1999
DOI: 10.1159/000013428
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Oxalate Kinetics and Reversal of the Complications after Orthotopic Liver Transplantation in a Patient with Primary Hyperoxalosis Type 1 Awaiting Renal Transplantation

Abstract: 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 maintenance hemodialysis, and despite intensification of the dialytic treatment, developed severe livedo reticularis in her extremities leading to ischemic cutaneous ulcerations, necessitating continuous intravenous infusion of narcotics for pain control. She received a liver transplant after native hepatectomy. However, due to positive crossmatch, she could not receive… Show more

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Cited by 11 publications
(4 citation statements)
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“…Our subjects were highly selected for normality and may represent unusually healthy liver. Comparison with age‐matched hyperoxalosis normal liver tissue, often used in domino liver transplantation,32‐38 vindicated this approach, as there was no discernible difference in telomere length between the groups.…”
Section: Discussionmentioning
confidence: 99%
“…Our subjects were highly selected for normality and may represent unusually healthy liver. Comparison with age‐matched hyperoxalosis normal liver tissue, often used in domino liver transplantation,32‐38 vindicated this approach, as there was no discernible difference in telomere length between the groups.…”
Section: Discussionmentioning
confidence: 99%
“…Calcinosis cutis due to the deposition of oxalate in patients with primary hyperoxaluria improves after correction of the metabolic defect by liver transplantation 34. The same holds true for the livedo reticularis occasionally observed in patients with primary hyperoxaluria 35. The photosensitivity affecting patients with erythropoietic protoporphyria as a consequence of ferrochelatase deficiency disappears after liver transplantation but this may take months and even years 36–39.…”
Section: Evolution Of Preexisiting Dermatologic Conditions After Livementioning
confidence: 97%
“…113 Treatment with both pyridoxine and orthophosphate improved renal survival from 20% to 74% at 20 years in patients with PHO type I and II. 113 Liver transplantation to treat the enzyme deficiency is the definitive therapy for this condition, 114 but combined kidney-liver transplant may be necessary if nephrocalcinosis has developed. 115…”
Section: Hyperoxaluriamentioning
confidence: 99%