Native portal vein embolization for persistent hyperoxaluria following kidney and auxiliary partial liver transplantation.

[primary hyperoxaluria type 1]

Type 1 primary hyperoxaluria (PH 1 ) causes renal failure , for which isolated kidney transplantation (KT ) is usually unsuccessful treatment due to early oxalate stone recurrence . Although hepatectomy and liver transplantation (LT ) corrects PH 1 enzymatic defect , simultaneous auxiliary partial liver transplantation (APLT ) and KT have been suggested as an alternative approach . APLT advantages include preservation of the donor pool and retention of native liver function in the event of liver graft loss . However , APLT relative mass may be inadequate to correct the defect . We here report the first case of native portal vein embolization (PVE ) to increase APLT to native liver mass ratio (APLT /NLM-R ). Following initial combined APLT-KT , both allografts functioned well , but oxalate plasma levels did not normalize . We postulated the inadequate APLT /NLM-R could be corrected by trans-hepatic native PVE . The resulting increased APLT /NLM-R decreased serum oxalate to normal levels within 1 month following PVE . We conclude that persistently elevated oxalate levels after combined APLT-KT for PH 1 treatment , results from inadequate relative functional capacity . This can be reversed by partial native PVE to decrease portal flow to the native liver . This approach might be applicable to other scenarios where partial grafts have been transplanted to replace native liver function .

Diseases
Validation

Diseases presenting "inadequate relative functional capacity" symptom

primary hyperoxaluria type 1

You can validate or delete this automatically detected symptom