Figure 1712

Primary hyperoxaluria type I in renal failure. Primary hyperoxaluria type I is an autosomal recessive inborn error of metabolism resulting from a deficiency (or occasionally incorrect subcellular localization) of hepatic peroxisomal alanine-glyoxylate aminotransferase [8]. Patients excrete excess oxalate as a result of the increased glyoxylate pool. In many patients, renal disease is manifested by chronic renal failure. Once the glomerular filtration rate has decreased below 25 mL/min the combination of oxalate overproduction and reduced urinary excretion leads to systemic oxalosis, with calcium oxalate deposition in many tissues. Renal transplantation alone has yielded poor results in the past, with 1-year graft survival rates of only 26% [3]. Combined hepatorenal transplantation simultaneously replaces renal function and corrects the underlying metabolic defect. The 1-year liver graft survival rate is 88%, with patient survival of 80% at 5 years. Of 24 renal grafts from the European experience of hepatorenal transplantation, 17 were still functioning at 3 months to 2 years after transplantation [19].

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