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Addison's disease

Addison's disease (also known as chronic adrenal insufficiency, or hypocortisolism) is a rare endocrine disorder, first described by British physician Thomas Addison. more...

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It is estimated that it affects about 1 to 2 in 100,000 people.

It occurs when the adrenal glands, seated above the kidneys, fail to produce enough of the hormone cortisol and, sometimes, the hormone aldosterone.

Addison's disease refers specifically to primary adrenal insufficiency, in which the adrenal glands themselves malfunction; secondary adrenal insufficiency occurs when the pituitary gland does not produce enough adrenocorticotropic hormone (ACTH) to adequately stimulate the adrenal glands.

Signs and symptoms

Early signs

Addison's disease progresses slowly, and symptoms may not present or be noticed until some stressful illness or situation occurs. Common symptoms are:

  • chronic fatigue that gradually worsens
  • muscle weakness
  • loss of appetite
  • weight loss
  • nausea/vomiting
  • diarrhea
  • low blood pressure that falls further when standing (orthostatic hypotension)
  • areas of hyperpigmentation (darkened skin), known as melasma suprarenale.
  • irritability
  • depression
  • craving for salt and salty foods
  • hypoglycemia (worse in children)
  • for women, menstrual periods that become irregular or cease
  • tetany (particularly after drinking milk) due to phosphate excess
  • numbness of the extremities, sometimes with paralysis, due to potassium excess

Addisonian crisis

An illness or accident can aggravate the adrenal problems and cause an Addisonian crisis in which the symptoms include:

  • brown coating on tongue and teeth due to iron loss hemolysis
  • sudden penetrating pain in the legs, lower back or abdomen
  • severe vomiting and diarrhea, resulting in dehydration
  • low blood pressure
  • loss of consciousness
  • hypoglycemia

Untreated, an Addisonian crisis can be fatal. It is a medical emergency.

Diagnosis

In suspected cases of Addison's disease, one needs to demonstrate that adrenal hormone levels are low after appropriate stimulation with synthetic pituitary hormone.

Once demonstrated, the cause of adrenal failure needs to be elucidated. The most common cause is autoimmune, and can be tested for with an assay for 21-hydroxylase antibodies. If there are no antibodies present, infectious or genetic causes should be sought. This may include imaging of the adrenal glands, tests for tuberculosis or HIV infection, and searching for metastatic cancer.

Pathophysiology

Eighty to ninety percent of cases of Addison's disease are said to be due to autoantibodies directed against adrenal cells containing 21-hydroxylase, an enzyme involved in the production of cortisol and aldosterone.

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Myths & facts...about Addison's disease
From Nursing, 8/1/02 by McConnell, Edwina A

MYTH: Disorders of the adrenal glands cause both primary and secondary adrenal insufficiency.

FACT: Disorders of the adrenal glands cause primary adrenal insufficiency.

MYTH: Most cases of Addison's disease are caused by infection or trauma.

FACT: About 70% of Addison's disease cases are caused by autoimmune disorders. In primary Addison's disease, this leads to gradual destruction of the adrenal cortex. Secondary Addison's disease is caused by inadequate secretion of adrenocorticotropic hormone (ACTH) from the pituitary gland.

MYTH: For most patients, symptom onset is sudden.

FACT: Clinical manifestations of Addison's disease develop gradually. Common signs and symptoms include chronic, escalating fatigue; muscle weakness; loss of appetite; and weight loss. Patients may also experience abdominal pain, nausea, vomiting, diarrhea, decreased resistance to infection or stress, low blood pressure with orthostatic changes, amenorrhea or irregular menstrual periods, decreased libido, decreased pubic and axillary hair, irritability, depression, hyperpigmentation, a craving for salty foods, hyponatremia, and hyperkalemia.

MYTH: X-ray studies are the most specific test to diagnose Addison's disease.

FACT: Stimulation with ACTH is the most specific diagnostic test for Addison's disease, although imaging studies and X-rays of the adrenal and pituitary glands can help determine a primary or secondary cause. In the rapid ACTH test, the patient receives an IN. injection of a synthetic form of ACTH. His blood cortisol level is measured before the injection and 30 to 60 minutes after the injection. Normally, urine and blood cortisol levels increase; patients with any form of adrenal insufficiency won't respond or barely respond.

MYTH: A patient with Addison's disease relies on parenteral medication to maintain stability.

FACT: The patient will take oral hydrocortisone for maintenance therapy. He may need to raise the dosage in certain conditions, such as mild respiratory infection or emotional stress.

EDWINA A. McCONNELL, RN, PHD, FRCNA

Edwina A McConnell is an independent nurse-consultant in Gorham, Me. Selected references for this article are available on request.

Copyright Springhouse Corporation Aug 2002
Provided by ProQuest Information and Learning Company. All rights Reserved

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