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primary adrenocortical deficiency
Addison's disease occurs when the adrenal
glands, seated above the kidneys, fail to produce enough of the hormone cortisol and, sometimes, the hormone aldosterone.
There are two forms of Addison's disease, based on the reason for the
disorder. Primary adrenal insufficiency occurs when the adrenal glands
themselves are not working properly; secondary adrenal insufficiency
occurs when the pituitary gland does not produce enough
adrenocorticotropic hormone (ACTH) to adequately stimulate the adrenal
Suspect when fatigue, light headedness is accompanied by weight loss
(weight gain excludes the diagnosis). This suspicion is strengthened
when accompanied by postural hypotension, hyperpigmentation,
hyperkalemia, hyponatremia and hypoglycemia. Some patients may present
with esinophilia. Other symptoms include vomiting and abdominal pain.
Symptoms and signs
Addison's disease (also known as chronic adrenal insufficiency, or
hypocortisolism) is a rare endocrine disorder. It is estimated that it
affects about 1 to 5 in 100,000 people.
Addison's disease progresses slowly, and symptoms may not present or be
noticed until some stressful illness or situation occurs. Common
- chronic fatigue that gradually worsens
- muscle weakness
- loss of appetite
- weight loss
- low blood pressure that falls further when standing (orthostatic
- areas of hyperpigmentation (darkened skin)
- craving for salt and salty foods
- hypoglycaemia (worse in children)
- for women, menstrual periods that become irregular or that stop
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Once suspected investigations should be urgent, a blood sample is
taken for measurement of plasma cortisol and serum electrolytes.
Hydrocortisone should be given IM.
Serum cortisol taken at 8:00am < 5ug/dL [SI:140nmol/L] (some
authorities say < 3ug/dL [SI: 86nmol/L]) confirms the diagnosis. A
result of > 20ug/dL [SI: 550nmol/L] excludes the diagnosis in an
Between these values if the condition is still suspected a rapid ACTH
stimulation test should be performed. When impaired a long ACTH
stimulation test should be performed to differentiate primary from
A high plasma ACTH level with a low or normal cortisol level confirms
primary adrenocortical insufficiency.
Treatment for Addison's disease basically involves replacing the missing
cortisol and, if necessary, providing replacement therapy for the
missing aldosterone. Caution must be exercised when the person with
Addison's disease has surgery or becomes pregnant.
An illness or accident can aggravate the adrenal problems and cause an
Addisonian crisis in which the symptoms include:
- sudden penetrating pain in the legs, lower back or abdomen
- severe vomiting and diarrhea, resulting in dehydration
- low blood pressure
- loss of consciousness
Untreated, an Addisonian crisis can be fatal.