Disorders of the Adrenal Glands
Proper secretion of the adrenal hormones is essential to life. Without these vital hormones, many processes will malfunction and cause an imbalance. We will discuss the imbalances pertaining to the adrenal cortex and medulla, which can lead to adrenal insufficiency (Addison's Disease), adrenocortical hyperfunction (Cushing's Syndrome) and pheochromocytoma.
Addison's Disease (adrenal insufficiency)
Like many other endocrine disorders mentioned previously, adrenal insufficiency is either primary or secondary.
Primary adrenal insufficency (Addison's) is caused by a destruction of the adrenal cortex by diseases such as tuberculosis (which accounts for less than 20% of cases according to NIDDK (2012)). Addison's can also be an autoimmune disorder, where antibodies attack and destroy the adrenal tissue. In many cases, the cause is idiopathic meaning the cause is unknown. Since the cortex is responsible for the secretion of corticosteroids (and sex hormones), these hormone levels will be decreased or absent in adrenal insufficiency which leads to decreased availability of glucose, causing hypoglycemia and reduced renal function. The glomerular filtration rate (GFR) will progressively decrease leading to decreased urea nitrogen excretion leaving the waste products will remain in the body.
All three types of adrenal steroids are down
THINK 3 S:
Decreased glucocorticoids (sugar)
Decreased mineralocorticoids (salt)
Decreased gonadocorticoids aka androgens (sex)
Secondary adrenal insufficiency results from "surgical removal of both adrenal glands (bilateral adrenalectomy), hemorrhagic infarction of the glands, hypopituitarism, suppression of adrenal function by administration of corticosteroids" (Timby & Smith, 2013, p. 773). When clients have undergone bilateral adrenalectomy or hypophysectomy, they may not experience loss of these hormones because they will be treated with corticosteroids, which will replace the hormones no longer secreted by the adrenals.
Usually glucocorticoids and androgen levels will be low, mineralocorticoids rarely are.
Primary adrenal insufficency (Addison's) is caused by a destruction of the adrenal cortex by diseases such as tuberculosis (which accounts for less than 20% of cases according to NIDDK (2012)). Addison's can also be an autoimmune disorder, where antibodies attack and destroy the adrenal tissue. In many cases, the cause is idiopathic meaning the cause is unknown. Since the cortex is responsible for the secretion of corticosteroids (and sex hormones), these hormone levels will be decreased or absent in adrenal insufficiency which leads to decreased availability of glucose, causing hypoglycemia and reduced renal function. The glomerular filtration rate (GFR) will progressively decrease leading to decreased urea nitrogen excretion leaving the waste products will remain in the body.
All three types of adrenal steroids are down
THINK 3 S:
Decreased glucocorticoids (sugar)
Decreased mineralocorticoids (salt)
Decreased gonadocorticoids aka androgens (sex)
Secondary adrenal insufficiency results from "surgical removal of both adrenal glands (bilateral adrenalectomy), hemorrhagic infarction of the glands, hypopituitarism, suppression of adrenal function by administration of corticosteroids" (Timby & Smith, 2013, p. 773). When clients have undergone bilateral adrenalectomy or hypophysectomy, they may not experience loss of these hormones because they will be treated with corticosteroids, which will replace the hormones no longer secreted by the adrenals.
Usually glucocorticoids and androgen levels will be low, mineralocorticoids rarely are.
Assessment Findings
The symptoms of adrenal insufficiency usually begin gradually.
The most common symptoms include:
Most dangerous feature of Addison’s disease is hypotension, which may cause shock, especially during stress.
Review Box 50-1 pg. 773 in Timby & Smith for Signs and Symptoms of Adrenal Insufficiency
The symptoms of adrenal insufficiency usually begin gradually.
