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10.1007/8904_2017_44 http://scihub22266oqcxt.onion/10.1007/8904_2017_44 C5953902!5953902!28699143     free     free     free Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       JIMD+Rep 2018 ; 39 (ä): 25-30 Nephropedia Template TP {{tp|p=28699143|t=2018. Glutaric Aciduria Type 1 and Acute Renal Failure: Case Report and Suggested Pathomechanisms |pdf=|usr=}}{{28699143}} gab.com Text Glutaric Aciduria Type 1 and Acute Renal Failure: Case Report and Suggested Pathomechanisms JO: JIMD Rep http://www.kidney.de/pget.php?&tw=1&pmid=28699143 #FOAvip Glutaric aciduria type 1 (GA1) is caused by deficiency of the mitochondrial matrix enzyme glutaryl-CoA dehydrogenase (GCDH), leading to accumulation of glutaric acid (GA) and 3-hydroxyglutaric acid (3OHGA) in tissues and body fluids. During catabolic crises, GA1 patients are prone to the development of striatal necrosis and a subsequent irreversible movement disorder during a time window of vulnerability in early infancy. Thus, GA1 had been considered a pure ?cerebral organic aciduria? in the past. Single case reports have indicated the occurrence of acute renal dysfunction in children affected by GA1. In addition, growing evidence arises that GA1 patients may develop chronic renal failure during adulthood independent of the previous occurrence of encephalopathic crises. The underlying mechanisms are yet unknown. Here we report on a 3-year-old GA1 patient who died following the development of acute renal failure most likely due to haemolytic uraemic syndrome associated with a pneumococcal infection. We hypothesise that known GA1 pathomechanisms, namely the endothelial dysfunction mediated by 3OHGA, as well as the transporter mechanisms for the urinary excretion of GA and 3OHGA, are involved in the development of glomerular and tubular dysfunction, respectively, and may contribute to a pre-disposition of GA1 patients to renal disease. We recommend careful differential monitoring of glomerular and tubular renal function in GA1 patients. Twit Text FOAVip Glutaric Aciduria Type 1 and Acute Renal Failure: Case Report and Suggested Pathomechanisms ????JIMD Rep http://www.kidney.de/pget.php?&tw=1&pmid=28699143 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=28699143 #FOAvip English Wikipedia <ref>{{Cite pmid|28699143}}</ref> Glutaric Aciduria Type 1 and Acute Renal Failure: Case Report and Suggested Pathomechanisms #MMPMID28699143 du Moulin M; Thies B; Blohm M; Oh J; Kemper MJ; Santer R; Mühlhausen C JIMD Rep 2018[]; 39 (ä): 25-30 PMID28699143show ga Glutaric aciduria type 1 (GA1) is caused by deficiency of the mitochondrial matrix enzyme glutaryl-CoA dehydrogenase (GCDH), leading to accumulation of glutaric acid (GA) and 3-hydroxyglutaric acid (3OHGA) in tissues and body fluids. During catabolic crises, GA1 patients are prone to the development of striatal necrosis and a subsequent irreversible movement disorder during a time window of vulnerability in early infancy. Thus, GA1 had been considered a pure ?cerebral organic aciduria? in the past. Single case reports have indicated the occurrence of acute renal dysfunction in children affected by GA1. In addition, growing evidence arises that GA1 patients may develop chronic renal failure during adulthood independent of the previous occurrence of encephalopathic crises. The underlying mechanisms are yet unknown. Here we report on a 3-year-old GA1 patient who died following the development of acute renal failure most likely due to haemolytic uraemic syndrome associated with a pneumococcal infection. We hypothesise that known GA1 pathomechanisms, namely the endothelial dysfunction mediated by 3OHGA, as well as the transporter mechanisms for the urinary excretion of GA and 3OHGA, are involved in the development of glomerular and tubular dysfunction, respectively, and may contribute to a pre-disposition of GA1 patients to renal disease. We recommend careful differential monitoring of glomerular and tubular renal function in GA1 patients. äGlutaric Aciduria Type 1 and Acute Renal Failure: Case Report and Sugg(epmc).pdf DeepDyve Pubget Overpricing