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Adrenal gland disorders

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Adrenal gland

About Adrenal Gland Disorders

The adrenal glands, located on the top of each kidney, are responsible for releasing different hormones. Adrenal gland disorders occur when the adrenal glands produce too much or too little of these hormones.

The adrenal glands, located on the top of each kidney, are responsible for releasing different classes of hormones.

The outer part of the gland, called the adrenal cortex, produces the hormones cortisol (pronounced KAWR-tuh-sohl) and aldosterone (pronounced al-DOS-tuh-rohn). The inner part of the gland, called the adrenal medulla (pronounced muh-DUHL-uh), produces the hormones adrenaline and noradrenaline.

These hormones—cortisol, aldosterone, adrenaline, and noradrenaline—control many important functions in the body, including:

  • Maintaining metabolic processes, such as managing blood sugar levels and regulating inflammation
  • Regulating the balance of salt and water
  • Controlling the "fight or flight" response to stress
  • Maintaining pregnancy
  • Initiating and controlling sexual maturation during childhood and puberty

The adrenal glands are also an important source of sex steroids, such as estrogen and testosterone.

Adrenal gland disorders occur when the adrenal glands do not work properly. They can be classified into disorders that occur when too much hormone is produced or when too little hormone is produced.

These disorders can occur if there is a problem with the adrenal gland itself, such as a disease, genetic mutation, tumor, or infection. Or, sometimes the disorder results from a problem in another gland, such as the pituitary, which helps to regulate the adrenal gland. In addition, some medications can cause problems with how the adrenal glands function. When the adrenal glands produce too little or too many hormones, or when too many hormones come into the body from an outside source, serious health problems can develop.

Overall, adrenal gland disorders are generally rare. The number of people affected and at risk depends on the specific type of adrenal gland disorder.

Cushing's syndrome

What are some types of adrenal gland disorders?

There are several types of adrenal gland disorders, each with its own symptoms and treatments.

Most adrenal gland tumors—abnormal growths on the adrenal glands—are not cancerous. They often do not cause symptoms or require treatment. However, adrenal gland tumors can produce a variety of different hormones, leading hormone levels to get too high.

Adrenal tumors can cause:

  • Cushing's syndrome, by producing cortisol so that body levels get too high
  • Primary hyperaldosteronism, by creating high levels of the hormone aldosterone (controls blood pressure and body salt and potassium levels)
  • Pheochromocytoma, by producing too much adrenaline (regulates the "fight-or-flight" response)

This is a cancerous adrenal tumor that tends to develop in the outer layer of the adrenal gland. Cancerous adrenal tumors are often found years after they start growing, at which point the cancer have spread to other organs.

Cushing syndrome is a rare disease that results from having too much cortisol hormone in the body. In some cases, Cushing syndrome develops from long-term or overuse of steroid medications(medicines that act like cortisol in the body). In other cases, the body itself produces too much cortisol. This overproduction can happen for several reasons, including the presence of tumors(abnormal growths) such as a:

  • Tumor of the pituitary gland (this is called Cushing disease)
  • Tumor of the adrenal gland (as explained above)
  • Tumor in another part of the body (these are called "ectopic" tumors and are more commonly found in the pancreas, lung, or the thyroid gland)

CAH is a common genetic disorder in which the body makes too little cortisol. People with CAH may also have other hormone imbalances. For example, their bodies might not make enough aldosterone (controls blood pressure and body salt and potassium levels), but might make too much androgen (promotes the development of male sexual organs).

The pituitary gland is located at the base of the brain. It releases hormones that affect many of the body's functions. Among those hormones is the adrenocorticotropic (pronounced a-DREE-noh kawr-tuh-koh-TRO-pic) hormone (ACTH), which stimulates the adrenal glands to release the hormone cortisol.

Sometimes, benign (noncancerous) pituitary tumors or—more rarely cancerous tumors1—may grow on the pituitary gland, which can cause a variety of problems. Some pituitary tumors release too much ACTH, which, in turn, can cause the adrenal glands to produce too much cortisol. Cushing's disease refers to pituitary tumors that cause Cushing's syndrome.

Pheochromocytomas (pronounced fee-oh-kroh-moh-sigh-TOH-muhs) are part of a larger family of tumors, called paragangliomas (pronounced pair-uh-gang-lee-OH-muhs). Pheochromocytoma is a type of tumor that develops in the adrenal medulla, the inner part of the adrenal gland. It produces adrenaline, causing high levels of this hormone in the body. In most cases, the tumors are not cancerous and do not spread to other parts of the body. But in about 10% of cases, the tumors are cancerous and can spread.

The normal activity of the adrenal glands can be suppressed—or reduced—when people take steroid medications (medicines that act like cortisol in the body) such as prednisone, hydrocortisone, or dexamethasone.2 Steroid medications, most often prednisone, may be prescribed to treat certain types of arthritis, severe allergic reactions, asthma, autoimmune (pronounced awh-toh-im-YOON) diseases, and other conditions.

Ordinarily, someone taking steroids takes gradually lower and lower doses as time goes by until they stop taking the drug completely. This is called "tapering" the dose. When steroid medications are stopped suddenly, especially after being taken for several weeks or more, the adrenal glands may be unable to produce steroid hormones (most importantly, cortisol) in sufficient amounts for several weeks or even months. This situation can cause health problems because of the imbalance of hormone levels that continues until the adrenal glands start functioning normally again.

This rare disorder develops when the adrenal glands do not make enough cortisol. In most cases of Addison's disease, the body also doesn't make enough of the hormone aldosterone.

Addison's is an autoimmune disease—a condition in which the immune system, which is supposed to protect the body, mistakenly attacks the body's own tissues and cells. In the case of Addison's disease, this reaction results in damage to the adrenal glands.4 In the long term, this damage can get worse until eventually the adrenal glands aren't working at all.

This disorder occurs when the body produces too much aldosterone, a hormone that controls blood pressure and regulates the body's salt and potassium levels. The extra aldosterone is produced either by a tumor, which typically affects one adrenal gland, or by abnormal growth of both glands, a condition called "hyperplasia."

What are the symptoms of adrenal gland disorders?

The adrenal glands, located on the top of each kidney, are responsible for releasing different hormones. Adrenal gland disorders occur when the adrenal glands produce too much or too little of these hormones.

Common symptoms of Cushing's syndrome (due to an adrenal, pituitary, or ectopic tumor) can include:

  • Upper body obesity, round face and neck, and thinning arms and legs
  • Skin problems, such as acne or reddish-blue streaks on the abdomen or underarm area
  • High blood pressure
  • Muscle and bone weakness
  • Moodiness, irritability, or depression
  • High blood sugars
  • Slow growth rates in children

Women may also have increased growth of hair on their face and body and experience menstrual irregularities. Men may become less fertile and have a reduced or absent sex drive.

Symptoms of CAH range from mild to serious. Some people with mild CAH are never diagnosed because their symptoms do not cause them any problems.

Symptoms of the mild form of CAH, which can be diagnosed in children or adults, may include1:

  • Shorter than average final height
  • Early signs of puberty (in children)
  • Acne
  • Irregular menstrual periods and possible trouble getting pregnant (in women)
  • Excess facial hair (in women)

Symptoms of the severe form of CAH, which is diagnosed in children, may include:

  • Dehydration
  • Low blood pressure
  • Low blood sugar level
  • Trouble keeping enough salt in the body
  • Altered development of the external genitalia in girls, which is noted at birth and may require surgery to correct
  • Shorter than average final height
  • Early signs of puberty
  • Irregular periods and possible trouble getting pregnant (in women)
  • Excess facial hair (in women)
  • Benign testicular tumors and infertility (in men)

The symptoms of functioning pituitary tumors depend on the particular hormone the tumor is overproducing.

Too much prolactin may cause:

  • Headache
  • Some loss of vision
  • Less frequent or no menstrual periods or menstrual periods with a very light flow
  • Difficulty getting pregnant
  • Impotence in men
  • Lower sex drive
  • The flow of breast milk in a woman who is not pregnant or breastfeeding

Too much adrenocorticotropic hormone (ACTH) may cause:

  • Headache
  • Some loss of vision
  • Weight gain reflected in the face, neck, and trunk of the body, but thin arms and legs
  • A lump of fat on the back of the neck
  • Thin skin that may include purple or pink stretch marks on the chest or abdomen
  • Easy bruising
  • Growth of fine hair on the face, upper back, or arms
  • Bones that break easily
  • Anxiety, irritability, depression
  • Growth deceleration with weight gain in children
  • Irregular menses

Too much growth hormone may cause:

  • Headache
  • Some loss of vision
  • In adults, growth of the bones in the face, hands, and feet
  • In children, excessive growth of the whole body
  • Tingling or numbness in the hands and fingers
  • Snoring or pauses in breathing during sleep
  • Joint pain
  • Sweating more than usual
  • Extreme dislike of or concern about one or more parts of the body

Too much thyroid-stimulating hormone (TSH) may cause:

  • Irregular heartbeat
  • Shakiness
  • Weight loss
  • Trouble sleeping
  • Frequent bowel movements
  • Sweating

Nonfunctioning tumors press on or damage the pituitary and prevent it from secreting enough hormones. If there is too little of a particular hormone, the gland or organ it normally controls will not function correctly. Symptoms of nonfunctioning pituitary tumors are:

  • Headache
  • Some loss of vision
  • Loss of body hair
  • In women, less frequent menstrual periods or no periods at all, or no milk from the breasts
  • In men, loss of facial hair, growth of breast tissue, and impotence
  • In women and men, lower sex drive
  • In children, slowed growth and sexual development

Other general symptoms of pituitary tumors include the following:

  • Nausea and vomiting
  • Confusion
  • Dizziness
  • Seizure
  • Runny or drippy nose

Most people with pheochromocytoma have high blood pressure (hypertension) because the tumor causes the adrenal gland to produce too much adrenaline or noradrenaline. Other symptoms may include:

  • Rapid heart rate
  • Headache
  • Sweating
  • Episodes of high or low blood pressure
  • Anxiety or panic attack
  • Shaking (tremors) of the hands
  • Pale skin
  • Blurred vision
  • Weight loss
  • Constipation
  • Abdominal pain
  • High blood sugar
  • Psychiatric disturbances

Symptoms can vary, depending on what causes the disease. Symptoms typically include:

  • Weight loss
  • Weakness
  • Extreme fatigue
  • Nausea and/or vomiting
  • Low blood pressure
  • Patches of darker skin
  • Craving for salt
  • Dizziness upon standing
  • Depression

The main symptom is moderate to high blood pressure (hypertension), which can be difficult to control. Other symptoms include:

  • Low potassium levels
  • Muscle cramping or spasms
  • Excessive urination, sometimes at night
  • Headache
  • Generalized weakness

What causes adrenal gland disorders?

Adrenal gland disorders are caused by problems with one or both adrenal glands or by problems with other glands, such as the pituitary gland.

Specific disorders can develop when the adrenal glands produce too few or too many hormones, or when too many hormones are introduced from an outside source.

Cushing's syndrome occurs when the body is exposed to high levels of the hormone cortisol over a long period of time.

Sometimes Cushing's syndrome develops when people take certain hormones for asthma, rheumatoid arthritis, lupus, and other inflammatory diseases. The syndrome also can occur when hormones are taken to suppress the immune system so that a patient's body will not reject a transplanted organ.

Other people develop Cushing's syndrome because their bodies produce too much cortisol.2 Other causes of Cushing's syndrome include pituitary adenomas (a type of benign tumor), ectopic adrenocorticotropic hormone syndrome, adrenal tumors, or familial Cushing's syndrome. Cushing's syndrome due to tumors occurs more commonly in women.

CAH is a group of inherited disorders of the adrenal glands. It affects men and women equally. Both parents must carry the gene in order for a child to be born with CAH. Researchers have identified the location of the gene that causes the most common forms of CAH as chromosome

Scientists have not yet discovered what causes pituitary tumors. Most pituitary tumors are not inherited; only a small percentage of cases run in families.

Most of the time, pheochromocytoma and paraganglioma tumors are "sporadic," meaning why they develop is not associated with any known risk factor or genetic mutation. However, research increasingly shows that a number of patients, possibly up to one-quarter, have genetic mutations responsible for tumor development. In these patients, family members may also be affected.

In all cases of Addison's disease, the adrenal glands do not produce enough of the hormone cortisol. In most cases, the glands also make too little of the hormone aldosterone. This is also termed primary adrenal insufficiency. This disease can be caused by an autoimmune disorder, infection (for example, tuberculosis), or other rare diseases that cause infiltration and thus destruction of the adrenal glands (for example, sarcoidosis or amyloidosis). The most common cause of Addison's disease in developed countries is autoimmune disorders.

There are two causes of hyperaldosteronism. One cause is an excessive growth of normal cells in both adrenal glands. The other cause is a non-cancerous tumor in one of the glands.8 There are no known gene mutations associated with this disorder at this time. However, rarely, hyperaldosteronism can run in families.

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