Nephrol Dial Transplant 1991;6(7):502-11

Combined liver-kidney and isolated liver transplantations for primary hyperoxaluria type 1: the European experience. The European Study Group on Transplantation in Hyperoxaluria Type 1

Watts RW, Danpure CJ, De Pauw L, Toussaint

Royal Postgraduate Medical School, Hammersmith, Hospital, London, UK

Abstract
The data provided by 14 European centres concerning 22 combined liver-kidney and
two isolated liver grafts performed in primary hyperoxaluria type 1 (PH1) were
discussed at a workshop which drew the following main conclusions: 1. In
end-stage renal failure due to PH1 1-year kidney graft survival rate is far
better after combined liver-kidney transplantation than after kidney
transplantation alone. This may be due to enhanced renal graft tolerance induced
by the simultaneously grafted liver, in addition to the reduced risk of
oxalate-induced damage to the kidney graft because the oxalate overproduction
has been corrected. 2. Prolonged dialysis using conventional regimes gives rise
to extensive systemic oxalosis, especially oxalate osteopathy, which leads to
long-lasting excretion of large amounts of oxalate even after oxalate synthesis
has been normalised by liver-kidney transplantation, with the risk of
jeopardising the success of the kidney graft. In addition, oxalate arteriopathy
may endanger the recipient's life. 3. Patients whose GFR is in the range of
25-60 ml/min per 1.73 m2 should be followed up closely, with sequential
assessments based on the rate of loss of overall renal function and the plasma
and urine oxalate values. An isolated liver transplantation should be considered
once the disease has been shown to be following an aggressive course. If this
strategy is not followed, planning for an elective liver-kidney graft should
begin when GFR decreases to about 25 ml/min per 1.73 m2 and the operation should
be as soon as possible. 4. As orthotopic liver transplantation involves the
removal of the recipient's biochemically defective but otherwise normal liver,
the diagnosis of PH1 should be unequivocally established in every case by the
measurement of alanine: glyoxylate aminotransferase enzyme activity in a
preoperative liver biopsy