Primary hyperoxaluria type 1 (PH1) is a rare genetic disorder characterized by hepatic overproduction of oxalate. Excretion excess oxalate the kidneys leads to recurrent kidney stones, nephrocalcinosis, progressive disease, and multiorgan damage from systemic oxalosis. Patients progressing or presenting with failure require invasive approaches such as dialysis liver and/or transplantation. Ther...

SIR—Type 1 primary hyperoxaluria (PH1) is an innate error of the metabolism characterized by the deficiency or absence of the peroxisomal hepatic enzyme alanine-glyoxalate aminotransferase, which promotes the transamination of glyoxalate to glycine. Renal and skeletal systems are the main targets of the disease that also may involve heart, nerves, joints, arteries, skin, soft tissues, and retin...

Ascorbic acid overload and vitamin B6 deficiency have been implicated in the development of hyperoxalemia in dialysis patients, but there is still disagreement about this. Hemodialysis patients who are exposed long-term hyperoxalemia may develop secondary oxalosis with an increased risk of cardiac, vascular, and bone disease, and thus may benefit from maintaining a low serum oxalic acid level. ...

BACKGROUND Primary hyperoxaluria (PH) is a rare genetic disease, in which high urinary oxalate (Uox) cause recurrent kidney stones and/or progressive nephrocalcinosis, often followed by early end-stage renal disease, as well as extremely high plasma oxalate, systemic oxalosis and premature death. Oxalobacter formigenes, an anaerobic oxalate degrading bacterium, naturally colonizes the colon of ...

Ketohexokinase (EC 2.7.1.3) was purified to homogeneity from human liver, and fructose-bisphosphate aldolase (EC 4.1.2.13) was partially purified from the same source. Ketohexokinase was shown, by column chromatography and polyacrylamide-gel electrophoresis, to be a dimer of Mr 75000. Inhibition studies with p-chloromercuribenzoate and N-ethylmaleimide indicate that ketohexokinase contains thio...

Many patients receiving renal allografts become identified simply as recipients of kidney transplantation. All subsequent events involving changes in renal function are attributed to the process and natural history of transplantation itself: acute and chronic rejection, immunosuppressive drug nephrotoxicity, graft vasculature thrombosis or stenosis, ischemia, infection, and lymphoproliferative ...