Oxalate kinetics and reversal of the complications after orthotopic liver transplantation in a patient with primary hyperoxalosis type 1 awaiting renal transplantation
B. Bastani et al., Oxalate kinetics and reversal of the complications after orthotopic liver transplantation in a patient with primary hyperoxalosis type 1 awaiting renal transplantation, AM J NEPHR, 19(1), 1999, pp. 64-69
We present the case of a young woman with end-stage renal disease secondary
to primary hyperoxaluria type 1, who after 3 years and 6 months of mainten
ance hemodialysis, and despite intensification of the dialytic treatment, d
eveloped severe livedo reticularis in her extremities leading to ischemic c
utaneous ulcerations, necessitating continuous intravenous infusion of narc
otics for pain control. She received a liver transplant after native hepate
ctomy. However, due to positive crossmatch, she could not receive a kidney
from that donor. After transplantation, following serial serum oxalate leve
ls, the hemodialysis regimen was safely reduced from 4 h daily to 3 h three
times weekly. Over the course of 6 weeks after liver transplantation, her
livedo reticularis resolved, the ischemic ulcers markedly improved, she was
weaned off all pain medications, and her erythropoietin-resistant anemia r
esolved. Our results suggest that in patients with primary hyperoxaluria ty
pe 1, who have received a liver transplant and are on maintenance hemodialy
sis, after serial serum oxalate determinations, some may safely be changed
to a thrice-weekly maintenance hemodialysis regimen. Moreover, with this re
gimen the complications of systemic oxalosis can reverse.