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12 - Adrenal failure

Published online by Cambridge University Press:  15 February 2010

Amanda Ogilvy-Stuart
Affiliation:
University of Cambridge
Paula Midgley
Affiliation:
University of Edinburgh
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Summary

Clinical presentation

  • Hypoglycaemia (see Chapter 2).

  • Collapse (see Chapter 13).

  • Hypotension (see Chapter 14).

  • Ambiguous genitalia (see Chapters 8 and 10).

  • Skin pigmentation (commonly, but not exclusively, scrotal, see Chapter 11).

  • Hyponatraemia (see Chapter 15).

Approach to the problem

  • Assess perfusion.

  • Measure blood pressure.

  • Measure blood glucose.

  • Measure blood sodium and potassium.

  • Is there a history of asphyxia? (Bilateral adrenal haemorrhage.)

  • History of maternal steroid excess (therapeutic administration or Cushing's syndrome).

Differential diagnosis

  • Other causes of hypoglycaemia e.g. hyperinsulinism, or metabolic cause (see Chapters 2 and 3).

  • Other causes of circulatory collapse, e.g. sepsis (see Chapter 13).

  • Addisonian crisis (structural or functional absence of pituitary or adrenal glands).

Primary adrenal failure

  • Congenital adrenal hyperplasia (CAH, including lipoid CAH caused by an abnormality in the steroidogenic acute regulatory (StAR) protein).

  • Adrenal hypoplasia congenita (AHC).

  • Adrenoleucodystrophy (ALD).

Secondary adrenal failure

  • Adrenal suppression following steroid therapy.

  • Adrenocorticotrophic hormone (ACTH) deficiency (see Chapter 7).

  • ACTH resistance (familial glucocorticoid deficiency or FGD).

  • Antenatal steroids administered to the mother.

  • Maternal Cushing's disease/syndrome.

  • Postnatal steroid therapy.

Investigations

Immediate

  • Three random blood cortisol measurements (timing is not important) to look for low cortisol levels. (Plasma cortisol is difficult to interpret on a single sample because of the pulsatile nature of cortisol secretion, therefore taking 2–3 random samples makes it less likely that all will fall at a nadir. Cortisol may also be easier to interpret in the first week of life when cortisol levels are higher.)

  • […]

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Publisher: Cambridge University Press
Print publication year: 2006

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