Addison disease, first described in 1849 by Dr T Addison, is a rare condition which is due to the destruction of the adrenal cortex leading to a deficiency or absence of cortisol and other adrenal hormones. The symptoms are due to the failure of the production of cortical hormones, which are responsible for the rate at which sugar is made available to the tissues of the body, and the amount of salt lost from the body. The deficiency may have a number of causes, such as auto-immunity, tuberculosis, or it may be spontaneous.

The symptoms of chronic adrenal failure have a very gradual onset, so that the condition is difficult to appreciate. Lack of energy, malaise, or giddiness when standing up, are almost always present. There is usually an increase in skin pigmentation. Often, the development of an intercurrent illness decompensates the condition which suddenly deteriorates into circulatory collapse, hypoglycaemia and, if untreated, death.

The condition, once diagnosed, can be successfully treated with replacement hormones. Extra steroid hormones will be necessary to cover periods of intercurrent illnesses and surgical stress.

Inheritance patterns
The condition may occasionally be genetic: for example, there is an X-linked form of the disease which is related to adrenoleukodystrophy.

Prenatal diagnosis
None

Medical text written November 1991 by Contact a Family. Approved by Professor Michael Patton, Professor of Medical Genetics, St George's Hospital Medical School, London, UK and Dr J E Wraith, Consultant Paediatrician, Royal Manchester Children's Hospital, Manchester, UK. Last updated September 2005 by Dr R Stanhope, Consultant Paediatric Endocrinologist, Great Ormond Street Hospital, London, UK.

Further Online Resources
Medical texts in The Contact a Family Directory are designed to give a short, clear description of specific conditions and rare disorders. More extensive information on this condition can be found on a range of reliable, validated web sites and links to them are included in the CD-ROM version of this Directory. Further information on these resources can be found in our Medical Information on the Internet article.

ADDISON'S DISEASE SELF HELP GROUP

Addison's Disease Self Help Group
21 George Road
Guildford
GU1 4NP
e-mail: info@addisons.org.uk
Web: http://www.addisons.org.uk

The Group is a National Registered Charity No. 1106791 started in 1984. It offers contact with others with same condition. It publishes a bi-monthly newsletter and has information available, details on request. Please send SAE for information. The Group represents 14 affected children and 800 adults.

Group details last updated May 2007.