The most common symptoms include:
- Chronic, worsening fatigue
- Muscular weakness
- Loss of appetite and weight loss
- Other symptoms can include:
- nausea
- vomiting
- diarrhea
- low blood pressure that falls further when standing, causing dizziness or fainting
- irritability and depression
- a craving for salty foods due to salt loss
- hypoglycemia, or low blood glucose
- headache
- sweating
- in women, irregular or absent menstrual periods
- decreased hair growth
- hyperpigmentation (dark) can occur in exposed areas of skin such as lips and mucous membranes (lining of cheek); skin folds, elbows, knees, knuckles, and toes.
- Hyperpigmentation and decreased hair growth occur only in primary insufficiency.
Most dangerous feature of Addison’s disease is hypotension, which may cause shock, especially during stress.
Review Box 50-1 pg. 773 in Timby & Smith for Signs and Symptoms of Adrenal Insufficiency
Diagnostic Findings
ACTH Stimulation Test - Screens for adrenal function using synthetic ACTH. Serum and urine cortisol levels checked after administration. Normal response will be that cortisol levels rise. In primary adrenal insufficiency, the response will be absent or low cortisol levels. In secondary insufficiency, the response may be less noticeable since the gland may still respond to ACTH. A low dose of ACTH may be administered to diagnose secondary adrenal insufficiency since the low dose should still cause cortisol levels to rise in normal responses. If there is still no response then it is secondary adrenal insufficiency (NIDDK, 2012).
Other diagnostic tests include:
ACTH Stimulation Test - Screens for adrenal function using synthetic ACTH. Serum and urine cortisol levels checked after administration. Normal response will be that cortisol levels rise. In primary adrenal insufficiency, the response will be absent or low cortisol levels. In secondary insufficiency, the response may be less noticeable since the gland may still respond to ACTH. A low dose of ACTH may be administered to diagnose secondary adrenal insufficiency since the low dose should still cause cortisol levels to rise in normal responses. If there is still no response then it is secondary adrenal insufficiency (NIDDK, 2012).
Other diagnostic tests include:
- Serum cortisol levels (decreased)
- Serum sodium and chloride (decreased)
- Fasting blood glucose levels (decreased)
- Serum potassium (elevated)
- Serum calcium (elevated)
- Serum BUN (elevated)
- WBC (elevated)
- Adrenal X-Rays (calcification)
- CT ( atrophy of adrenal glands)
Medical Management
Treatment is aimed at replacing the hormones that the glands are no longer producing.
Primary and secondary adrenal insufficiency treatment includes daily corticosteroid replacement therapy for lifetime.
Usually synthetic steroid fludrocortisone (Florinef), is preferred because it contains both glucocorticoid and a mineralocorticoid properties. An additional glucocorticoid (hydrocortisone, prednisone, dexamethasone) may be added depending on the client's response to therapy (Timby & Smith, 2013). SE of corticoids, especially fludrocortisone may be hypertension, edema, CHF, increased sweating, hypokalemia, muscle weakness, headache, allergic skin reaction (Roach & Ford, 2010, p. 440)
Aldosterone may be supplemented to help with sodium depletion. In addition, increased sodium intake is advised (NIDDK, 2012).
If secondary adrenal insufficiency occurs from abrupt discontinuation of steroid therapy or hemorrhagic infarction of the gland, the treatment may vary according to the function of the adrenals (p. 774).
It is important to educate on the importance of medication compliance since not taking medication could cause acute adrenal crisis. It is important to educate client that steroid therapy must be tapered off (discontinued over time).
Treatment is aimed at replacing the hormones that the glands are no longer producing.
Primary and secondary adrenal insufficiency treatment includes daily corticosteroid replacement therapy for lifetime.
Usually synthetic steroid fludrocortisone (Florinef), is preferred because it contains both glucocorticoid and a mineralocorticoid properties. An additional glucocorticoid (hydrocortisone, prednisone, dexamethasone) may be added depending on the client's response to therapy (Timby & Smith, 2013). SE of corticoids, especially fludrocortisone may be hypertension, edema, CHF, increased sweating, hypokalemia, muscle weakness, headache, allergic skin reaction (Roach & Ford, 2010, p. 440)
Aldosterone may be supplemented to help with sodium depletion. In addition, increased sodium intake is advised (NIDDK, 2012).
