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AKTUĞLU ZEYBEK A. Ç., Kiykim E., SOYUÇEN E., CANSEVER M. Ş., Altay S., Zubarioglu T., ...More
PEDIATRICS INTERNATIONAL, vol.57, no.2, pp.281-289, 2015 (SCI-Expanded)
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Publication Type:
Article / Article
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Volume:
57
Issue:
2
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Publication Date:
2015
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Doi Number:
10.1111/ped.12503
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Journal Name:
PEDIATRICS INTERNATIONAL
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Journal Indexes:
Science Citation Index Expanded (SCI-EXPANDED), Scopus
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Page Numbers:
pp.281-289
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Keywords:
hepatocellular carcinoma, liver transplantation, NTBC, Turkey, tyrosinemia type 1, NTBC TREATMENT, HEPATORENAL TYROSINEMIA, NEUROLOGICAL CRISIS, NITISINONE, 2-(2-NITRO-4-TRIFLUOROMETHYLBENZOYL)-1,3-CYCLOHEXANEDIONE, FUMARYLACETOACETATE, INHIBITION, PATHWAY
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Akdeniz University Affiliated:
Yes
Abstract
BackgroundHereditary tyrosinemia type 1(HT1) is a chronic disorder leading to severe hepatic, renal and peripheral nerve damage if left untreated. Despite nitisinone treatment HT1 still carries the risks of hepatocellular carcinoma (HCC) and neuropsychological outcome.