CAH – how controlled and monitored

R J M Ross

University of Sheffield

Summer School 13-16 July 2004
St Anne's College, Oxford University, UK

Patients with congenital adrenal hyperplasia (CAH) have a defect in the production of cortisol from the adrenal gland. Because of this defect the pituitary (by producing ACTH) attempts to drive the adrenal to produce more cortisol but in the process makes the adrenal produce excess androgens. The consequence in the child is virilisation of the female, precocious puberty in the boy, short stature and for the adult infertility, virilisation, and adrenal and testicular tumours. Current formulations of steroids cannot adequately treat this condition because the pituitary produces its ACTH drive at 3-4am and therefore, androgens are produced at this time. There is no consensus on the treatment protocols and physicians use either hydrocortisone, prednisolone or dexamethasone according to the patients needs and the prescription may be given once, twice or thrice daily in a circadian or reverse circadian fashion. As with treatment there is no agreed consensus on monitoring although most endocrinologists will measure androgens and use the measurement of rennin to detect the need for fludrocortisone replacement. In my presentation I will explore current practise and its rationale and give practical guidelines for treating patients with CAH.

The opinions expressed in this paper are those of the speaker and do not necessarily reflect the views of the Society

Revised: 05-Nov-2004