Combined liver and kidney transplantation (CLKT) is an important treatment option for patients suffering from diseases like autosomal recessive polycystic kidney disease (ARPKD) or primary hyperoxaluria type 1 (PH1). To examine the long-term outcome of this cohort all children undergoing CLKT at our centre between 1998 and 2014 were included in this retrospective analysis. 29 CLKT have been performed in 27 patients, suffering from PH1 (n= 15), ARPKD (n=11) and nephronophtisis type 11 (n=1). Median Age at tranplantation was 8.2 (1.3- 15.9) years with a body weight of 25 (9-53) kg. After a median follow-up of 6 (0.6- 10.8) years, patient-survival is 93% and liver and kidney graft survival 76 % and 79%, respectively. Currently liver function tests are in the normal range (median AST, ALT and INR 27 U/l, 23 U/l, 1.04). Three of the PH1 patients are on dialysis again at present; the median eGFR of the other patients is 72 (27- 132) ml/min/1,73m[sup2]. 6 out of 11 PH1 patients with functional renal grafts still have pathological range hyperoxaluria of > 0.45 mmol/1.73m[sup2] day. 4 of the PH1 patients have visual loss due to retinal oxalate deposition which did not improve after transplantation. The regression analysis yielded a significant impact on the long- term eGFR by analysing the eGFR baseline (p< 0.001), the period of dialysis before TX (p= 0.04) and the donor/recipient age ratio (p=0.019). In conclusion our results indicate that CLKT is a valuable and important option in the treatment of PH1 and ARPKD.

CITATION INFORMATION: Kemper M, Nashan B, Fischer L, Brinkert F. Combined Liver-Kidney Transplantation in Children: Long-Term Follow-Up in 27 Children. Am J Transplant. 2016;16 (suppl 3).

« Back to 2016 American Transplant Congress