Journal of Inherited Metabolic Disease

1. Front Matter

   Pages I-406

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2. The Role of the Liver in Metabolic Homeostasis: Implications for Inborn Errors of Metabolism

   • G. van den Berghe

   Pages 407-420

3. Detoxification Pathways in the Liver

   • D. M. Grant

   Pages 421-430

4. Hereditary Variation of Liver Enzymes involved with Detoxification and Neurodegenerative Disease

   • A. C. Williams, G. B. Steventon, S. Sturman, R. H. Waring

   Pages 431-435

5. Interrelationships of Liver and Brain with Special Reference to Reye Syndrome

   • J. K. Brown, H. Imam

   Pages 436-458

6. Fetal and Neonatal Bile Acid Synthesis and Metabolism — Clinical Implications

   • W. F. Balistreri

   Pages 459-477

7. Inborn Errors of Bile Acid Metabolism

   • P. T. Clayton

   Pages 478-496

8. α1-Antitrypsin Deficiency and Liver Disease: Clinical Presentation, Diagnosis and Treatment

   • M. Hussain, G. Mieli-Vergani, A. P. Mowat

   Pages 497-511

9. α ₁-Antitrypsin Deficiency and Liver Disease

   • P. Birrer, N. G. McElvaney, L. M. Chang-Stroman, R. G. Crystal

   Pages 512-525

10. Clinical Presentation of Metabolic Liver Disease

    • M. Odievre

    Pages 526-530

11. Investigation of Paediatric Liver Disease

    • D. Kelly, A. Green

    Pages 531-537

12. The Role of Histochemical Investigations in Metabolic Disorders Affecting the Liver

    • B. D. Lake

    Pages 538-545

13. Techniques for Studying Hepatic Metabolism in vivo

    • J. V. Leonard, G. N. Thompson

    Pages 546-553

14. Tyrosinaemia Type I — an Update

    • E. A. Kvittingen

    Pages 554-562

15. Investigation of the Molecular Basis of the Genetic Deficiency of UDP-Glucuronosyl-transferase in Crigler—Najjar Syndrome

    • K. J. Robertson, D. Clarke, L. Sutherland, R. Wooster, M. W. H. Coughtrie, B. Burchell

    Pages 563-579

16. Niemann-Pick Disease Type C: An Update

    • M. T. Vanier, P. Pentchev, C. Rodriguez-Lafrasse, R. Rousson

    Pages 580-595

17. Paediatric Liver Transplantation: Review of Current Experience

    • J. A. C. Buckels

    Pages 596-603

18. Treatment of Inherited Metabolic Disorders by Liver Transplantation

    • M. Burdelski, B. Rodeck, K. Latta, K. Latta, J. Brodehl, B. Ringe et al.

    Pages 604-618

19. The Place of Fetal Liver Transplantation in the Treatment of Inborn Errors of Metabolism

    • J.-L. Touraine

    Pages 619-626

20. Screening and Economics

    • J. A. Stilwell

    Pages 627-632

The articles in Issue 4 of Journal of Inherited Metabolic Disease, Vol. 14 (1991) contain the main lectures presented at the 28th Annual Symposium of the Society for the Study of Inborn Errors of Metabolism, Birmingham, UK, 1990, which was dedicated to `The Liver and Inherited Metabolic Disease' with a half-day workshop on `Screening and Economics'.
The subjects covered include metabolic functions of the liver, bile acids, alpha-1-antitrypsin deficiency, tyrosinaemia type I, Crigler-Najjar disease type I and Niemann-Pick disease type C, providing updates on a wide range of metabolic disorders and illustrating the importance of the complementary contributions from professionals in different disciplines. Also covered in detail is the exciting potential of liver transplantation as treatment for several inborn errors of metabolism.
This state-of-the-art review will be of interest to clinicians and research workers alike.

• enzymes
• liver
• liver disease
• liver transplantation
• metabolism
• transplantation

• London, UK

  R. A. Harkness

• Sheffield, UK

  R. J. Pollitt

• Manchester, UK

  G. M. Addison

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