The adrenal glands are (normally) small golden-yellow pyramid shaped glands located on top of each kidney. The gland consists of an outer part (cortex) and an inner part (medulla). The adrenal glands make hormones that are absolutely essential for body functions, the so called steroid hormones.
The outer layer makes
(i) cortisol required as our body’s main stress hormone,
(ii) aldosterone, which is required for salt and water balance, and
(iii) androgens (male sexual hormones).
The inner layer of the gland makes adrenaline and noradrenaline , responsible for our “fight and flight” response.
Cortisol is one of the body’s most important hormones; its principal role is to help the body respond to stress.
Cortisol also contributes to:
Maintaining blood pressure and cardiovascular function
Contributing to maintenance of blood sugar levels, together with insulin
Suppressing the immune system's inflammatory response
Regulating the metabolism of proteins, carbohydrates, and fats
The hormone aldosterone, together with the kidneys, regulates the balance of sodium and potassium in the body and plays a major role in maintaining normal blood pressure.
The adrenal medulla normally produces adrenaline (epinephrine) and noradrenaline (norepinephrine) in stressful situations.
Are weak male sex hormones (Androstenedione, Dehydroepiandrosterone DHEA) present in both women and men. In women, they regulate some sexual characteristics like pubic and armpit hair growth. DHEA-Sulfate (DHEAS) has gained a lot of attention as anti-aging supplement, click here to read more.
Adrenal disorders can be broadly divided into three groups:
Under-activity – Adrenal Insufficiency
Over-activity – Cushing’s syndrome, Conn’s syndrome, Pheochromocytoma
Tumors - both benign and malignant (adrenal cancer)
Adrenal Insufficiency (AI)
Adrenal insufficiency is a condition in which the cortex does not make enough hormones. We further differentiate primary versus secondary AI.
Primary adrenal insufficiency - Addison's Disease
In this rare condition, the adrenal glands don’t work properly and cannot make enough cortisol. Usually, production of aldosterone and androgens is also low. The most common cause of primary adrenal insufficiency is autoimmune disease, meaning the body’s defense system attacks and destroys the body’s own tissues. When adrenal glands are damaged, they can’t produce hormones. Other causes of primary adrenal insufficiency include bleeding within the glands, infections, genetic diseases, and surgical removal of the adrenal glands.
Secondary adrenal insufficiency
This far more common type of adrenal insufficiency results when the pituitary gland, the master gland in the brain, does not signal the adrenal glands to make cortisol. Normally, the pituitary gland makes a hormone called adrenocorticotrophic hormone (ACTH), which stimulates the adrenal glands to make cortisol. But in secondary adrenal insufficiency, the pituitary gland doesn’t send ACTH to the adrenal glands. No cortisol is made. Some causes may be temporary, such as taking cortisol-like steroids like prednisone (suggesting to the pituitary that there is sufficient cortisol in the body). Other causes may be permanent, such as hormone problems present at birth, tumors or infections in the pituitary, or surgical or radiation damage to the pituitary.
What are the symptoms of true adrenal insufficiency?
Symptoms begin little by little. They include:
Nausea, vomiting, and diarrhea
Pain in the muscles and joints
Low blood pressure leading to dizziness upon standing
Cravings for salt (in primary adrenal insufficiency)
Symptoms of low blood glucose, such as sweating
Darkened skin on the face, neck, and back of the hands (in primary adrenal insufficiency)
Irregular menstrual periods in women
Some people don’t know they have adrenal insufficiency until they have a sudden worsening of symptoms called an adrenal crisis.
Physical stress caused by illness, infection, surgery, or an accident can suddenly make symptoms of adrenal insufficiency much worse, an emergency condition called adrenal crisis. If untreated, adrenal crisis will cause death. Adrenal crisis occurs mainly in people with primary adrenal insufficiency. Symptoms of an adrenal crisis include sudden pain in the back, abdomen, or legs, severe nausea and vomiting, and diarrhea. People in adrenal crisis may be dehydrated and confused. They can have low blood pressure and might pass out or even die. People in adrenal crisis need an injection of glucocorticoid right away. Then they need to be brought to the hospital for more treatment. If you have adrenal insufficiency, you should know the warning signs of adrenal crisis. You also should tell family and friends what to do if a crisis occurs. Always carry a medical alert bracelet and have a dose of injectable steroid (Solu-Cortef) readily available. Dr. Schneider will discuss these aspects with you and provide necessary information and prescriptions.
How is adrenal insufficiency diagnosed?
Dr. Schneider will review your symptoms and medical history. He will check blood levels of cortisol, other hormones, sodium, potassium, and glucose to diagnose Adrenal Insufficiency and help determine the cause. The gold standard test is the Cosyntropin Test. This involves an injection of synthetic ACTH (Cosyntropin) and measuring cortisol levels before and at 30 and 60 minutes after injection. Saliva cortisol measurement is another option, albeit not as well validated yet.
