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Adrenal cortex disorders: Hormones out of kilter

Crawford, Ann PhD, RN; Harris, Helene MSN, RN

doi: 10.1097/01.NURSE.0000419427.99685.16
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Adrenal cortical hormones affect virtually every body system. By reviewing two complex disorders—adrenal cortical insufficiency (Addison disease) and adrenal cortical hypersecretion (Cushing syndrome)—nurses will be better prepared to recognize the wide-ranging effects on their patients' health and intervene appropriately.

Ann Crawford is a professor in the College of Nursing at the University of Mary Hardin-Baylor in Belton, Tex. Helene Harris is a clinical educator at Central Texas Veterans Healthcare System in Temple, Tex.

This article originally appeared in Nursing2011 CriticalCare. 2011;7(4):20-35.

The authors have disclosed that they have no financial relationships related to this article.

Figure

Figure

MRS. S, 46, IS HOSPITALIZED for treatment of an adrenal gland disorder. On admission, she complained of weakness, fatigue, and difficulty falling and staying asleep. For the past 6 weeks, she's noticed weight gain and menstrual irregularities. The nurse observes that she has a moon-face appearance, with an obese trunk and thin arms and legs. Her skin appears fragile and parchment-like, with scattered ecchymoses and striae on her abdomen. She's slightly tachycardic with an elevated BP. Her husband states that Mrs. S has been increasingly irritable and moody lately. Is Mrs. S suffering from adrenal cortical insufficiency or adrenal cortical hypersecretion?

This article will describe adrenal cortical insufficiency (also called Addison disease) and adrenal cortical hypersecretion (also called hypercortisolism and Cushing syndrome), and explain how to recognize and manage these conditions. For more on signs and symptoms, see Recognizing two adrenal disorders and Lab findings for patients with adrenal disorders.

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About the adrenal glands

The adrenal glands are highly vascular, tent-shaped organs located on top of the kidneys. The outer portion is known as the cortex while the inner portion is the medulla. Each portion has independent glandular functions. Their hormonal secretions affect organs and tissues throughout the body.1,2

The adrenal cortex produces and secretes hormones known as adrenal steroids or corticosteroids. These are classified as mineralocorticoids, which help control body fluid and electrolyte levels, and glucocorticoids, which are involved in many essential life processes. The adrenal medulla secretes small amounts of catecholamines as needed to help with stress response requirements.1,2

Aldosterone, the principal mineralocorticoid, maintains extracellular fluid volume as part of the renin-angiotensin-aldosterone system. Secreted in response to decreased extracellular volume, aldosterone stimulates the kidneys to reabsorb sodium and water and excrete potassium in an effort to increase plasma fluid volume and restore normal osmolality. Other factors that stimulate aldosterone secretion include elevated serum potassium levels and adrenocorticotropic hormone (ACTH) stimulation.1

Cortisol, the main glucocorticoid, influences metabolism of protein, fat, and carbohydrates, and also affects the body's stress response, emotional stability, and immune function. Cortisol release is regulated directly by ACTH (released from the anterior pituitary gland) and indirectly by corticotropin-releasing hormone (CRH), released from the hypothalamus.

Low circulating cortisol levels stimulate the hypothalamus to secrete CRH. The increased levels of CRH stimulate ACTH secretion, which in turn stimulates cortisol secretion. Conversely, high cortisol levels inhibit CRH release, comprising a negative feedback system. The pattern of hormonal secretion is affected by diurnal cycles as well as physiologic or psychological stress.13 Adrenal sex hormones for both genders (primarily androgens) are also secreted by the adrenal cortex, though not in amounts as large as secreted by the gonads.

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Adrenal cortical insufficiency

Primary adrenal cortical insufficiency is a relatively rare disorder also known as Addison disease. It occurs when the adrenal cortex layers are destroyed by autoimmune disorder, tuberculosis, metastatic carcinomas, hemorrhage, infections, toxins, or certain medications (for example, ketoconazole or mitotane). Secondary adrenal insufficiency may be caused by hypopituitarism, surgical removal of the pituitary gland, or, more commonly, sudden cessation of long-term, high-dose glucocorticoid therapy.1,3,4 Tertiary adrenal insufficiency is caused by problems with the hypothalamus.

