title: Primary hyperoxaluria: simultaneous combined liver and kidney transplantation from a living related donor.
abstract: 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.
authors.affiliation: General Surgery, Transplantation Unit, Dokuz Eylul University, School of Medicine, Inciralti, Izmir, Turkey.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Astarcioglu
authors.forename: Sedat
authors.initials: S
authors.lastname: Karademir
authors.forename: Hüseyin
authors.initials: H
authors.lastname: Gülay
authors.forename: Seymen
authors.initials: S
authors.lastname: Bora
authors.forename: Hüseyin
authors.initials: H
authors.lastname: Astarcioglu
authors.forename: Salih
authors.initials: S
authors.lastname: Kavukcu
authors.forename: Mehmet
authors.initials: M
authors.lastname: Türkmen
authors.forename: Alper
authors.initials: A
authors.lastname: Soylu
mesh_terms: 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
chemical_list: D010070:Oxalates