Primary hyperoxaluria: Simultaneous combined liver and kidney transplantation from a living related donor

Primary hyperoxaluria type 1 (PH1) is a rare inherited metabolic disorder in which deficiency of the liver enzyme AGT leads to renal failure and systemic oxalosis. Timely, combined cadaveric liver-kidney transplantation (LKT) is recommended for end-stage renal failure (ESRF) caused by PH1; however,...

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Bibliographic Details
Published in:Liver transplantation 2003-04, Vol.9 (4), p.433-436
Main Authors: Astarcioglu, Ibrahim, Karademir, Sedat, Gülay, Hüseyin, Bora, Seymen, Astarcioglu, Hüseyin, Kavukcu, Salih, Türkmen, Mehmet, Soylu, Alper
Format: Article
Language:English
Subjects:
Adult
Child
Humans
Hyperoxaluria, Primary - complications
Hyperoxaluria, Primary - surgery
Kidney Failure, Chronic - etiology
Kidney Failure, Chronic - surgery
Kidney Transplantation
Liver Transplantation
Living Donors
Male
Oxalates - urine
Time Factors
Treatment Outcome
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Summary:Primary hyperoxaluria type 1 (PH1) is a rare inherited metabolic disorder in which deficiency of the liver enzyme AGT leads to renal failure and systemic oxalosis. Timely, combined cadaveric liver-kidney transplantation (LKT) is recommended for end-stage renal failure (ESRF) caused by PH1; however, the shortage of cadaveric organs has generated enthusiasm for living-related transplantation in years. Recently, successful sequential LKT from the same living donor has been reported in a child with PH1. We present a sister-to-brother simultaneous LKT in a pediatric patient who suffered from PH1 with ESRF. Twelve months after transplantation, his daily urine oxalate excretion was decreased from 160 mg to 19.5 mg with normal liver and renal allograft functions. In addition to the well-known advantages of living organ transplantation, simultaneous LKT may facilitate early postoperative hemodynamic stability and may induce immunotolerance and allow for low-dose immunosuppression. (Liver Transpl 2003;9:433-436.)
ISSN:1527-6465
1527-6473
DOI:10.1053/jlts.2003.50072