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Addison's disease
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Addison's disease
Addison's disease, also known as primary adrenal insufficiency, is a rare long-term endocrine disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of the cells of the adrenal glands (adrenal cortex), causing adrenal insufficiency. Symptoms generally develop slowly and insidiously and may include abdominal pain and gastrointestinal abnormalities, weakness, and weight loss. Darkening of the skin in certain areas may also occur. Under certain circumstances, an adrenal crisis may occur with low blood pressure, vomiting, lower back pain, and loss of consciousness. Mood changes may also occur. Rapid onset of symptoms indicates acute adrenal failure, which is a clinical emergency. An adrenal crisis can be triggered by stress, such as from an injury, surgery, or infection.
Addison's disease arises when the adrenal gland does not produce sufficient amounts of the steroid hormones cortisol and (sometimes) aldosterone. It is an autoimmune disease which affects some genetically predisposed people in whom the body's own immune system has started to target the adrenal glands. In many adult cases it is unclear what has triggered the onset of this disease, though it sometimes follows tuberculosis. Causes can include certain medications, sepsis, and bleeding into both adrenal glands. Addison's disease is generally diagnosed by blood tests, urine tests, and medical imaging.
Treatment involves replacing the absent or low hormones. This involves taking a synthetic corticosteroid, such as hydrocortisone or fludrocortisone. These medications are typically taken orally. Lifelong, continuous steroid replacement therapy is required, with regular follow-up treatment and monitoring for other health problems which may occur. A high-salt diet may also be useful in some people. If symptoms worsen, an injection of corticosteroid is recommended (people need to carry a dose with them at all times). Often, large amounts of intravenous fluids with the sugar dextrose are also required. With appropriate treatment, the overall outcome is generally favorable, and most people are able to lead a reasonably normal life. Without treatment, an adrenal crisis can result in death.
Addison's disease affects about 9 to 14 per 100,000 people in the developed world. It occurs most frequently in middle-aged females. The disease is named after Thomas Addison, a graduate of the University of Edinburgh Medical School, who first described the condition in 1855.
The symptoms of Addison's disease can develop over several months and resemble other medical conditions. Most common symptoms are caused by low levels of hormones that would normally be produced by the adrenal glands. Low blood cortisol can cause a variety of symptoms, including fatigue, malaise, muscle and joint pain, reduced appetite, weight loss, and increased sensitivity to cold. Gastrointestinal symptoms such as nausea, abdominal pain, and vomiting are particularly common. Low aldosterone can cause affected people to crave salty foods, as well as develop low blood pressure that leads to dizziness upon standing. In women, low dehydroepiandrosterone (DHEA) can result in dry and itchy skin, loss of armpit and pubic hair, and reduced sexual drive. Young children with Addison's disease may have insufficient weight gain and recurrent infections. Low cortisol also interferes with adrenocorticotropic hormone (ACTH) regulation, sometimes resulting in the darkening of the skin and mucous membranes, particularly in areas exposed to sun or regular friction.
Blood tests in people with Addison's disease often reveal low blood sodium. Many also have high blood potassium and/or high thyroid-stimulating hormone (TSH).
Most people with Addison's disease develop or have a preexisting autoimmune disease. Particularly common comorbid conditions are autoimmune thyroid disease (40% of people with Addison's), premature ovarian failure (up to 16% of people with Addison's), type 1 diabetes (11%), pernicious anemia (10%), vitiligo (6%) and celiac disease (2%). The combination of Addison's disease in addition to mucocutaneous candidiasis, hypoparathyroidism, or both, is called autoimmune polyendocrine syndrome type 1. The presence of Addison's in addition to autoimmune thyroid disease, type 1 diabetes, or both, is called autoimmune polyendocrine syndrome type 2.
An "adrenal crisis" or "Addisonian crisis" is a constellation of symptoms that indicates severe adrenal insufficiency. This may be the result of either previously undiagnosed Addison's disease, a disease process suddenly affecting adrenal function (such as adrenal hemorrhage), or an intercurrent problem (e.g., infection, trauma) in someone known to have Addison's disease. It is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment.
