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, 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.
Astarcioglu
Ibrahim
I
General Surgery, Transplantation Unit, Dokuz Eylul University, School of Medicine, Inciralti, Izmir, Turkey.
Karademir
Sedat
S
Gülay
Hüseyin
H
Bora
Seymen
S
Astarcioglu
Hüseyin
H
Kavukcu
Salih
S
Türkmen
Mehmet
M
Soylu
Alper
A
D000328:Adult; D002648:Child; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications* / Q000601:surgery*; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000601:surgery*; D016030:Kidney Transplantation; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D010070:Oxalates / Q000652:urine; D013997:Time Factors; D016896:Treatment Outcome
D010070:Oxalates