Nephrol Dial Transplant 1995;10 Suppl 8:47-52

When should isolated liver transplantation be performed in primary hyperoxaluria
type 1? Follow-up report of two children

Haffner D, Cochat P, Otto G, Steffen H, Scharer K

Department of Pediatrics, University of Heidelberg, Germany

Abstract
The value of isolated liver transplantation in primary hyperoxaluria type 1
(PH1) and its timing are controversial. Two children with PH1 were followed up
for 6 years after liver transplantation. A boy aged 5 years with a glomerular
filtration rate (GFR) of 65 ml/min/1.73 m2 at the time of transplantation
subsequently showed regression of calcium deposits in the kidneys, but GFR
slightly declined, probably related to cyclosporin A toxicity. A girl with a
severe neonatal form of PH1 received a liver at the age of 2 years when GFR had
already declined to about 15 ml/min/1.73 m2 and extrarenal manifestations
(bones, eyes) were present. In spite of these and further complications
post-transplantation GFR stabilized. Growth failure was efficiently treated by
growth hormone. Plasma and urinary oxalate concentrations, however, remained
increased. It is suggested that in PH1 liver transplantation should be
considered only if GFR is not rapidly declining and stays above 30 ml/min/1.73
m2. For an individual decision the different risks of liver transplantation and
of combined kidney-liver transplantation as well as the possible benefits from
previous conservative treatment must be considered