To the Editor: Primary hyperoxaluria type I is an autosomalrecessive disorder characterized by a deficiency of liver-specificperoxisomal alanineglyoxylate aminotransferase (EC 2.6.1.44).The disease leads to increased urinary excretion of oxalate,causing urolithiasis, nephrocalcinosis, progressive renal insufficiency,and eventually, oxalosis that is, the accumulation ofinsoluble oxalate throughout the body.
The interval between the onset of symptoms and the developmentof end-stage renal failure varies. Although optimal conservativetherapy can delay end-stage renal failure, it usually cannotprevent it. Kidney transplantation to correct end-stage renalfailure is frequently followed by recurrence of the diseasein the transplanted kidney. . . . [Full Text of this Article]
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