Manifestations of adrenal insufficiency are related primarily to glucocorticoid and mineralocorticoid deficiency, and may develop in a slow progression or more quickly with stress (see Assessment findings in patients with adrenal insufficiency). Decreased glucocorticoid secretion impairs the body's ability to regulate blood glucose levels and to control the effects of the immune and inflammatory responses. Reduced cortisol release leads to hypoglycemia as glycogen stores are depleted; a decrease in urea nitrogen excretion by the kidneys and a decrease in gastric acid production may lead to anorexia and weight loss.13

Table

Table

Diminished aldosterone secretion triggers fluid and electrolyte imbalances. Sodium and water excretion is increased, with resultant hyponatremia and hypovolemia. Potassium excretion is reduced, causing hyperkalemia. Potassium retention also facilitates reabsorption of hydrogen ions within the renal tubules, which may cause acidosis.

Acute adrenal crisis (also called Addisonian crisis) is a life-threatening complication in which adrenal insufficiency manifests without warning, often due to a stressful event such as surgery, trauma, or infection. The patient's need for cortisol and aldosterone is greater than the body's supply, so hyponatremia, hypovolemia, and hyperkalemia progress rapidly, putting the patient at risk for cardiovascular collapse (more on this later).1,3

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Recognizing adrenal insufficiency

Lab results show decreased serum levels of cortisol, fasting glucose, and sodium. Potassium and blood urea nitrogen (BUN) levels are elevated. Patients with primary adrenal insufficiency also have an elevated ACTH level and an elevated eosinophil count secondary to decreased levels of circulating cortisol.

The most conclusive diagnostic test for adrenal insufficiency is an ACTH stimulation test, performed by administering I.V. ACTH and assessing plasma cortisol levels at 30 minutes and 1 hour. Patients with primary insufficiency will show little or no cortisol response; patients with secondary insufficiency will have elevated cortisol levels.1 Computed tomography, magnetic resonance imaging, arteriography, and X-rays may all help determine pituitary or adrenal causes for insufficiency.

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Treating adrenal insufficiency

Hormone replacement therapy, usually a combination of glucocorticoids and mineralocorticoids, is prescribed to treat adrenal insufficiency. Glucocorticoid replacement therapy includes hydrocortisone for maintenance, with the addition of fludrocortisone (a mineralocorticoid) for patients who don't attain an adequate salt-retaining effect with hydrocortisone (see Treating patients with adrenal insufficiency).3

Pain in adrenal insufficiency, related to fluid and electrolyte imbalances, can occur in the abdomen, lower back, and legs. Over-the-counter (OTC) medications such as acetaminophen help manage pain; patients also may need adjustments to the dosages of glucocorticoid replacement drugs.

Adrenal crisis requires immediate treatment to maintain essential metabolic functions. Follow the “5 S's” for management:

  1. salt replacement
  2. sugar (dextrose) replacement
  3. steroid replacement
  4. support of physiologic functions.
  5. search for and treat any identified cause.3

Replace extracellular fluid volume with 5% dextrose and 0.9% sodium chloride solution as prescribed. Monitor for electrolyte abnormalities; hyperkalemia is often present and may be treated with I.V. insulin and glucose to shift potassium into the cells. Administer I.V. hydrocortisone or dexamethasone, followed by continued use of hydrocortisone for long-term treatment, as prescribed.

Mineralocorticoid treatment usually isn't required during the acute phase of treatment due to the larger amounts of hydrocortisone being administered, but as the corticosteroid dosage is decreased, fludrocortisone will be added for maintenance.13

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Adrenal cortical hypersecretion

Hyperfunction of the adrenal cortex can result in oversecretion of one or more hormones. Hypersecretion of the adrenal cortex may produce hypercortisolism, hyperaldosteronism (Conn syndrome), or elevated androgen production.

Excessive secretion of cortisol can be due to problems with the hypothalamus or the anterior pituitary gland (Cushing disease), the adrenal gland, or long-term administration of potent glucocorticoid medications (iatrogenic Cushing syndrome). All body systems will be affected by this excessive secretion. Without treatment, hypercortisolism can be fatal.

A dexamethasone suppression test is the most widely used screening tool for Cushing disease with pituitary and adrenal causes. Dexamethasone is administered at 2300 to 2400 hours, and a plasma cortisol level is drawn at 0800 hours the next morning. Suppression of cortisol indicates normal functioning of the pituitary and adrenal glands. If plasma cortisol levels are elevated (greater than 5 mg/dL), further testing is needed to determine the cause. Because stress, obesity, depression, and certain medications may cause false elevation in cortisol levels, clinical suspicion and additional lab findings will also help to point toward the diagnosis.2,5

Altered plasma ACTH levels will differ depending on the cause. ACTH is elevated in Addison disease and pituitary tumor-related Cushing disease. Levels are usually normal or decreased in Cushing syndrome caused by glucocorticoid-containing medications such as prednisone or by adrenal tumors.

