Liver Transplants for Pediatric Metabolic Diseases in the Last Ten Years: Immediate and Long-Term Results

Objective: Liver transplantation is an accepted treatment modality in the pediatric patient group due to metabolic diseases. Patients are a more special population due to the presence of concomitant metabolic problems and the pediatric age group. There is no consensus on the long-term prognosis of pediatric patients with various rare liver diseases and inherited metabolic diseases. For this reason, we retrospectively evaluated our pediatric patients who underwent liver transplantation for pediatric metabolic liver diseases between 2012 and 2021.

Methods: Seventeen patients were included in the study. The mean Pediatric End-Stage Liver Disease (PELD) score was 21.47 (±8.47). Eight (47,1%) children received cadaveric, and 9 (52,9%) children received living liver transplantation. The mean age was 8.03 years (±6.37) (13 months-18 years). Left lateral graft was used in 11 patients (64%), right lobe graft in 3 patients (18%) and left lobe graft in 3 patients (18%). Six patients had biliary strictures out of which, 5 (29.4%) had percutaneous transhepatic biliary dilatation, and ERCP was performed in 1 (5.8%) patient.

Results: Our early survival rate was 100% and 2 patients (11,7%) were exitus in the late period for reasons unrelated to liver transplantation. Our mean follow-up period after transplantation was 70,6 months (±20.75) and no additional metabolic crisis was detected during the follow-up of the patients, including patients whose donors were relatives.

Conclusion: Metabolic liver diseases have severe systemic effects, and it is important to be evaluated by a multidisciplinary team in their diagnosis and treatment and to perform planned liver transplants rapidly without delay. Liver transplantation performed at the right time can prevent permanent additional organ damage.

Cite this article as: Umman V, Gümüş T, Barut D, et al. Liver transplants for pediatric metabolic diseases in the last ten years: Immediate and long-term results. Arch Basic Clin Res., 2023;5(3):404-408.