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Polish Experience with Liver Transplantation and Post-Transplant Outcomes in Children with Urea Cycle Disorders
Edyta Szymańska1, Piotr Kaliciński2, Joanna Pawłowska3
1Department of Pediatrics, Nutrition and Metabolic Disorders, The Children's Memorial Health Institute, Warsaw, Poland.
Insights
Liver transplantation (LT) effectively treats urea cycle disorders (UCDs), eradicating hyperammonemia and improving neurocognitive outcomes. This study highlights successful LT in pediatric UCD patients, with high survival rates and improved quality of life.
Area of Science:
- Pediatric Hepatology
- Metabolic Disorders
- Transplant Surgery
Background:
- Liver transplantation (LT) is a recommended treatment for urea cycle disorders (UCDs).
- Evaluating LT outcomes for UCDs in a pediatric population is crucial for understanding treatment efficacy.
- This study focuses on LT for UCDs at a tertiary reference hospital in Warsaw, Poland.
Purpose of the Study:
- To determine the indications for liver transplantation in children with urea cycle disorders.
- To assess the outcomes, including survival and neurocognitive development, of liver transplantation for UCDs.
- To analyze graft and patient survival rates following LT for UCDs.
Main Methods:
- Retrospective review of medical charts of pediatric UCD patients who underwent LT between 2008 and July 2016.
- Analysis of pre- and post-transplant parameters: symptoms, age at diagnosis/transplantation, graft characteristics, survival, complications, and biochemical results.
- Evaluation of neurocognitive development and developmental delay before and after LT.
Main Results:
- Twelve pediatric patients with UCD received 14 liver grafts (whole or reduced-size) via living or deceased donation.
- High survival rates: 100% patient survival at 30 days and 1 year, 93% graft survival at 30 days, and 85.7% at 1 year.
- LT successfully managed hyperammonemia, allowed dietary liberalization, and stabilized or improved neurocognitive status in most patients.
Conclusions:
- Liver transplantation is a highly effective treatment for pediatric urea cycle disorders.
- LT leads to the eradication of hyperammonemia and removal of dietary restrictions.
- Transplantation can improve or maintain neurocognitive development in children with UCDs.
Abstract:
BACKGROUND Liver transplantation (LT) is recommended for various metabolic diseases, including urea cycle disorders (UCDs). The aim of this study was to determine indications and outcomes of LT for UCDs in the tertiary reference Children's Hospital in Warsaw, Poland. MATERIAL AND METHODS Medical charts of children with UCD who underwent LT between 2008 and July 2016 were retrospectively reviewed. The following parameters were analyzed: symptoms at time of diagnosis, age at diagnosis, age at transplantation, graft characteristics and survival, postsurgical complications, and biochemical and laboratory results before and after transplantation. RESULTS Twelve patients with UCD who underwent LT at a mean age of 5 y (0.5-14 y) received a total of 14 liver grafts. Four children (33%) received a living donor graft, while 8 (68%) got a deceased donor liver graft. A total number of transplanted organs consisted of 9 (64%) whole-liver grafts and 5 (36%) reduced-size grafts. The 30-day post-transplant patient survival rate was 100% and graft survival rate was 93% (13/14). For those with a post-transplant follow-up of at least 1 year (n=10/12), the 1-year patient survival rate was 100% and the graft survival rate was 85.7% (12/14). Median peak of blood ammonia at presentation was 653 (159-2613) µg/dL (normal <80 µg/dl), and median peak of blood glutamine was 1273.2 µmol/l (964-3900 µmol/l). There was 1 episode of hyperammonemia following LT, but it was not due to UCD. Six (50%) patients were diagnosed with some degree of developmental delay/neurological impairment before transplantation, which remained stable or slightly improved after transplantation. Patients without developmental delay before transplantation maintained their cognitive abilities at follow-up. CONCLUSIONS LT leads to eradication of hyperammonemia, withdrawal of dietary restrictions with low-protein diet, and potentially improved neurocognitive development.
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