Comprehensive analysis of the efficacy of liver transplantation in pediatric patients with Wilson’s disease

SARICI B., ŞAHİN T. T., ara c., AYDIN C., KARAKAS S., İNCE V., ...Daha Fazla

Annals of Medical Research, cilt.30, sa.1, ss.1-6, 2023 (TRDizin)

Özet

Aim: The aim of the present study is to evaluate the results of liver transplantation (LT) in pediatric Wilson disease (WD) with a specific sub-analysis in patients with neuropsychiatric symptoms. Materials and Methods: Demographic, operative, laboratory and neurologic findings of 23 pediatric patients with WD that underwent LT were analyzed by examining the patient charts. Results: Median age of the patients was 13 ( 7 to 17) years. Median Wilson’s Index scores of the patients were 7 (5-13). Median Child-Pugh Score, MELD-Na and PELD scores of the patients were 10 (5-12), 19 (8-34) and 25.4 (8.4-30.7); respectively. Eight patients (34.8%) had Kayser-Fleischer rings on examination. Five patients (21.7%%) presented with acute decompensated Wilson’s disease. Fifteen patients (65.2%) received living donor liver transplantation. Totally, 10 patients (43,4%) had nervous system involvement in the preoperative period. Two patients fully recovered; 2 patients showed partial recovery. On the other hand, 4 patients showed no improvement and 2 patients had progression of their disease in the postoperative period. Conclusions: The results of the present study show that LT is an effective and safe alternative in end-stage liver failure in WD. However, in these patients, nervous system involvement may not improve despite successful LT.