It may also be necessary to look at the adrenal glands or the pituitary gland with imaging tests, such as CT or MRI scans.
What is the treatment for adrenal insufficiency?
The goal of treatment is to ensure proper hormone levels day-to-day. You may need daily replacement of hormones for life. You will take glucocorticoids (hydrocortisone) to replace the cortisol that your body no longer makes. You may also need mineralocorticoids (aldosterone) if your body doesn’t make enough. Extra glucocorticoids may be needed during times of stress, such as serious illness or surgery. Your doctor will provide personalized advice on adjusting medicines for stress. Understanding your disease and knowing when and how to adjust your medications can help you live a long and healthy life with adrenal insufficiency
What is Adrenal Fatigue?
Adrenal fatigue is a fictional construct used by many alternative medicine practitioners to explain unspecific symptoms and to legitimize a large number of unnecessary and unvalidated tests and ailments. Contrary to their and to popular belief, the adrenal glands do not “fatigue” or lose function as a result of mental or physical stress. Also, there is no deficiency of the inner medulla and consequent impaired adrenaline or noradrenaline production. True adrenal insufficiency is a rare but potentially fatal condition that should only be diagnosed by an endocrinologist using standard testing procedures. If you believe or were told that you are suffering with adrenal fatigue, speak with Dr. Schneider. He will carefully evaluate your adrenal function to exclude true adrenal insufficiency.
Hormone Excess And Tumors
Cushing's Syndrome and Cushing's Disease
The overproduction of cortisol by the adrenal glands leads to Cushing's syndrome. More commonly, Cushing's syndrome is caused by the prolonged intake of glucocorticoid drugs such as prednisone, methylprednisolone, or dexamethasone. Even glucocorticoids that are present in skin creams used for eczema or psoriasis will be absorbed through the skin and may result in Cushing’s syndrome.
The overproduction of cortisol can be caused by a primary overproduction of cortisol by the adrenal gland, often associated with an adrenal tumor, or by an overproduction of ACTH (adrenocorticotropic hormone) either in the pituitary or in a benign or malignant tumor outside of the pituitary such as in the lung, thymus gland, or pancreas. The pituitary form of the disease is called Cushing's disease.
Cushing's syndrome is characterized by:
obesity - characteristically sparing the arms and legs resulting in a round, red face, a neck fat pad (the obsolete term is "buffalo hump") and a large abdomen with purplish "stretch" marks
high blood pressure
high blood sugar
Irritability, anxiety, and depression
Women with Cushing's syndrome will usually have excess hair growth on their face, neck, chest, abdomen and thighs that can be very bothersome. In women the menstrual periods may be irregular
Men may have decreased fertility and a reduced desire for sex (low libido)
The diagnosis of Cushing’s syndrome requires special tests and scans. The only definitive cure of an adrenal tumor producing excess cortisol is surgery. This is usually key hole surgery: either laparoscopic or retroperitoneoscopic.
Conn’s syndrome is a condition characterized by the excess production of aldosterone. This may be produced either by both adrenal glands that are dysfunctional or by a single adrenal benign tumor (adenoma). The hormone aldosterone plays a key role in the maintenance of normal salt balance in the blood. The excess production of aldosterone in Conn’s syndrome causes a high blood pressure typically but not necessarily with a low serum potassium. The disease can be treated medically or, if caused by a single adenoma, can be cured by surgical removal of the affected adrenal gland (adrenalectomy), which today is generally performed via a keyhole technique – a laparoscopic or retroperitoneoscopic adrenalectomy.
Most adrenal tumors are benign adenoma, but rarely adrenal tumors can be cancers.
Pheochromocytomas are tumors of the adrenal medulla. Most pheochromocytomas produce excess amounts of adrenaline or noradrenaline and as such may cause episodes of severe elevations in blood pressure, which can be fatal.
The typical symptoms of this disease include:
recurring unexplained sweating episodes
feelings of high anxiety or of impending doom
chest pain or tightness
The diagnosis of a pheochromocytoma relies on having an index of suspicion that initiates the biochemical investigations. These include a 24 hour urine collections to measure levels of adrenaline and noradrenaline, and their breakdown products, metanephrines. Blood levels of metanephrines may also be measured. Once the biochemical diagnosis has been made, localization studies are required to identify the site of the hormone overproduction. These include CT, ultrasound and MRI scans and radionuclid imaging.
Once diagnosed, the biochemical effects of excess catecholamine secretion must be treated immediately in order to make the patient safe from the potentially fatal blood pressure fluctuations. This should be followed by surgery that usually is a laparoscopic (keyhole) adrenalectomy.