8. The Adrenal Gland

  1. Charles G. D. Brook MA, MD, FRCP, FRCPCH1 and
  2. Mehul T. Dattani MD, FRCP, FRCPCH, DCH2,3

Published Online: 23 FEB 2012

DOI: 10.1002/9781119968153.ch8

Handbook of Clinical Pediatric Endocrinology, Second Edition

Handbook of Clinical Pediatric Endocrinology, Second Edition

How to Cite

Brook, C. G. D. and Dattani, M. T. (2012) The Adrenal Gland, in Handbook of Clinical Pediatric Endocrinology, Second Edition, Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781119968153.ch8

Author Information

  1. 1

    University College London, London, UK

  2. 2

    Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, UCL Institute of Child Health, UK

  3. 3

    Great Ormond Street Hospital for Children, London and University College London Hospitals, UK

Publication History

  1. Published Online: 23 FEB 2012
  2. Published Print: 13 APR 2012

ISBN Information

Print ISBN: 9780470657881

Online ISBN: 9781119968153

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Keywords:

  • Addison disease;
  • adrenal neoplasia;
  • adrenal steroids;
  • adrenarche;
  • adrenocorticotropic hormone (ACTH);
  • aldosterone;
  • angiotensin;
  • congenital adrenal hyperplasia;
  • congenital adrenal hypoplasia;
  • cortisol;
  • Cushing syndrome;
  • DAX-1;
  • dehydroepiandrosterone (DHEA);
  • renin

Summary

This chapter describes the development and function of the three layers of the adrenal cortex and the three steroids they produce: cortisol, aldosterone and sex steroids. Understanding the biosynthetic pathways explains the congenital disorders of adrenal function and the chapter simplifies these. Adrenal malignancy remains an intractable problem with a poor prognosis and is often diagnosed late; the chapter addresses this issue. After congenital adrenal hyperplasia, the most common cause of endogenous hypoadrenalism in children is autoimmune adrenalitis. Adrenoleukodystrophy should be considered in any male with hypoadrenalism. Management of adrenal deficiency states is complicated by side-effects which can be avoided, as the chapter makes clear. The most common cause of Cushing syndrome is iatrogenic followed by Cushing disease. The diagnosis and management of Cushing syndrome can be extremely difficult in children and adolescents. Steroid withdrawal and the management of stress in hypoadrenal patients require careful attention to detail.