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

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Kemal Baris Sarici
Tevfik Tolga Sahin
Cengiz Ara
Cemalettin Aydin
Serdar Karakas
Volkan Ince
Fatma Ilknur Varol
Bilge Ozgor
Mukadder Ayse Selimoglu
Sezai Yilmaz

Abstract

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.

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How to Cite
Sarici, K. B., Sahin, T. T., Ara, C., Aydin, C., Karakas, S., Ince, V., Varol, F. I., Ozgor, B., Selimoglu, M. A., & Yilmaz, S. (2023). Comprehensive analysis of the efficacy of liver transplantation in pediatric patients with Wilson’s disease. Annals of Medical Research, 30(1), 1–6. Retrieved from https://annalsmedres.org/index.php/aomr/article/view/4208
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