Difference between revisions of "Addison's disease" - New World Encyclopedia

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== Treatment ==
 
== Treatment ==
Treatment for Addison's disease involves replacing the missing cortisol and, if necessary, [[fludrocortisone]] as replacement for the missing aldosterone. Caution must be exercised when the person with Addison's disease has [[surgery]] or becomes [[pregnant]].
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Treatment for Addison's disease involves replacing the lacking cortisol and, if necessary, [[fludrocortisone]] as a replacement for the missing aldosterone. Caution must be exercised when a person with Addison's disease faces a stressful situation, such as surgery or [[pregnancy]].
  
 
==Famous patients==
 
==Famous patients==

Revision as of 02:33, 23 May 2006

Addison's disease is the hyposecretion of all adrenal steroid hormones. It usually follows the autoimmune destruction (immune system creates antibodies against the body's own tissues) of the adrenal cortex. Inherited disorders in which defective enzymes for adrenal steroid production are produced are often marked by excess androgen (sex steroid) secretion. This is because substrates that cannot be made into cortisol or aldosterone are converted to androgens (sex steroids).

Hyposecretion pathologies are far less common than Cushing's syndrome.

Addison's disease
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ICD-10 E27.1-E27.2
ICD-O: {{{ICDO}}}
ICD-9 255.4
OMIM {{{OMIM}}}
MedlinePlus {{{MedlinePlus}}}
eMedicine {{{eMedicineSubj}}}/{{{eMedicineTopic}}}
DiseasesDB {{{DiseasesDB}}}

Addison's disease, also known as chronic adrenal insufficiency, or hypocortisolism, is a rare endocrine disorder. The disease was first described by British physician Thomas Addison in his 1855 publication: On the Constitutional and Local Effects of Disease of the Suprarenal Capsules.

It is estimated that the disease affects about 1 to 2 per 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 anterior 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 physical 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, or low blood glucose levels, which are worse in children
  • For women, menstrual periods that become irregular or cease
  • Tetany (particularly after drinking milk) due to excess phosphate
  • Numbness of the extremities, sometimes with paralysis, due to potassium excess
  • Eosinophilia (an increased number of eosinophils, which are white blood cells that play a major role in allergeric reactions)
  • Polyuria (increased urine output)

Addisonian crisis

An illness or accident can aggravate the adrenal problems and cause an Addisonian crisis; however, the most common cause is discontinuing corticosteroid therapy without tapering the dose. Symptoms that may occur include:

  • Brown coating on tongue and teeth due to hemolyis, or iron loss
  • Sudden penetrating pain in the legs, lower back, and/ or abdomen
  • Severe vomiting and diarrhea, resulting in dehydration
  • Low blood pressure
  • Loss of consciousness
  • Hypoglycemia

If left untreated, an Addisonian crisis can be fatal. It is considered a medical emergency.

Diagnosis

In suspected cases of Addison's disease, low adrenal hormone levels must be demonstated after appropriate stimulation with synthetic pituitary hormone.

Once demonstrated, the cause of adrenal failure needs to be elucidated. The most common cause of Addison's disease is autoimmune and occurs when the body's immune system creates antibodies against the body's own tissues. It can be tested for with an assay for 21-hydroxylase antibodies. If no antibodies are present, infectious or genetic causes ought to be sought. These may include imaging of the adrenal glands, tests for tuberculosis or HIV infection, and searching for metastatic cancer.

Etiology

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.

The remainder of cases are due to tuberculosis, HIV, sarcoidosis (a disease of unknown origin characterized by the formation of granulomatous lesions that appear especially in the liver, lungs, skin, and lymph nodes; also called sarcoid), amyloidosis (a disorder marked by the deposition of amyloid in various organs and tissues of the body; may be associated with a chronic disease such as rheumatoid arthritis, tuberculosis, or multiple myeloma), hemochromatosis, metastatic cancer to the adrenal glands, adrenal hemorrhage (profuse bleeding), Waterhouse-Friderichsen syndrome (massive, usually bilateral, hemorrhage into the adrenal glands caused by fulminant meningococcemia), and congenital adrenal hyperplasia.

Addison's disease can be an expression of an autoimmune polyendocrine syndrome, or APS, when autoimmune reactions against other organs are also present. In APS type 1, 70% suffer from Addison's disease, while in type 2, 100% do.

Through these syndromes, Addison's disease is associated with hypothyroidism, diabetes mellitus (type 1), vitiligo, alopecia (baldness), and celiac disease.

Treatment

Treatment for Addison's disease involves replacing the lacking cortisol and, if necessary, fludrocortisone as a replacement for the missing aldosterone. Caution must be exercised when a person with Addison's disease faces a stressful situation, such as surgery or pregnancy.

Famous patients

A famous sufferer of Addison's Disease was President John F. Kennedy. Jane Austen is thought to have been another, although the disease had not been described during her lifetime.

In animals

The condition is rare in dogs, but it has been diagnosed in Bearded Collie and Chihuahua, in rare cases also in Standard Poodles. In dogs it is inherited via the female line - possibly due to autoimmune antibodies in the milk.

The first sign of Addison's Disease is that the dog urinates much longer than normally. Urine is very light and pressure while urinating is lower than normal. When dog loses its appetite and changes its temperament, a blood test must be taken as soon as possible.

Addison's disease is treated with Prednisolone and Florinef. The dog must have its medicine twice a day after a meal. The amounts of the medicine depends on the weight of the dog. Even if the dog feels good, it's absolutely necessary to take blood samples after every 3-4 months, to make sure the dog's condition is stable. An alternative to oral Florinef is a monthly injection called Percorten V. The active ingredient is DOCP (Desoxycorticosterone Pivalate.)

One of the medicines makes the dog drink more and the other one makes it urinate more. It is absolutely vital to give to the ill dog fresh water all the time.

The owner of the ill dog, should measure dog's pulse while it is relaxed. In stressful situations it is necessary to know when dog's heart is pumping too fast and it needs to be relaxed. If the owner knows about upcoming stressful situations (show, travelling etc.) he/she may give a larger amount of medicine.

The stress level of the ill dog must be as low as possible (both positive and negative stress). For example agility trainings, shows, guests etc. must cause stress as little as possible.

Reference

  • Addison T. On the constitutional and local effects of disease of the suprarenal capsules. London: P. Highley, 1855. An exact reprint with colour images is available via the external link below.

See also

External Links

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