Liver-kidney-transplantation in type 1 primary hyperoxaluria: description and comments on a case

Background: Primary hyperoxaluria leads to oxalosis, a systemic illness wit h fatal prognosis in uremic youngsters because of systemic complications. C ase report: A 14-year old boy with primary type 1 hyperoxaluria who had a l ong-lasting history of nephrolithiasis and passed from normal renal functio n to end-stage renal disease within 7 months. Measurement of alanine: glyox ylate aminotransferase (AGT) catalytic activity in the liver biopsy disclos ed very low activity which was not responsive to pyridoxin., thus the patie nt entered onto a priority national waiting list for liver-kidney transplan tation and a week later received a combined transplant. in order to increas e body clearance of oxalate, the patient underwent medical treatment to inc rease urine oxalate solubility (sodium and potassium citrate oral therapy, magnesium supplementation and increase of diuresis) and intensive dialysis both before and after transplantation. Comment: The medical approach to the treatment of this rare illness is discussed. Since the major risk for the grafted kidney is related to the oxalate burden, i.e. oxalate deposition fr om the body deposits to the kidney that becomes irreversibly damaged, treat ment consists of increasing the body clearance of oxalate both by increasin g oxalate solubility in the urine and with intensive dialysis performed bot h before and after combined transplantation. To the same extent (by limitin g body oxalate deposits), a relatively early (native GFR 20-25 ml/minute) t ransplantation is advisable.