If secondary adrenal insufficiency occurs from abrupt discontinuation of steroid therapy or hemorrhagic infarction of the gland, the treatment may vary according to the function of the adrenals (p. 774).
It is important to educate on the importance of medication compliance since not taking medication could cause acute adrenal crisis. It is important to educate client that steroid therapy must be tapered off (discontinued over time).
Nursing Management
- Teach client about disease process and importance of therapy regimen compliance
- Teach client about SE of corticosteroid therapy and effects of abruptly stopping
- Monitor I & Os
- Daily weights
- Monitor for dehydration, hyponatremia, weight loss and notify physician of these changes
- Encourage increased fluid intake and prescribed diet to maintain fluid and electrolyte balance (due to Na+ loss, increased K+, and Ca++).
- Encourage increased sodium intake, if hyponatremic
- Monitor blood glucose levels (for hypoglycemia)
- Monitor blood pressure (for hypotension)
- Advise to obtain MedicAlert tag/bracelet indicating client has adrenal insufficiency
- (Timby & Smith, 2013, p. 774-775)
Read about Acute Adrenal Crisis (Addisonian Crisis) on your own in Timby & Smith, 2013, p. 775-776
Cushing's Syndrome
At the opposite end of the spectrum from adrenal insufficiency is adrenocortical HYPERfunction (Cushing's syndrome), which occurs from excessive levels of hormone produced by the adrenal cortex, mainly cortisol. The adrenal cortex produces too much hormone. Reasons for overproduction are:
- The pituitary gland produces too much ACTH (adrenocorticotropic hormone), which results in overstimulation of the adrenal cortex and in turn, results in excessive production and secretion of adrenocortical hormones (glucocorticoids, mineralocorticoids, and sex hormones).
- Benign and malignant tumors of the pituitary gland or adrenal cortex
- Long-term use of corticosteroids (steroids).
The most common cause of Cushing's is prolonged use of corticosteroids medication (e.g., prednisone for chronic inflammatory disorders, such as rheumatoid arthritis, chronic obstructive pulmonary disease, and Crohn’s disease). For this reason, it is important to instruct patients to taper off when taking steroids. Steroids medications cannot be stopped abruptly; it must be done over time.
For some unexplained reason, Cushing's is 3-5 times more common in females, especially between 20-60 years of age. 10-15 per million people develop Cushing's syndrome (Genetics Home Reference, 2012)
For some unexplained reason, Cushing's is 3-5 times more common in females, especially between 20-60 years of age. 10-15 per million people develop Cushing's syndrome (Genetics Home Reference, 2012)
Assessment Findings
A patient with Cushing's might appear as if they have gained weight and have a moon face- fullness, round, with big cheeks. Physical characteristics are referred to as cushingoid syndrome.
The following are other typical physical and symptomatic characteristics of Cushing's syndrome:
A patient with Cushing's might appear as if they have gained weight and have a moon face- fullness, round, with big cheeks. Physical characteristics are referred to as cushingoid syndrome.
The following are other typical physical and symptomatic characteristics of Cushing's syndrome:
- Buffalo hump (from fat redistribution)
- Facial redness
- Thin skin
- Muscle wasting and weakness (from protein depletion) so extremities will be thin
- Hirsutism in women (facial hair), secondary sex characteristics
- Amenorrhea (no menstrual cycle)
- In males, erectile dysfunction can occur
- Decreased sexual drive in both genders
- thinning hair
- bruises easily
- stretch marks (striae)
- Kyphosis due to osteoporosis (slouched appearance, c/o back ache, collapse of vertebrae)
- Peripheral edema (retains Na+ and water)
- Mood changes, depression
- Mental changes (some serious)
- Diabetes mellitus (from carb intolerance)
- Impaired wound healing and increased susceptibility to wounds
- Hypertension (from Na+ retention and water retention)
- Suppression of inflammatory response
- Hypokalemia (results from hypernatremia [retention of Na=])
Diagnostic Findings
Diagnosis is based on symptoms and confirmed with urine and blood tests.
