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

Authors

  • Kemal Baris Sarici Inonu University, Liver Transplantation Institute, Department of General Surgery, Malatya, Türkiye
  • Tevfik Tolga Sahin Inonu University, Liver Transplantation Institute, Department of General Surgery, Malatya, TürkiyeTevfik Tolga
  • Cengiz Ara Inonu University, Liver Transplantation Institute, Department of General Surgery, Malatya, Türkiye
  • Cemalettin Aydin Inonu University, Liver Transplantation Institute, Department of General Surgery, Malatya, Türkiye
  • Serdar Karakas Inonu University, Liver Transplantation Institute, Department of General Surgery, Malatya, Türkiye
  • Volkan Ince Inonu University, Liver Transplantation Institute, Department of General Surgery, Malatya, Türkiye
  • Fatma Ilknur Varol Inonu University, Faculty of Medicine, Departments of Pediatrics Gastroenterology, Hepatology and Nutrition, Malatya, Türkiye
  • Bilge Ozgor Inonu University, Faculty of Medicine, Department of Pediatric Neurology, Malatya, Türkiye
  • Mukadder Ayse Selimoglu Inonu University, Faculty of Medicine, Departments of Pediatric Gastroenterology, Hepatology and Nutrition, Malatya, Türkiye
  • Sezai Yilmaz Inonu University, Liver Transplantation Institute, Department of General Surgery, Malatya, Türkiye

Keywords:

Wilson’s disease, Pediatric liver transplantation, Neurologic symptoms

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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Published

2023-01-21

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Original Articles

How to Cite

1.
Comprehensive analysis of the efficacy of liver transplantation in pediatric patients with Wilson’s disease. Ann Med Res . 2023;30(1):1-6. Accessed February 15, 2025. https://annalsmedres.org/index.php/aomr/article/view/4208

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