Akademik Veri Yönetim Sistemi
Turkiye Klinikleri Pediatri, cilt.30, sa.2, ss.138-144, 2021 (Scopus)
Objective: In this study, the importance of indication, preparation, process, results and liver transplant timing were rewieved in patients who underwent liver transplantation due to Tyrosinemia Type 1 (HT-1) and the suspicion of hepatocellular carcinoma (HCC). Material and Methods: All patients who underwent liver transplantations due to HT-1 in our clinic were included in the present study. The demographic findings, preoperative alpha-fetoprotein (AFP) levels and tomography/MRI findings of the patients and live donors were examined. Indications for surgery were questioned. The presence of HCC, tumor grade, extention and burden in the hepatectomy materials were recorded. The postoperative complications, the recurrences of HCC and the survival rates were investigated. Results: A total of 11 patients with Tyrosinemia Type1, 6 of whom were female and 5 were male, with an median age of 8.5±7 years and a median weight of 30.6±23.4 kg were included in this study. The mean AFP value before surgery was 10.682±22.612 U/ml (R: 8-76000). AFP value was within normal limits in only one patient. HCC was not detected in three patients transplanted due to liver failure. In seven (87%) of 8 patients who were transplanted with the diagnosis of HCC, HCC was determined. Multifocal lung metastases were detected in one at the postoperative sixth month. The average follow-up time after transplantation is 4.6±3.4 years and all patients are still alive. Conclusion: Living donor liver transplantation is a curative treatment method in HT-1 patients with liver failure findings or the diagnosis of clinical or radiological HCC. The patients should be carefully selected because of the possible complications and mortality of the liver transplantation, lifelong immunosuppression usage and the fact of requirement for a re-transplantation in a long-term period.