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Addison's disease
Addison's disease, also known as primary adrenal insufficiency, is a rare long-term endocrine disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of the cells of the adrenal glands (adrenal cortex), causing adrenal insufficiency. Symptoms generally develop slowly and insidiously and may include abdominal pain and gastrointestinal abnormalities, weakness, and weight loss. Darkening of the skin in certain areas may also occur. Under certain circumstances, an adrenal crisis may occur with low blood pressure, vomiting, lower back pain, and loss of consciousness. Mood changes may also occur. Rapid onset of symptoms indicates acute adrenal failure, which is a clinical emergency. An adrenal crisis can be triggered by stress, such as from an injury, surgery, or infection.
Addison's disease arises when the adrenal gland does not produce sufficient amounts of the steroid hormones cortisol and (sometimes) aldosterone. It is an autoimmune disease which affects some genetically predisposed people in whom the body's own immune system has started to target the adrenal glands. In many adult cases it is unclear what has triggered the onset of this disease, though it sometimes follows tuberculosis. Causes can include certain medications, sepsis, and bleeding into both adrenal glands. Addison's disease is generally diagnosed by blood tests, urine tests, and medical imaging.
Treatment involves replacing the absent or low hormones. This involves taking a synthetic corticosteroid, such as hydrocortisone or fludrocortisone. These medications are typically taken orally. Lifelong, continuous steroid replacement therapy is required, with regular follow-up treatment and monitoring for other health problems which may occur. A high-salt diet may also be useful in some people. If symptoms worsen, an injection of corticosteroid is recommended (people need to carry a dose with them at all times). Often, large amounts of intravenous fluids with the sugar dextrose are also required. With appropriate treatment, the overall outcome is generally favorable, and most people are able to lead a reasonably normal life. Without treatment, an adrenal crisis can result in death.
Addison's disease affects about 9 to 14 per 100,000 people in the developed world. It occurs most frequently in middle-aged females. The disease is named after Thomas Addison, a graduate of the University of Edinburgh Medical School, who first described the condition in 1855.
The symptoms of Addison's disease can develop over several months and resemble other medical conditions. Most common symptoms are caused by low levels of hormones that would normally be produced by the adrenal glands. Low blood cortisol can cause a variety of symptoms, including fatigue, malaise, muscle and joint pain, reduced appetite, weight loss, and increased sensitivity to cold. Gastrointestinal symptoms such as nausea, abdominal pain, and vomiting are particularly common. Low aldosterone can cause affected people to crave salty foods, as well as develop low blood pressure that leads to dizziness upon standing. In women, low dehydroepiandrosterone (DHEA) can result in dry and itchy skin, loss of armpit and pubic hair, and reduced sexual drive. Young children with Addison's disease may have insufficient weight gain and recurrent infections. Low cortisol also interferes with adrenocorticotropic hormone (ACTH) regulation, sometimes resulting in the darkening of the skin and mucous membranes, particularly in areas exposed to sun or regular friction.
Blood tests in people with Addison's disease often reveal low blood sodium. Many also have high blood potassium and/or high thyroid-stimulating hormone (TSH).
Most people with Addison's disease develop or have a preexisting autoimmune disease. Particularly common comorbid conditions are autoimmune thyroid disease (40% of people with Addison's), premature ovarian failure (up to 16% of people with Addison's), type 1 diabetes (11%), pernicious anemia (10%), vitiligo (6%) and celiac disease (2%). The combination of Addison's disease in addition to mucocutaneous candidiasis, hypoparathyroidism, or both, is called autoimmune polyendocrine syndrome type 1. The presence of Addison's in addition to autoimmune thyroid disease, type 1 diabetes, or both, is called autoimmune polyendocrine syndrome type 2.
An "adrenal crisis" or "Addisonian crisis" is a constellation of symptoms that indicates severe adrenal insufficiency. This may be the result of either previously undiagnosed Addison's disease, a disease process suddenly affecting adrenal function (such as adrenal hemorrhage), or an intercurrent problem (e.g., infection, trauma) in someone known to have Addison's disease. It is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment.
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