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10.1007/8904_2014_403 http://scihub22266oqcxt.onion/10.1007/8904_2014_403 C4582031!4582031!25665838     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       JIMD+Rep 2015 ; 24 (ä): 13-20 Nephropedia Template TP {{tp|p=25665838|t=2015. Metabolic Effects of Increasing Doses of Nitisinone in the Treatment of Alkaptonuria |pdf=|usr=}}{{25665838}} gab.com Text Metabolic Effects of Increasing Doses of Nitisinone in the Treatment of Alkaptonuria JO: JIMD Rep http://www.kidney.de/pget.php?&tw=1&pmid=25665838 #FOAvip Alkaptonuria is an autosomal recessive disease involving a deficiency of the enzyme homogentisate dioxygenase, which is involved in the tyrosine degradation pathway. The enzymatic deficiency results in high concentrations of homogentisic acid (HGA), which results in orthopedic and cardiac complications, among other symptoms. Nitisinone (NTBC) has been shown to effectively treat alkaptonuria by blocking the conversion of 4-hydroxyphenylpyruvate to HGA, but there have been concerns that using doses higher than about 2 mg/day could cause excessively high levels of tyrosine, resulting in crystal deposition and corneal pathology. We have enrolled seven patients in a study to determine whether higher doses of NTBC were effective at further reducing HGA levels while maintaining tyrosine at acceptable levels. Patients were given varying doses of NTBC (ranging from 2 to 8 mg/day) over the course of between 0.5 and 3.5 years. Urine HGA, plasma tyrosine levels, and plasma NTBC were then measured longitudinally at various doses. We found that tyrosine concentrations plateaued and did not reach significantly higher levels as NTBC doses were increased above 2 mg/day, while a significant drop in HGA continued from 2 to 4 mg/day, with no significant changes at higher doses. We also demonstrated using untargeted metabolomics that elevations in tyrosine from treatment resulted in proportional elevations in alternative tyrosine metabolic products, that of N-acetyltyrosine and ?-glutamyltyrosine.Electronic supplementary material: The online version of this chapter (doi:10.1007/8904_2014_403) contains supplementary material, which is available to authorized users. Twit Text FOAVip Metabolic Effects of Increasing Doses of Nitisinone in the Treatment of Alkaptonuria ????JIMD Rep http://www.kidney.de/pget.php?&tw=1&pmid=25665838 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=25665838 #FOAvip English Wikipedia <ref>{{Cite pmid|25665838}}</ref> Metabolic Effects of Increasing Doses of Nitisinone in the Treatment of Alkaptonuria #MMPMID25665838 Gertsman I; Barshop BA; Panyard-Davis J; Gangoiti JA; Nyhan WL; Zschocke J JIMD Rep 2015[]; 24 (ä): 13-20 PMID25665838show ga Alkaptonuria is an autosomal recessive disease involving a deficiency of the enzyme homogentisate dioxygenase, which is involved in the tyrosine degradation pathway. The enzymatic deficiency results in high concentrations of homogentisic acid (HGA), which results in orthopedic and cardiac complications, among other symptoms. Nitisinone (NTBC) has been shown to effectively treat alkaptonuria by blocking the conversion of 4-hydroxyphenylpyruvate to HGA, but there have been concerns that using doses higher than about 2 mg/day could cause excessively high levels of tyrosine, resulting in crystal deposition and corneal pathology. We have enrolled seven patients in a study to determine whether higher doses of NTBC were effective at further reducing HGA levels while maintaining tyrosine at acceptable levels. Patients were given varying doses of NTBC (ranging from 2 to 8 mg/day) over the course of between 0.5 and 3.5 years. Urine HGA, plasma tyrosine levels, and plasma NTBC were then measured longitudinally at various doses. We found that tyrosine concentrations plateaued and did not reach significantly higher levels as NTBC doses were increased above 2 mg/day, while a significant drop in HGA continued from 2 to 4 mg/day, with no significant changes at higher doses. We also demonstrated using untargeted metabolomics that elevations in tyrosine from treatment resulted in proportional elevations in alternative tyrosine metabolic products, that of N-acetyltyrosine and ?-glutamyltyrosine.Electronic supplementary material: The online version of this chapter (doi:10.1007/8904_2014_403) contains supplementary material, which is available to authorized users. äMetabolic Effects of Increasing Doses of Nitisinone in the Treatment o(epmc).pdf DeepDyve Pubget Overpricing