Adrenal Insufficiency

Definition

Definition of Adrenal Insufficiency
Adrenal insufficiency is a condition in which the adrenal glands, located above the kidneys, do not produce adequate amounts of steroid hormones (chemicals produced by the body that regulate organ function), primarily cortisol, but may also include impaired aldosterone production (a mineralocorticoid) which regulates sodium, potassium and water retention. Craving for salt or salty foods due to the urinary losses of sodium is common.

Addison's disease and congenital adrenal hyperplasia can manifest as adrenal insufficiency. If not treated, adrenal insufficiency may result in severe abdominal pains, diarrhea, vomiting, profound muscle weakness and fatigue, depression, extremely low blood pressure (hypotension), weight loss, kidney failure, changes in mood and personality, and shock (adrenal crisis). An adrenal crisis often occurs if the body is subjected to stress, such as an accident, injury, surgery, or severe infection; death may quickly follow.

Symptoms

Symptoms of Adrenal Insufficiency
Symptoms include: hypoglycemia, dehydration, weight loss, and disorientation. Symptoms may also include weakness, tiredness, dizziness, low blood pressure that falls further when standing (orthostatic hypotension), muscle aches, nausea, vomiting, and diarrhea. These problems may develop gradually and insidiously. Addison's can present with tanning of the skin that may be patchy or even all over the body. Characteristic sites of tanning are skin creases (e.g. of the hands) and the inside of the cheek (buccal mucosa). Goitre and vitiligo may also be present.

Causes

Causes of Adrenal Insufficiency
Causes of acute adrenal insufficiency are mainly Waterhouse-Friderichsen syndrome, sudden withdrawal of long-term corticosteroid therapy and stress in patients with underlying chronic adrenal insufficiency. The latter is termed critical illness-related corticosteroid insufficiency.

Adrenal Insufficiency can also be caused when a patient has a Craniopharyngioma which is a benign tumor that can damage the Pituitary gland causing the Adrenal Glands not to function. This would be an example of Secondary Adrenal Insufficiency Syndrome.

Diagnosis

Diagnosis of Adrenal Insufficiency
If the person is in adrenal crisis, the ACTH stimulation test may be given. If not in crisis, cortisol, ACTH, aldosterone, renin, potassium and sodium are tested from a blood sample before the decision is made if the ACTH stimulation test needs to be performed. X-rays or CT of the adrenals may also be done. The best test for adrenal insufficiency of autoimmune origin, representing more than ninety percent of all cases in a Western population, is measurement of 21-hydroxylase autoantibodies.

Treatment

Treatment of Adrenal Insufficiency
Treatment of adrenal insufficiency involves replacing, or substituting, the hormones that the adrenal glands are not making. Cortisol is replaced with a synthetic glucocorticoid such as hydrocortisone, prednisone, or dexamethasone, taken orally once to three times each day, depending on which medication is chosen. If aldosterone is also deficient, it is replaced with oral doses of a mineralocorticoid, called fludrocortisone acetate (Florinef), taken once or twice a day. Doctors usually advise patients receiving aldosterone replacement therapy to increase their salt intake. Because people with secondary adrenal insufficiency normally maintain aldosterone production, they do not require aldosterone replacement therapy. The doses of each medication are adjusted to meet the needs of the individual.

During an Addisonian crisis, low blood pressure, low blood glucose, and high levels of potassium can be life threatening. Standard therapy involves intravenous injections of glucocorticoids and large volumes of intravenous saline solution with dextrose, a type of sugar. This treatment usually brings rapid improvement. When the patient can take fluids and medications by mouth, the amount of glucocorticoids is decreased until a maintenance dose is reached. If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate.

Prognosis

Prognosis of Adrenal Insufficiency
Consult with your doctor.

Prevention

Prevention of Adrenal Insufficiency
People with adrenal insufficiency should always carry identification stating their condition in case of an emergency. A card or medical alert tag should notify emergency health care providers of the need to inject cortisol if the person is found severely injured or unable to answer questions. The card or tag should also include the name and telephone number of the person’s doctor and the name and telephone number of a family member to be notified. The dose of hydrocortisone needed may vary with a person's age or size. For example, a child younger than 2 years of age can receive 25 milligrams (mg), a child between 2 and 8 years of age can receive 50 mg, and a child older than 8 years should receive the adult dose of 100 mg. When traveling, people with adrenal insufficiency should carry a needle, syringe, and an injectable form of cortisol for emergencies.