| Autor: |
Sarici, Kemal Baris, Sahin, Tevfik Tolga, Ara, Cengiz, Aydin, Cemalettin, Karakas, Serdar, Ince, Volkan, Varol, Fatma Ilknur, Ozgor, Bilge, Selimoglu, Mukadder Ayse, Yilmaz, Sezai |
| Předmět: |
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| Zdroj: |
Annals of Medical of Research; 2023, Vol. 30 Issue 1, p1-6, 6p |
| Abstrakt: |
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. Conclusion: 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. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
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