title: Long-term results of combined liver-kidney transplantation for primary hyperoxaluria type 1: the French experience.
abstract: Primary hyperoxaluria type 1 (PH1) is a hepatic metabolic defect leading to end-stage renal failure. The posttransplant recurrence of kidney disease can suggest a need for combined liver-kidney transplantation (LKT). However, the risk of LKT is theoretically far higher than the risk of kidney-alone transplantation (KAT). An unselected consecutive series of 54 patients with PH1 was analyzed according to the type of transplantation initially performed between May 1979 and June 2010 at 10 French centers. The duration of dialysis, extrarenal lesions, age, and follow-up were similar between the groups. Postoperative morbidity and mortality did not differ between the groups, and 10-year patient survival rates were similar for the LKT (n = 33) and KAT groups (n = 21; 78% versus 70%). Kidney graft survival at 10 years was better after LKT (87% versus 13%, P < .001) . Four patients (12.1%) lost their first kidney graft in the LKT group, whereas 19 (90%) did in the KAT group (P < .001). The recurrence of oxalosis occurred in 11 renal grafts (52%) in the KAT group but in none in the LKT group (P < .001). End-stage renal failure resulting from rejection was also higher in the KAT group (19% versus 9%, P < 0.0001). A second kidney transplant was performed for 15 patients (71%) in the KAT group versus 4 patients (12%) in the LKT group (P < 0.001). In conclusion, LKT for PH1 provides better kidney graft survival, less rejection, and similar long-term patient survival and is not associated with an increased short-term mortality risk. LKT must be the first-line treatment for PH1 patients with end-stage renal disease.
authors.affiliation: Service de Chirurgie Digestive et Hépato-Biliaire-Transplantation Hépatique.
authors.forename: Philippe
authors.initials: P
authors.lastname: Compagnon
authors.forename: Philippe
authors.initials: P
authors.lastname: Metzler
authors.forename: Didier
authors.initials: D
authors.lastname: Samuel
authors.forename: Christophe
authors.initials: C
authors.lastname: Camus
authors.forename: Patrick
authors.initials: P
authors.lastname: Niaudet
authors.forename: Antoine
authors.initials: A
authors.lastname: Durrbach
authors.forename: Philippe
authors.initials: P
authors.lastname: Lang
authors.forename: Daniel
authors.initials: D
authors.lastname: Azoulay
authors.forename: Christophe
authors.initials: C
authors.lastname: Duvoux
authors.forename: Francois
authors.initials: F
authors.lastname: Bayle
authors.forename: Joseph
authors.initials: J
authors.lastname: Rivalan
authors.forename: Pierre
authors.initials: P
authors.lastname: Merville
authors.forename: Gerard
authors.initials: G
authors.lastname: Pascal
authors.forename: Eric
authors.initials: E
authors.lastname: Thervet
authors.forename: Albert
authors.initials: A
authors.lastname: Bensman
authors.forename: Lionel
authors.initials: L
authors.lastname: Rostaing
authors.forename: Georges
authors.initials: G
authors.lastname: Deschenes
authors.forename: Jeff
authors.initials: J
authors.lastname: Morcet
authors.forename: Cyrille
authors.initials: C
authors.lastname: Feray
authors.forename: Karim
authors.initials: K
authors.lastname: Boudjema
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006085:Graft Survival; D006801:Humans; D006959:Hyperoxaluria / Q000150:complications / Q000601:surgery; D006960:Hyperoxaluria, Primary / Q000401:mortality / Q000601:surgery*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007676:Kidney Failure, Chronic / Q000601:surgery; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D012086:Reoperation; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents