Islets of Hope disorders associated with diabetes
Edited for content by Lahle Wolfe; NIH Publication No. 04–3054, June 2004
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...that It is important for patients receiving aldosterone replacement therapy to talk with their doctor about the need to increase their salt intake.
Patients with secondary adrenal insufficiency normally maintain aldosterone production and therefore, do not need aldosterone replacement therapy.
A person who has adrenal insufficiency should always carry identification stating his or her condition in case of an emergency. The card should alert emergency personnel about the need to inject 100 mg of cortisol if its bearer is found severely injured or unable to answer questions. The card should also include the doctor's name and telephone number and the name and telephone number of the nearest relative to be notified. When traveling, a needle, syringe, and an injectable form of cortisol should be carried for emergencies. A person with Addison's disease also should know how to increase medication during periods of stress or mild upper respiratory infections. Immediate medical attention is needed when severe infections, vomiting, or diarrhea occur. These conditions can precipitate an addisonian crisis. A patient who is vomiting may require injections of hydrocortisone.
People with medical problems like Addison's and diabetes should wear a descriptive warning bracelet or neck chain to alert emergency personnel. You can also print out emergency medical information forms from IOH's publications section.
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What is Addison's Disease?
Addison's disease is a disorder in which a person's adrenal glands (everyone has two) do not make enough cortisol and, in some cases the hormone aldosterone. When cortisol levels become too low various symptoms occur and untreated Addison's Disease can be fatal. Treatment includes oral medication to replace vital hormones. When pills cannot be swallowed injections are necessary.
Adrenal insufficiency can be difficult to diagnose in early stages. A doctor will take note of medical history, look for dark tanning of the skin, and run a series of laboratory tests to check the levels of cortisol. Your doctor may also wish to x-ray your adrenal and pituitary glands to help in determining if a person has Addison’s disease.
Addison's disease is an endocrine or hormonal disorder that occurs in all age groups and afflicts men and women equally. The disease is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and sometimes darkening of the skin in both exposed and nonexposed parts of the body.
Cortisol - Cortisol is normally produced by the adrenal glands, located just above the kidneys. It belongs to a class of hormones called glucocorticoids, which affect almost every organ and tissue in the body. Scientists think that cortisol has possibly hundreds of effects in the body. Cortisol's most important job is to help the body respond to stress. Among its other vital tasks, cortisol
Because cortisol is so vital to health, the amount of cortisol produced by the adrenals is precisely balanced. Like many other hormones, cortisol is regulated by the brain's hypothalamus and the pituitary gland, a bean-sized organ at the base of the brain. First, the hypothalamus sends "releasing hormones" to the pituitary gland. The pituitary responds by secreting hormones that regulate growth and thyroid and adrenal function, and sex hormones such as estrogen and testosterone. One of the pituitary's main functions is to secrete ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands. When the adrenals receive the pituitary's signal in the form of ACTH, they respond by producing cortisol. Completing the cycle, cortisol then signals the pituitary to lower secretion of ACTH.
Aldosterone - Aldosterone belongs to a class of hormones called mineralocorticoids, also produced by the adrenal glands. It helps maintain blood pressure and water and salt balance in the body by helping the kidney retain sodium and excrete potassium. When aldosterone production falls too low, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop.
Causes of Addison's Disease
Failure to produce adequate levels of cortisol can occur for different reasons. The problem may be due to a disorder of the adrenal glands themselves (primary adrenal insufficiency) or to inadequate secretion of ACTH by the pituitary gland (secondary adrenal insufficiency).
Primary Adrenal Insufficiency
Addison's disease affects about 1 in 100,000 people. Most cases are caused by the gradual destruction of the adrenal cortex, the outer layer of the adrenal glands, by the body's own immune system. About 70 percent of reported cases of Addison's disease are caused by autoimmune disorders, in which the immune system makes antibodies that attack the body's own tissues or organs and slowly destroy them. Adrenal insufficiency occurs when at least 90 percent of the adrenal cortex has been destroyed. As a result, often both glucocorticoid (cortisol) and mineralocorticoid (aldostertone) hormones are lacking. Sometimes only the adrenal gland is affected, as in idiopathic adrenal insufficiency; sometimes other glands also are affected, as in the polyendocrine deficiency syndrome.
Polyendocrine Deficiency Syndrome - The polyendocrine deficiency syndrome is classified into two separate forms, referred to as type I and type II.
Type I occurs in children, and adrenal insufficiency may be accompanied by
Type II, often called Schmidt's syndrome, usually afflicts young adults. Features of type II may include
Scientists think that the polyendocrine deficiency syndrome is inherited because frequently more than one family member tends to have one or more endocrine deficiencies.
Tuberculosis - Tuberculosis (TB), an infection which can destroy the adrenal glands, accounts for about 20 percent of cases of primary adrenal insufficiency in developed countries. When adrenal insufficiency was first identified by Dr. Thomas Addison in 1849, TB was found at autopsy in 70 to 90 percent of cases. As the treatment for TB improved, however, the incidence of adrenal insufficiency due to TB of the adrenal glands has greatly decreased.
Other Causes - Less common causes of primary adrenal insufficiency are
Secondary Adrenal Insufficiency
This form of adrenal insufficiency is much more common than primary adrenal insufficiency and can be traced to a lack of ACTH. Without ACTH to stimulate the adrenals, the adrenal glands' production of cortisol drops, but not aldosterone. A temporary form of secondary adrenal insufficiency may occur when a person who has been receiving a glucocorticoid hormone such as prednisone for a long time abruptly stops or interrupts taking the medication. Glucocorticoid hormones, which are often used to treat inflammatory illnesses like rheumatoid arthritis, asthma, or ulcerative colitis, block the release of both corticotropin-releasing hormone (CRH) and ACTH. Normally, CRH instructs the pituitary gland to release ACTH. If CRH levels drop, the pituitary is not stimulated to release ACTH, and the adrenals then fail to secrete sufficient levels of cortisol.
Another cause of secondary adrenal insufficiency is the surgical removal of benign, or noncancerous, ACTH-producing tumors of the pituitary gland (Cushing's disease). In this case, the source of ACTH is suddenly removed, and replacement hormone must be taken until normal ACTH and cortisol production resumes.
Less commonly, adrenal insufficiency occurs when the pituitary gland either decreases in size or stops producing ACTH. These events can result from
Symptoms of Addison's Disease
Addison’s has many symptoms and are not always present in everyone with the disease because they can appear gradually. Addison’s can cause irritability and depression and due to loss of salt loss, a craving for salty foods also is common. Hypoglycemia, or low blood glucose, is more severe in children than in adults. In women, menstrual periods may become irregular or stop. Addison’s can also appear with chronic, worsening fatigue, muscle weakness, loss of appetite, weight loss, low blood pressure (causing dizziness or fainting), hyperpgimentation (darkening of exposed and non-exposed skin including lips and mucous membranes). In half of Addison’s patients there may also be nausea, vomiting, and diarrhea.
In summary, the symptoms of adrenal insufficiency are:
About 50 percent of the time, one will notice
Other symptoms include
Addison's disease can cause irritability and depression. Because of salt loss, a craving for salty foods also is common. Hypoglycemia, or low blood glucose, is more severe in children than in adults. In women, menstrual periods may become irregular or stop.
Because the symptoms progress slowly, they are usually ignored until a stressful event like an illness or an accident causes them to become worse. This is called an addisonian crisis, or acute adrenal insufficiency. In most cases, symptoms are severe enough that patients seek medical treatment before a crisis occurs. However, in about 25 percent of patients, symptoms first appear during an addisonian crisis.
Symptoms of an addisonian crisis include
Prevalence of Addison's Disease
Addison's disease affects about 1 in 100,000 people. It is sometimes associated with certain types of diabetes. About 70% of reported Addison’s is an autoimmune disease and that slowly destroys the adrenal cortex, the outer layer of the adrenal glands.
Diagnostic Tests for Addison's Disease
In its early stages, adrenal insufficiency can be difficult to diagnose. A review of a patient's medical history based on the symptoms, especially the dark tanning of the skin, will lead a doctor to suspect Addison's disease.
A diagnosis of Addison's disease is made by laboratory tests. The aim of these tests is first to determine whether levels of cortisol are insufficient and then to establish the cause. X-ray exams of the adrenal and pituitary glands also are useful in helping to establish the cause.
ACTH Stimulation Test
This is the most specific test for diagnosing Addison's disease. In this test, blood cortisol, urine cortisol, or both are measured before and after a synthetic form of ACTH is given by injection. In the so-called short, or rapid, ACTH test, measurement of cortisol in blood is repeated 30 to 60 minutes after an intravenous ACTH injection. The normal response after an injection of ACTH is a rise in blood and urine cortisol levels. Patients with either form of adrenal insufficiency respond poorly or do not respond at all.
CRH Stimulation Test
When the response to the short ACTH test is abnormal, a "long" CRH stimulation test is required to determine the cause of adrenal insufficiency. In this test, synthetic CRH is injected intravenously and blood cortisol is measured before and 30, 60, 90, and 120 minutes after the injection. Patients with primary adrenal insufficiency have high ACTHs but do not produce cortisol. Patients with secondary adrenal insufficiency have deficient cortisol responses but absent or delayed ACTH responses. Absent ACTH response points to the pituitary as the cause; a delayed ACTH response points to the hypothalamus as the cause.
In patients suspected of having an addisonian crisis, the doctor must begin treatment with injections of salt, fluids, and glucocorticoid hormones immediately. Although a reliable diagnosis is not possible while the patient is being treated for the crisis, measurement of blood ACTH and cortisol during the crisis and before glucocorticoids are given is enough to make the diagnosis. Once the crisis is controlled and medication has been stopped, the doctor will delay further testing for up to 1 month to obtain an accurate diagnosis.
Once a diagnosis of primary adrenal insufficiency has been made, x-ray exams of the abdomen may be taken to see if the adrenals have any signs of calcium deposits. Calcium deposits may indicate TB. A tuberculin skin test also may be used.
If secondary adrenal insufficiency is the cause, doctors may use different imaging tools to reveal the size and shape of the pituitary gland. The most common is the CT scan, which produces a series of x-ray pictures giving a cross-sectional image of a body part. The function of the pituitary and its ability to produce other hormones also are tested.
Medical Concerns for Addison's Patients
It is critical that all persons with Addison’s wear a medical ID alert because those with chronic adrenal insufficiency will need special care in treating medical emergencies especially when surgery is required. Carry a medical card indicating to emergency personnel that you may require an injection of 100 mg of cortisol if you are severely injured or unable to communicate.
Women who have Addison’s will also need special medical care during pregnancy and delivery. It is important that you discuss pregnancy with your physician if you are thinking of becoming pregnant, or if you find out that you are.
Special care is also required during severe infections, or when vomiting or diarrhea are present in order to avoid a serious situation called addisonian crisis. A person unable to take oral medications may require injections of hydrocortisone.
Symptoms of an addisonian crisis, which left untreated, can be fatal, include:
Treatment for Addison's Disease
Treatment of Addison's disease includes replacing, or substituting, hormones that the adrenal glands stopped producing. Cortisol needs to be taken orally once or twice a day along with hydrocortisone tablets (a synthetic glucocorticoid). If the hormone aldosterone is also deficient, oral doses of a mineralocorticoid called fludrocortisone acetate (Florinef), needs to be taken once a day.
Patients receiving aldosterone replacement therapy are usually advised by a doctor to increase their salt intake. Because patients with secondary adrenal insufficiency normally maintain aldosterone production, they do not require aldosterone replacement therapy. The doses of each of these medications are adjusted to meet the needs of individual patients.
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 hydrocortisone, saline (salt water), and dextrose (sugar). This treatment usually brings rapid improvement. When the patient can take fluids and medications by mouth, the amount of hydrocortisone is decreased until a maintenance dose is achieved. If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate.
Page Updated 08/08/2006