PEDIATRICS

F. Brinkert¹, A. Briem-richter², S. Thorban³, B. Nashan⁴, R. Ganschow², M.J. Kemper^5
1Department Of Pediatrics, Pediatric Gastroenterology And Hepatology, University Medical Center Hamburg-Eppendorf, Hamburg/GERMANY, ²Department Of Hepatobiliary And Transplant Surgery, Pediatric Hepatology And Liver Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg/GERMANY, ³Department Of Surgery, Division Of Transplantation, Technische University, Munich/GERMANY, ⁴Hepatobiliary And Transplant Surgery, University Medical Centre Hamburg - Eppendorf, Hamburg/GERMANY, ⁵Pediatric Nephrology, Department Of Pediatrics, University Medical Center Hamburg, Hamburg/GERMANY

Body: Introduction: ARPKD often results in end-stage-renal disease and the necessity of renal replacement therapy in early childhood. Congenital liver fibrosis is a feature of ARPKD and may result in hepatosplenomegaly and portal hypertension, sometimes complicated by oesophageal varices. Thus, LKTx may be necessary. Aim of the study was to analyse the long-term course of children with ARPKD after LKTx. Methods: We conducted a retrospective chart analysis of seven patients who underwent a combined Tx between the years 2003 and 2009 at the University Medical Center Hamburg-Eppendorf. In all patients clinical and laboratory data were collected. Concerning the longitudinal growth development Standard Deviation Score (SDS) were recorded. Results: Seven children were transplanted at a median age of 10 years (range 1.7-16 years) and with a median weight of 18.2 kg (range 10.5–55 kg). After a median follow-up of 2.7 years (range 0.6-6.3 years) all patients are alive. One Patient underwent a solitary liver re-transplantation. The first transplanted patient had to be retransplanted due to kidney artery thrombosis. A single kidney transplantation was necessary due to non adherence in the later follow-up. Today the liver and kidney function of all patients are stable. The median creatinine of 0.69 mg/dl (range 0.36-1.57mg/dl) results in a calculated GFR of median 120 ml/min/1.73m² (range 68-133 ml/min/1.73m²). The longitudinal growth data show a catch-up growth after LKTx. Conclusion: The results of LKTx for children with ARPKD are encouraging. Both the lack of mortality and successful catch-up growth shows that LKTx is an important therapy for patients with ARPKD affecting liver and kidney.

Disclosure: All authors have declared no conflicts of interest.