Table A

Table A

Diagnosis of glucocorticoid hormone excess is contingent on the finding of cortisol hypersecretion. One of the earliest signs of hypercortisolism is the loss of diurnal pattern of cortisol secretion.3 Late night (between 2300 and 2400 hours) serum or salivary cortisol levels can be inappropriately elevated, and 24-hour urine tests will show elevated cortisol.3 Serum sodium and glucose levels will be increased, while serum potassium and eosinophil levels will be decreased.1,2

Imaging studies may identify abnormalities such as adrenal gland or anterior pituitary gland tumors causing hypercortisolism.1,2

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Treating adrenal cortical hypersecretion

Depending on the cause, treatment can include surgery, irradiation, or pharmacologic interventions to reduce plasma cortisol levels without causing permanent damage to the anterior pituitary or adrenal glands.3 Surgery to remove a pituitary or adrenal tumor is performed with the goal of leaving the remaining endocrine tissue for continued function. Following surgery, the patient is placed on cortisol replacement therapy for 6 months to a year, or until adrenal function returns. If the entire pituitary gland or both adrenal glands are removed, the patient requires lifelong hormonal replacement.

Irradiation may be used for pituitary tumors, often as an adjunctive therapy if pituitary surgery is unsuccessful. With radiation, the effects may not be immediately evident and normal tissue may be damaged, which can cause other adverse reactions such as headaches, hypertension, dry skin, and alopecia. Also, patients must continue medication therapy for several months while awaiting the final results of the radiation therapy. Regular monitoring is necessary to determine the effectiveness of radiation therapy and development of new pituitary hormone deficiency.2,6

If the cause of Cushing syndrome is long-term use of corticosteroids, treatment involves a slow weaning of the medication to let the body resume normal cortisol production. Abruptly discontinuing these medications could lead to deficient cortisol levels (mimicking adrenal insufficiency).2

Drug therapy also can help control manifestations of hypercortisolism. Medications are specifically used for controlling cortisol production and include ketoconazole, metyrapone, mifepristone, and mitotane, which have antiglucocorticoid properties.

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Nursing responsibilities

When caring for a patient with an adrenal hypersecretion disorder, obtain a thorough health history and perform a comprehensive physical assessment. Assess for muscle weakness, fatigue, and pain; assist with ambulation and activities of daily living as needed. Assess the patient's skin for discoloration and an increase in fat deposits. Assess for the development of hirsutism in women, as well as irregularities in the menstrual cycle.

Regularly assess the patient's cardiovascular and pulmonary status, renal function, and neurologic status. Monitor vital signs and assess for signs and symptoms of fluid volume overload, including hypertension, pulmonary crackles, peripheral edema, and jugular vein distension, secondary to sodium and water retention. Monitor intake and output, serum glucose and electrolyte levels for abnormalities, especially hypokalemia and hypernatremia.

Because of the decreased inflammatory and immune responses, monitor the patient for signs and symptoms of infection. Monitor the patient's mental status and be alert for mood swings, anger, and depression. Allow the patient to express concerns, and make appropriate referrals as necessary.1,2

Mrs. S, whom we met at the beginning of this article, has hypercortisolism. She's treated surgically with a unilateral adrenalectomy and placed on cortisol replacement medications. Postoperatively, she'll need to be monitored for several months to determine if adrenal function returns, and her medications will be adjusted accordingly.

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Patient education

Teach patients with hypercortisolism about the disease and its manifestations, and their medications and adverse reactions. Make sure they know when to call their healthcare provider. Explain the effects of hypercortisolism, treatment plans, and continued care needs. Emphasize the importance of keeping scheduled appointments with the healthcare provider and of notifying the healthcare provider if symptoms increase. Encourage patients to wear a medical-alert bracelet or medal in case of emergency.1,2

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Staying in balance

Adrenal cortical hormones affect virtually every body system. When these hormone levels are out of kilter, they can cause many problems and even be life threatening. By understanding manifestations of hormonal abnormalities, treatments, and appropriate nursing interventions, you can help patients with adrenal disorders keep their balance.

