Liver Transplantation in Primary Hyperoxaluria Type 1: We Have to Find an Alternative!

Abstract

FIGURE 1. Glyoxylate metabolism in the hepatocyte in primary hyperoxaluria type I. In the peroxisome of normal hepatocyte, GO catalyzes the conversion of glycolate to glyoxylate. Then AGT catalyzes the conversion of glyoxylate to glycine. In primary hyperoxaluria type 1, glyoxylate accumulates as a result of AGT deficiency and is converted to oxalate by hepatic LDH and GO and to glycolate by GRHPR. Oxalate and glycolate are finally eliminated from the body by the kidneys. AGT, alanine-glyoxylate aminotransferase; GO, glycolate oxidase; GRHPR, glyoxylate reductase-hydroxypyruvate reductase; LDH, lactate dehydrogenase.

Primary hyperoxaluria diagnosed after kidney transplantation: a case report and literature review

“…However, hemodialysis cannot completely solve the problem of continued deposition of CaOx in important organs. Currently, liver transplantation is the only way to cure PH [ 28 ]. Our patient underwent a liver transplantation 3 months after loss of renal allograft function.…”

Monogenic Kidney Diseases in Kidney Transplantation

“… 42 , 43 However, solid organ transplantation is challenging for patients with PH with KF, because of the high-risk of recurrence of oxalate nephropathy in the transplanted kidney, with risk of graft loss. 42 , 43 , 44 , 45 , 46 Isolated KT may be considered in very selected patients who are vitamin B6-sensitive. 47 A more accurate assessment of systemic oxalosis using 18-Fluoro-D glucose positron emission tomography imaging may help to decide the right timing of KT.…”

Lumasiran for Advanced Primary Hyperoxaluria Type 1: Phase 3 ILLUMINATE-C Trial

“…There is hope in the medical community that novel therapies that target hepatic oxalate production will obviate the need for a liver transplant. [34][35][36] Kidney-only transplantation has been performed with good outcomes in a limited number of patients after adequate response to pyridoxine. 37 More recently, in a young patient diagnosed with PH1 following a kidney transplant, graft kidney function stabilized following lumasiran and aggressive kidney replacement therapy, allowing for discontinuation of dialysis.…”