Primary hyperoxaluria type 1: strategy for organ transplantation.
CURR OPIN ORGAN TRAN
590 - 593.
Purpose of reviewPrimary hyperoxaluria type 1, the most common form of primary hyperoxaluria, is an autosomal recessive disorder caused by a deficiency of the liver-specific enzyme alanine: glyoxylate aminotransferase. This results in increased synthesis and subsequent urinary excretion of the metabolic end-product oxalate and the deposition of insoluble calcium oxalate in the kidney and urinary tract. As glomerular filtration rate decreases due to progressive renal involvement, oxalate accumulates and results in systemic oxalosis.Recent findingsDiagnosis is still often delayed. It is mainly established on the basis of clinical and sonographic findings, urinary oxalate +/- glycolate assessment, and DNA analysis.SummaryFollowing specific conservative measures, the ultimate management is based on organ transplantation. Correction of the enzyme defect by liver transplantation should be planned before systemic oxalosis develops to optimize outcomes and may be either simultaneous (immunological benefit) or sequential (biochemical benefit) liver-kidney transplantation depending on disease staging, facilities, and access to deceased or living donors. Allograft and patient survival currently approaches that of transplant patients with kidney transplantation alone and with other diseases requiring combined liver-kidney transplantation. In addition, this strategy has also provided significant improvement in both quality of life and statural growth.
|Title:||Primary hyperoxaluria type 1: strategy for organ transplantation|
|Keywords:||alanine:glyoxylate aminotransferase, combined liver-kidney transplantation, kidney transplantation, liver transplantation, primary hyperoxaluria type 1, SINGLE-CENTER EXPERIENCE, KIDNEY-TRANSPLANTATION, ALLOGRAFT SURVIVAL, LIVER, CHILDREN|
|UCL classification:||UCL > School of Life and Medical Sciences > Faculty of Life Sciences > Biosciences (Division of)|
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