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Lab findings for patients with adrenal disorders

Addison disease

  • Hyponatremia
  • Hyperkalemia
  • Hypoglycemia
  • Hypercalcemia
  • Leukocytosis
  • Elevated BUN
  • Eosinophilia
  • Possible acidosis
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Cushing syndrome

  • Hypernatremia
  • Hypokalemia
  • Hyperglycemia
  • Lymphocytopenia
  • Eosinopenia
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Treating patients with adrenal insufficiency3,7-9

Hydrocortisone is a synthetic adrenocortical steroid with glucocorticoid and mineralocorticoid properties. This drug mimics the effects of the body's own corticosteroids and achieves similar effects. Hydrocortisone also has immunosuppressive properties and alters the body's metabolism of fats, carbohydrates, and proteins.

Fludrocortisone, another synthetic adrenocortical steroid, is used for its mineralocorticoid properties (it also has glucocorticoid properties). Fludrocortisone binds the mineralocorticoid receptor (aldosterone receptor) and causes an increase in ion and water transport, raising extracellular fluid volume and BP and lowering serum potassium levels.

Nursing considerations: Perform a thorough history and physical assessment as a baseline for further assessment findings and continue to monitor the patient as follows.

  • Assess for infections before beginning these drugs and continue to monitor for signs and symptoms of infection.
  • Monitor vital signs regularly, assessing for orthostatic hypotension and other signs and symptoms of decreased cardiac output.
  • Monitor intake and output, and watch for signs and symptoms of dehydration.
  • Monitor serum electrolytes regularly, and assess for abnormalities, especially hyponatremia and hyperkalemia. Also monitor serum glucose levels and weight.
  • Administer potassium supplements and place the patient on a low-sodium, high-potassium diet as prescribed.
  • Assess for bowel sounds and monitor for nausea, vomiting, and diarrhea, which may predispose the patient to fluid volume deficit.
  • Auscultate lung and heart sounds, and assess peripheral pulses and capillary refill.
  • Assess the patient's skin for hyperpigmentation, especially around the nipples and in skin folds.
  • Assess the patient's mental status, being cognizant of the potential for mood swings and depression.
  • Monitor for cushingoid effects such as moon face, and for petechiae.
  • Assess for signs of peptic ulcer disease or ulcerative colitis.
  • Institute fall prevention strategies and assist with ambulation and daily activities as needed.
  • Regularly assess the patient's pain. Use a pain intensity rating scale and intervene appropriately.

Patient education: Tell patients to notify their healthcare provider if they gain three or more pounds in 1 week and to notify their healthcare provider immediately if they develop heartburn or indigestion. Tell patients not to take OTC medications for heartburn, or any OTC or herbal medications without talking to their healthcare provider. Instruct patients to report insomnia or mood changes to their healthcare provider. Explain the importance of avoiding alcohol and aspirin, which may contribute to the development of peptic ulcer disease. Teach patients which foods are high in vitamin D, protein, and calcium to help reduce the risk of osteoporosis. Tell the patient or family to report any wounds that are slow to heal or aren't healing. Explain the importance of adhering to the medication regimen and follow-up appointments with their healthcare provider. Tell patients not to discontinue taking medication without consulting with their healthcare provider.

Because many patients with adrenal insufficiency have episodes of hyponatremia and hypoglycemia, they should maintain a regular schedule for food intake and activity. Advise patients to wear a medical-alert bracelet or medal identifying their condition in case of emergency.

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REFERENCES

1. Ignatavicius D, Workman L. Medical-surgical Nursing, Critical Thinking for Collaborative Care. 6th ed. Philadelphia, PA: Elsevier Saunders; 2010.
2. Smeltzer SC, Bare BG, Hinkle JL, Cheever KH. Textbook of Medical-surgical Nursing. 11th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008.
3. Porth C, Matfin G. Pathophysiology: Concepts of Altered Health States. 8th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2009.
4. Reddy P. Clinical approach to adrenal insufficiency in hospitalized patients. Int J Clin Pract. 2011;65(10):1059-1066.
5. Carroll TB, Findling JW. The diagnosis of Cushing's syndrome. Rev Endocr Metab Disord. 2010;11(2):147-153.
6. Vance ML. Cushing's disease: radiation therapy. Pituitary. 2009;12(1):11-14.
7. Adams M, Koch R. Pharmacology: Connections to Nursing Practice. Upper Saddle River, NJ: Pearson; 2010.
8. Skidmore-Roth L. Mosby's Nursing Drug Reference. 23rd ed. St. Louis, MO: Elsevier Mosby; 2010.
    9. Hodgson B, Kizior R. Saunders Nursing Drug Handbook. St. Louis, MO: Elsevier Saunders; 2012.
    © 2012 Lippincott Williams & Wilkins, Inc.