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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The Place of Fetal Liver Transplantation in the Treatment of Inborn Errors of Metabolism,Enzyme levels were not significantly and durably increased in peripheral blood but the quantities of substrates detected in sera and urines were significantly reduced and tissue deposits were stabilized.

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Investigation of Paediatric Liver Disease,al methods including biochemistry, haematology, radiology, electrophysiology and histology. As the clinical presentation of many diseases is similar, it is appropriate to perform a variety of first-line tests, proceeding to more complex investigations only as indicated.

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Hereditary Variation of Liver Enzymes involved with Detoxification and Neurodegenerative Disease, is important in the pathogenesis of these diseases. Potentially such individuals could be identified preclinically and these diseases postponed by reduction in the load of toxin or modification of the relevant enzymic activity.

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,α1-Antitrypsin Deficiency and Liver Disease: Clinical Presentation, Diagnosis and Treatment,rability of the newborn liver are discussed in this review. A plea is made for a trial of infusions of α.-antitrypsin in early infancy, as is used safely but without proven efficacy in the emphysematous PIZZ subject. Prospects of therapy by gene modification are also reviewed.

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