Blood tests include:
Serum sodium (increased)
Serum potassium (decreased)
Blood glucose (increased)
Urine levels of (17-OHCS) and (17-KS), a type of steroid, are elevated
Plasma and urine cortisol levels are elevated
Overnight dexamethasone suppression test serves as an initial screening tool; One milligram of oral dexamethasone is given to patient and the next morning the plasma cortisol levels are drawn. Additional doses are given if results are above normal (see Timby & Smith, 2013, p. 777).
To check whether cause is related to pituitary stimulation, plasma ACTH is measured along with the administration of dexamethasone. When both the levels of ACTH and cortisol are elevated, it is more likely that underlying cause is due to pituitary or hypothalamus involvement (p. 777).
Additional diagnostic test performed are:
Diagnosis is based on symptoms and confirmed with urine and blood tests.
Blood tests include:
Serum sodium (increased)
Serum potassium (decreased)
Blood glucose (increased)
Urine levels of (17-OHCS) and (17-KS), a type of steroid, are elevated
Plasma and urine cortisol levels are elevated
Overnight dexamethasone suppression test serves as an initial screening tool; One milligram of oral dexamethasone is given to patient and the next morning the plasma cortisol levels are drawn. Additional doses are given if results are above normal (see Timby & Smith, 2013, p. 777).
To check whether cause is related to pituitary stimulation, plasma ACTH is measured along with the administration of dexamethasone. When both the levels of ACTH and cortisol are elevated, it is more likely that underlying cause is due to pituitary or hypothalamus involvement (p. 777).
Additional diagnostic test performed are:
- Abdominal x-rays, CT, MRI which show adrenal enlargement
- IV pyelogram "may show changes in renal shadow caused by abnormally large adrenal gland" (p. 777).
Medical Management
Treatment and management are aimed at treating the underlying cause and correcting the imbalances of cortisol levels. If the cause is related to a tumor, it must be removed surgically. If the cause is related to adrenal hyperplasia, then the pituitary may be removed or receive radiation treatment. If both adrenal glands are affected, then perhaps a bilateral adenalectomy may be performed.
Treatment and management are aimed at treating the underlying cause and correcting the imbalances of cortisol levels. If the cause is related to a tumor, it must be removed surgically. If the cause is related to adrenal hyperplasia, then the pituitary may be removed or receive radiation treatment. If both adrenal glands are affected, then perhaps a bilateral adenalectomy may be performed.
- Medication therapy consists of diuretics for the edema and fluid retention and also to reduce HTN.
- Diet modification with sodium restrictions (low salt diet) to prevent edema.
- Low Carb diet to help control glycemic levels (to decrease blood glucose)
- Antibiotics are used to treat infections (since susceptible to infections)
- In Cushingoid syndrome, slowly cut down medication dose as to not abruptly stop medication (steroids).
- If taking steroids for leukemia or for immunosuppression (organ transplant), syndrome is allowed to persist since benefits outweigh risks (p. 778).
Nursing Management
An important part of nursing managing is to help the patient understand the disease process. Part of the nursing care given to the patient, is to
–focus on signs and symptoms of hormone and drug toxicity
–assess for complications
•CV, DM, infection (due to decreased resistance), nephrolithiasis, pathological fractures
•Client with Cushing’s syndrome is seriously ill and debilitated.
•Therapeutic interventions may have many side effects, the focus of daily assessment is on signs and symptoms of hormone and drug toxicity and complicating conditions such as CV disease, DM, infection, due to decreased resistance, nephrolithiasis, and pathological fractures, DT osteoporosis.
