Journal of Inherited Metabolic Disease

, Volume 14, Issue 4, pp 459–477

Fetal and neonatal bile acid synthesis and metabolism — Clinical implications


  • W. F. Balistreri
    • Children's Hospital Research Foundation

DOI: 10.1007/BF01797918

Cite this article as:
Balistreri, W.F. J Inherit Metab Dis (1991) 14: 459. doi:10.1007/BF01797918


It has become apparent that with sophisticated technology we are now able to recognize defective bile acid metabolism in a wide variety of disease states. Recognition of specific aberrations, such as inborn errors in bile acid metabolism manifesting as neonatal cholestasis, offers new opportunities for therapeutic intervention. Future studies should determine the incidence of inborn errors in patients with enigmatic and unexplained liver diseases such as idiopathic neonatal hepatitis.

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© SSIEM and Kluwer Academic Publishers 1991