15 September 2017 : Original article
Polish Experience with Liver Transplantation and Post-Transplant Outcomes in Children with Urea Cycle Disorders
Edyta Szymańska1ABCDEF*, Piotr Kaliciński2ADE, Joanna Pawłowska3ADE, Sylwia Szymańska4E, Maciej Pronicki4D, Marek Stefanowicz2B, Joanna Teisseyre2B, Dorota Broniszczak2B, Dariusz Rokicki1ABDEFDOI: 10.12659/AOT.904580
Ann Transplant 2017; 22:555-562
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.
Keywords: hyperammonemia, Liver Transplantation, Urea Cycle Disorders, Inborn
In Press
18 Mar 2024 : Original article
Does Antibiotic Use Increase the Risk of Post-Transplantation Diabetes Mellitus? A Retrospective Study of R...Ann Transplant In Press; DOI: 10.12659/AOT.943282
20 Mar 2024 : Original article
Transplant Nephrectomy: A Comparative Study of Timing and Techniques in a Single InstitutionAnn Transplant In Press; DOI: 10.12659/AOT.942252
28 Mar 2024 : Original article
Association Between FEV₁ Decline Rate and Mortality in Long-Term Follow-Up of a 21-Patient Pilot Clinical T...Ann Transplant In Press; DOI: 10.12659/AOT.942823
02 Apr 2024 : Original article
Liver Transplantation from Brain-Dead Donors with Hepatitis B or C in South Korea: A 2014-2020 Korean Organ...Ann Transplant In Press; DOI: 10.12659/AOT.943588
Most Viewed Current Articles
05 Apr 2022 : Original article
Impact of Statins on Hepatocellular Carcinoma Recurrence After Living-Donor Liver TransplantationDOI :10.12659/AOT.935604
Ann Transplant 2022; 27:e935604
12 Jan 2022 : Original article
Risk Factors for Developing BK Virus-Associated Nephropathy: A Single-Center Retrospective Cohort Study of ...DOI :10.12659/AOT.934738
Ann Transplant 2022; 27:e934738
22 Nov 2022 : Original article
Long-Term Effects of Everolimus-Facilitated Tacrolimus Reduction in Living-Donor Liver Transplant Recipient...DOI :10.12659/AOT.937988
Ann Transplant 2022; 27:e937988
15 Mar 2022 : Case report
Combined Liver, Pancreas-Duodenum, and Kidney Transplantation for Patients with Hepatitis B Cirrhosis, Urem...DOI :10.12659/AOT.935860
Ann Transplant 2022; 27:e935860