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Published on: February 21, 2016
Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth
Florian Brinkert1, Rainer Ganschow, Knut Helmke
1Department of Pediatrics, Pediatric Gastroenterology and Hepatology, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany. f.brinkert@uke.de
Insights
Liver transplantation (LTx) can cure primary hyperoxaluria type 1 (PH1). Preemptive LTx (PLTx) is an option, while combined liver-kidney transplantation (LKTx) is effective for end-stage renal disease (ESRD) and improves growth.
Area of Science:
- Nephrology
- Hepatology
- Pediatric Gastroenterology
Background:
- Primary hyperoxaluria type 1 (PH1) is a metabolic disorder treatable with liver transplantation (LTx).
- Preemptive LTx (PLTx) aims to prevent kidney disease, but timing is challenging due to PH1 heterogeneity and effective conservative treatments.
- Combined liver-kidney transplantation (LKTx) addresses both metabolic defect and renal function, particularly for end-stage renal disease (ESRD).
Purpose of the Study:
- To evaluate the outcomes of different transplantation strategies in patients with PH1.
- To assess the efficacy of preemptive liver transplantation (PLTx) and combined liver-kidney transplantation (LKTx) in PH1 management.
- To analyze growth patterns post-transplantation in pediatric PH1 patients.
Main Methods:
- Retrospective analysis of 13 PH1 patients undergoing transplantation since 1995.
- Review of patients who received PLTx or combined LKTx.
- Assessment of graft survival, renal function, and growth parameters.
Main Results:
- Two patients undergoing sequential LKTx died early from infections.
- Four patients received PLTx, with three maintaining sufficient renal function after a median of 11.6 years.
- Seven ESRD patients received LKTx (including four with infantile oxalosis and three under 10 kg) with no mortality and observed catch-up growth.
Conclusions:
- Transplantation for PH1 is challenging but yields encouraging results, including improved growth.
- PLTx is a viable option for PH1 despite timing difficulties.
- LKTx is indicated for ESRD in PH1, feasible even in severe cases, and can enhance longitudinal growth.
Background:
Cure of the metabolic defect in primary hyperoxaluria type 1 (PH1) is possible with liver transplantation (LTx). Preemptive LTx (PLTx) was promoted to prevent chronic kidney disease due to nephrocalcinosis and urolithiasis. However, timing of this procedure is difficult in view of the heterogeneity of PH1 and effective conservative treatment. Combined liver-kidney transplantation (LKTx) is able to cure metabolic defect and replace renal function at the same time and is effective and indicated for patients with or approaching end-stage renal disease (ESRD). Sometimes a sequential approach for LKTx (first liver, then kidney) has been recommended.
Methods:
We report on 13 patients with PH1 since 1995 who underwent transplantation procedures in our center for PH1 at a median age of 4.7 (range 1.4-8.9) years.
Results:
The first two patients, planned for a sequential strategy, died early after LTx because of infectious complications. Four patients underwent PLTx at a median glomerular filtration rate of 65 (range 27-98) mL/min/1.73 m/day (Hoppe et al., Pediatr Nephrol 1996; 10: 488), and three patients still have sufficient residual renal function after a follow-up of median 11.6 years. Seven patients with ESRD received a combined LKTx, including four with infantile oxalosis, and three weighing less than 10 kg. There was no mortality and catch-up growth was observed in most patients.
Conclusion:
In summary and conclusion, transplantation procedures are challenging in PH1, but our results including growth data are encouraging. PLTx remains an option despite the difficulties in timing the procedure. LKTx is indicated for patients with ESRD and is possible even in patients with infantile oxalosis and may improve longitudinal growth.
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