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Review
Update on adrenal steroid hormone biosynthesis and clinical implications
  1. Irina-Alexandra Bacila,
  2. Charlotte Elder,
  3. Nils Krone
  1. Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK
  1. Correspondence to Dr Nils Krone, Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield S10 2TH, UK; n.krone{at}sheffield.ac.uk

Abstract

Steroid biosynthesis is a complex process in which cholesterol is converted to steroid hormones with the involvement of multiple enzymes and cofactors. Inborn conditions affecting adrenal steroidogenesis are relatively common in paediatric practice and have serious implications on patient mortality and morbidity. This paper provides an overview of novel insights into human adrenal steroid biosynthesis. Inborn errors of steroidogenesis associated with congenital adrenal hyperplasia are discussed, with a particular focus on the pathophysiology and clinical features of 21-hydroxylase deficiency. The final section of the review presents more recent findings and clinical implications of adrenal-specific androgen biosynthesis.

  • adrenal steroid biosynthesis
  • steroid hormones
  • congenital adrenal hyperplasia
  • 21-hydroxylase deficiency

http://dx.doi.org/10.1136/archdischild-2017-313873

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    Footnotes

    • Contributors I-AB and NK made substantial contributions to the original concept and design of the manuscript. I-AB and NK produced the first draft of the manuscript. CE revised critically the first draft of the manuscript. I-AB, CE and NK all approved of the submitted version of the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Commissioned; externally peer reviewed.

    • Patient consent for publication Not required.