title: Renal transplantation in primary hyperoxaluria.
abstract: The increased production and excretion of oxalate in primary hyperoxaluria causes urolithiasis, nephrocalcinosis with renal failure, and systemic oxalosis. Systemic oxalosis occurs late in the course of the disease when there is both oxalate retention and increased oxalate synthesis. The uraemia can be controlled by conventional haemodialysis or peritoneal dialysis but treatment cannot usually keep up with accelerated rate of oxalate production, and dialysed patients develop systemic oxalosis. Most attempts to treat primary hyperoxaluria by renal transplantation have been unsuccessful because of rapid recurrence of nephrocalcinosis with uraemia and systemic oxalosis. Dynamic studies of overall oxalate metabolism in vivo have shown that the renal retention factor becomes a major determinant of oxalosis when the GFR decreases to less than 25 ml min-1 1.73 m-2. We conclude provisionally that vigorous haemodialysis should be begun and transplantation arranged when the GFR reaches this level. Such early transplantation with vigorous perioperative haemodialysis and a large perioperative diuresis of water gives good immediate graft function and oxalate mobilisation from the miscible oxalate pool. The longer term outlook is then influenced more by the factors which determine the success of renal transplantation in non-hyperoxaluric patients.
authors.affiliation: Division of Inherited Metabolic Diseases, MRC Clinical Research Centre, Harrow, United Kingdom.
authors.forename: R W
authors.initials: RW
authors.lastname: Watts
authors.forename: S H
authors.initials: SH
authors.lastname: Morgan
authors.forename: M A
authors.initials: MA
authors.lastname: Mansell
authors.forename: P
authors.initials: P
authors.lastname: Purkiss
mesh_terms: D005919:Glomerular Filtration Rate; D006801:Humans; D006959:Hyperoxaluria / Q000601:surgery*; D006960:Hyperoxaluria, Primary / Q000150:complications / Q000601:surgery*; D016030:Kidney Transplantation; D009397:Nephrocalcinosis / Q000209:etiology; D010070:Oxalates / Q000378:metabolism; D019815:Oxalic Acid; D012008:Recurrence; D006435:Renal Dialysis
chemical_list: D010070:Oxalates; D019815:Oxalic Acid