Daily nursing interventions include:
An important part of nursing managing is to help the patient understand the disease process. Part of the nursing care given to the patient, is to
–focus on signs and symptoms of hormone and drug toxicity
–assess for complications
•CV, DM, infection (due to decreased resistance), nephrolithiasis, pathological fractures
•Client with Cushing’s syndrome is seriously ill and debilitated.
•Therapeutic interventions may have many side effects, the focus of daily assessment is on signs and symptoms of hormone and drug toxicity and complicating conditions such as CV disease, DM, infection, due to decreased resistance, nephrolithiasis, and pathological fractures, DT osteoporosis.
Daily nursing interventions include:
- Maintain muscle tone (provide ROM exercises, assist with ambulation)
- Prevent accidents or falls and provide adequate rest
- Protect client from exposure to infection
- Maintain skin integrity (provide meticulous skin care, prevent tearing of skin; use paper tape, if necessary)
- Minimize stress in environment
- Monitor VS; observe for HTN, edema
- Measure I & O, daily weights
- Provide diet low in calories and sodium and high in protein, potassium, calcium, and Vitamin D
- Monitor urine for glucose and acetone; administer insulin if ordered
- Provide psychological support and acceptance
- Prepare client for hypophysectomy or radiation if condition is caused by a primary tumor
- Prepare client for an adrenalectomy if condition is caused by an adrenal tumor or hyperplasia
- Provide client teaching and discharge planning concerning: diet modifications; importance of adequate rest, need to avoid stress and infection, change in medication regime (alternate day therapy or reduced dosage) if cause of the condition is prolonged corticosteriod therapy.
Pheochromocytoma
A tumor (usually benign; 90%) of the adrenal medulla causing hyperfunction meaning it causes adrenal medulla to oversecrete catecholamines (epinephrine and norepinephrine). Since production of catecholamines can be triggered by any stressful event and factors that the body experiences such as exercise, emotional distress, surgery, and other traumas. Manipulation of the tumor can also cause secretion of catecholamines as well as postural changes.
Excessive secretion of catecholamines can lead to many complications such as hypertension (due to constriction of blood vessels), which then increases risk of cerebrovascular accident (CVA, stroke), palpitations, and tachycardia (Timby & Smith, 2013, p. 776).
It is important to assess for pheochromocytoma in patients who have uncontrollable HTN, take more than 4 medications for BP control, or develop HTN before 35 years of age.
Excessive secretion of catecholamines can lead to many complications such as hypertension (due to constriction of blood vessels), which then increases risk of cerebrovascular accident (CVA, stroke), palpitations, and tachycardia (Timby & Smith, 2013, p. 776).
It is important to assess for pheochromocytoma in patients who have uncontrollable HTN, take more than 4 medications for BP control, or develop HTN before 35 years of age.
Assessment
Symptoms include signs and symptoms of epinephrine/norepinephrine stimulation
Diagnostic tests that can be performed to confirm diagnosis are:
Symptoms include signs and symptoms of epinephrine/norepinephrine stimulation
- Increased BP (either intermittent, frequent, or persistent)
- Tremors
- Nervousness
- Sweating
- Headache (due to high BP)
- Nausea
- Vomiting
- Hyperglycemia (due to elevated epinephrine)
- Polyuria
- Vertigo
Diagnostic tests that can be performed to confirm diagnosis are:
- Vanillylmandelic acid 24 hour urine test (elevated) checks for by products of catecholamines
- CT, MRI, ultra sound, aortography, and retrograde pyelography reveal the tumor
- Drop in BP after test injection of alpha-adrenergic blocker phentolamine (Regitine) supports diagnosis
- Drop in BP (compared to baseline) shows systolic BP drops >35 mm Hg and diastolic BP drops >25 mm Hg
Medical and Surgical Management
Treating pheochromocytoma involves removing the tumor either surgically or laparoscopically (minimally invasive). One gland is removed (unilateral adrenalectomy). Prior to surgery phentolamine is given to control HTN and it is administered after surgery as well. Other alpha-adrenergic blockers such as phenoxybenzamine (Dibenzyline) can be given to control BP prior to surgery or even when patient is not a suitable surgical candidate, or in treating a malignant pheochromocytoma (Timby & Smith, 2013, p. 776).
