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Neurocognitive outcome in patients with hypertyrosinemia type I after long-term treatment with NTBC |
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| Authors: | Eva Thimm Renate Richter-Werkle Gudrun Kamp Bettina Molke Diran Herebian Dirk Klee Ertan Mayatepek Ute Spiekerkoetter |
| Institution: | (1) Department of General Pediatrics, Medical Faculty, University Dusseldorf, Moorenstrasse 5, 40225 D?sseldorf, Germany;(2) Medical Faculty, Institute for Diagnostic Radiology, University Dusseldorf, 40225 Dusseldorf, Germany |
| Abstract: | ObjectiveThe implementation of NTBC into treatment of hypertyrosinemia type I (HT I) greatly improved survival by prevention of acute liver failure and hepatocellular carcinoma. However, there are first reports of cognitive impairment in patients with elevated plasma tyrosine concentrations. |
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