free Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\29592975.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117       BMJ+Case+Rep 2018 ; 2018 (ä): ä Nephropedia Template TP {{tp|p=29592975|t=2018. Fulminant hepatic failure in the setting of progressive ANCA-associated vasculitis associated with a rare alpha-1 antitrypsin phenotype, ?PiEE |pdf=|usr=}}{{29592975}} gab.com Text Fulminant hepatic failure in the setting of progressive ANCA-associated vasculitis associated with a rare alpha-1 antitrypsin phenotype, PiEE JO: BMJ Case Rep http://www.kidney.de/pget.php?&tw=1&pmid=29592975 #FOAvip Abnormalities in alpha-1 antitrypsin (AAT) proteins are risk factors for human disease. While the most common is AAT deficiency, a genetic disorder associated with chronic obstructive pulmonary disease, additional disorders associated with AAT abnormalities are increasingly recognised. We describe a middle-aged woman who presented with fulminant hepatic and multiorgan failure. Evaluation revealed the patient to have a rare AAT phenotype PiEE. Her clinical presentation was consistent with antineutrophilic cytoplasmic antibody-associated vasculitis, and her history suggested features of panniculitis. This is the first description of this rare homozygous AAT phenotype and possible disease associations with the ?E' protein. Given that abnormal AAT are under-recognised, and that new mutations and phenotypes continue to be identified, we will need to expand on our knowledge base and report clinical manifestations associated with these abnormal phenotypes. Twit Text FOAVip Fulminant hepatic failure in the setting of progressive ANCA-associated vasculitis associated with a rare alpha-1 antitrypsin phenotype, PiEE ????BMJ Case Rep http://www.kidney.de/pget.php?&tw=1&pmid=29592975 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=29592975 #FOAvip English Wikipedia <ref>{{Cite pmid|29592975}}</ref> Fulminant hepatic failure in the setting of progressive ANCA-associated vasculitis associated with a rare alpha-1 antitrypsin phenotype, ?PiEE #MMPMID29592975 Reilkoff R; Stephenson L BMJ Case Rep 2018[]; 2018 (ä): ä PMID29592975show ga Abnormalities in alpha-1 antitrypsin (AAT) proteins are risk factors for human disease. While the most common is AAT deficiency, a genetic disorder associated with chronic obstructive pulmonary disease, additional disorders associated with AAT abnormalities are increasingly recognised. We describe a middle-aged woman who presented with fulminant hepatic and multiorgan failure. Evaluation revealed the patient to have a rare AAT phenotype PiEE. Her clinical presentation was consistent with antineutrophilic cytoplasmic antibody-associated vasculitis, and her history suggested features of panniculitis. This is the first description of this rare homozygous AAT phenotype and possible disease associations with the ?E' protein. Given that abnormal AAT are under-recognised, and that new mutations and phenotypes continue to be identified, we will need to expand on our knowledge base and report clinical manifestations associated with these abnormal phenotypes. äFulminant hepatic failure in the setting of progressive ANCA-associate(epmc).pdf DeepDyve Pubget Overpricing