コンテンツへスキップ
Merck
  • Primary hyperoxaluria Type 1: indications for screening and guidance for diagnosis and treatment.

Primary hyperoxaluria Type 1: indications for screening and guidance for diagnosis and treatment.

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association (2012-05-02)
Pierre Cochat, Sally-Anne Hulton, Cécile Acquaviva, Christopher J Danpure, Michel Daudon, Mario De Marchi, Sonia Fargue, Jaap Groothoff, Jérôme Harambat, Bernd Hoppe, Neville V Jamieson, Markus J Kemper, Giorgia Mandrile, Martino Marangella, Stefano Picca, Gill Rumsby, Eduardo Salido, Michael Straub, Christiaan S van Woerden
要旨

Primary hyperoxaluria Type 1 is a rare autosomal recessive inborn error of glyoxylate metabolism, caused by a deficiency of the liver-specific enzyme alanine:glyoxylate aminotransferase. The disorder results in overproduction and excessive urinary excretion of oxalate, causing recurrent urolithiasis and nephrocalcinosis. As glomerular filtration rate declines due to progressive renal involvement, oxalate accumulates leading to systemic oxalosis. The diagnosis is based on clinical and sonographic findings, urine oxalate assessment, enzymology and/or DNA analysis. Early initiation of conservative treatment (high fluid intake, pyridoxine, inhibitors of calcium oxalate crystallization) aims at maintaining renal function. In chronic kidney disease Stages 4 and 5, the best outcomes to date were achieved with combined liver-kidney transplantation.

材料
製品番号
ブランド
製品内容

Sigma-Aldrich
クエン酸カリウム 三塩基性 一水和物, ≥98% (GC/titration)
Sigma-Aldrich
クエン酸カリウム 三塩基性 一水和物, purum p.a., ≥99.0% (NT)
Sigma-Aldrich
クエン酸カリウム 三塩基性 一水和物, suitable for cell culture
Sigma-Aldrich
クエン酸カリウム 三塩基性 一水和物, meets USP testing specifications
Sigma-Aldrich
クエン酸カリウム 三塩基性 一水和物, meets analytical specification of Ph. Eur., BP, FCC, E332, 99-100.5% (calc with ref. to anhyd. subst.)
Sigma-Aldrich
クエン酸カリウム 三塩基性 一水和物, SAJ special grade, ≥99.0%