Other medical treatment given is metyrosine (Demser), an enzyme inhibitor used to reduce production of catecholamines in order to prevent rise in BP which can lead to hypertensive attacks.
Treating pheochromocytoma involves removing the tumor either surgically or laparoscopically (minimally invasive). One gland is removed (unilateral adrenalectomy). Prior to surgery phentolamine is given to control HTN and it is administered after surgery as well. Other alpha-adrenergic blockers such as phenoxybenzamine (Dibenzyline) can be given to control BP prior to surgery or even when patient is not a suitable surgical candidate, or in treating a malignant pheochromocytoma (Timby & Smith, 2013, p. 776).
Other medical treatment given is metyrosine (Demser), an enzyme inhibitor used to reduce production of catecholamines in order to prevent rise in BP which can lead to hypertensive attacks.
Nursing Management
As the nurse it is important to monitor the blood pressure closely as high BP can lead to stroke and organ damage. It is wise to monitor the patient's BP closely upon starting drug therapy and during dose changes. Any sudden decreases in BP should be notified to physician. If the patient undergoes surgery (adrenalectomy) it is important to monitor for signs and symptoms of adrenal insufficiency (Addison's disease) since the levels of hormones have suddenly decreased (Timby & Smith, 2013, p. 776).
As the nurse it is important to monitor the blood pressure closely as high BP can lead to stroke and organ damage. It is wise to monitor the patient's BP closely upon starting drug therapy and during dose changes. Any sudden decreases in BP should be notified to physician. If the patient undergoes surgery (adrenalectomy) it is important to monitor for signs and symptoms of adrenal insufficiency (Addison's disease) since the levels of hormones have suddenly decreased (Timby & Smith, 2013, p. 776).
Let's see how much you learned after reviewing this section of the module. Click on the brown button below which will take you to
the Endocrine Module test. It is a 50 question test reviewing information from this module. It will help you see how much information you have retained. You will have a separte Diabetes Module Test after you complete that section of the module as well. Ultimately, it will help you on the Endocrine Exam. These are study aids that I have created to help you do well on the exam. Be sure to type your name in the box so that I can keep track of your progress. Thank you!
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the Endocrine Module test. It is a 50 question test reviewing information from this module. It will help you see how much information you have retained. You will have a separte Diabetes Module Test after you complete that section of the module as well. Ultimately, it will help you on the Endocrine Exam. These are study aids that I have created to help you do well on the exam. Be sure to type your name in the box so that I can keep track of your progress. Thank you!
If you have any questions, please do not hesitate to contact me.
Resources:
Genetics Home Reference (2012, June). Cushing Disease. Retrieved September 15, 2013, from http://ghr.nlm.nih.gov/condition/cushing-disease
National Institute of Diabetes and Digestive and Kidney Diseases. (2012). Adrenal Insufficiency and Addison's Disease. Retrieved September 14, 2013, from http://endocrine.niddk.nih.gov/pubs/addison/addison.aspx#what
Patient.co.uk (2012). Cushing's Syndrome. Retrieved September 15, 2013, from http://www.patient.co.uk/health/cushings-syndrome-leaflet
Genetics Home Reference (2012, June). Cushing Disease. Retrieved September 15, 2013, from http://ghr.nlm.nih.gov/condition/cushing-disease
National Institute of Diabetes and Digestive and Kidney Diseases. (2012). Adrenal Insufficiency and Addison's Disease. Retrieved September 14, 2013, from http://endocrine.niddk.nih.gov/pubs/addison/addison.aspx#what
Patient.co.uk (2012). Cushing's Syndrome. Retrieved September 15, 2013, from http://www.patient.co.uk/health/cushings-syndrome-leaflet