Titlebook: Journal of Inherited Metabolic Disease; R. A. Harkness,R. J. Pollitt,G. M. Addison Book 1991 Springer Science+Business Media Dordrecht 199

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Paediatric Liver Transplantation: Review of Current Experience,n to fit a child. This has allowed a greater number of children to be grafted, including emergency cases such as fulminant hepatic failure in whom there is insufficient time to wait for a size-matched donor.

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Book 1991the Society for the Study of Inborn Errors ofMetabolism, Birmingham, UK, 1990, which was dedicated to `The Liverand Inherited Metabolic Disease‘ with a half-day workshop on`Screening and Economics‘. .The subjects covered include metabolic functions of the liver, bileacids, alpha-1-antitrypsin defici

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The Role of the Liver in Metabolic Homeostasis: Implications for Inborn Errors of Metabolism,he liver utilizes the energy generated by glucose oxidation to synthesize triglycerides. In the fasted state it utilizes that produced by .-oxidation of fatty acids to synthesize glucose. The mechanisms whereby a number of inborn errors of glycogen metabolism, of gluconeogenesis and of ketogenesis c

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Detoxification Pathways in the Liver, enzymes, implying that variations in the latter may be important predisposing factors for toxicity. Pharmacogenetic defects of xenobiotic biotransformation enzymes, a subclass of inborn errors of metabolism which are manifested only upon drug challenge, introduce marked variation into human populat

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antipsychotic

,Tyrosinaemia Type I — an Update,ial. Presence of a ‘pseudodeficiency’ gene for FAH prevents prenatal diagnosis by enzyme analysis in some families, and this gene also precludes identification of heterozygotes outside tyrosinaemia families. Immunoblot analyses show that acute patients and some chronic patients lack immunoreactive F