Page 43 Complete Your CE Test Online - Click Here Adrenal insufficiency, also known as Addison’s disease and adrenal hypofunction, is the result of hypofunction of the adrenal glands [5,25] . The disease occurs in two forms: primary and secondary[25] . ● ● Primary adrenal insufficiency: The primary form (commonly referred to as Addison’s disease) originates within the adrenal glands and is characterized by a decrease in mineralocorticoid, glucocorticoids, and androgen secretion [11,25] . ● ● Secondary adrenal insufficiency: The secondary form of the disease occurs secondary to a disorder (e.g., pituitary tumor) outside the adrenal glands. In this form, aldosterone secretion may not be affected[5,11] . Secondary adrenal insufficiency is more common than Addison’s disease[25] . Incidence Adrenal hypofunction affects males and females in equal numbers and can occur at any age[5,11] . Hypofunction of the adrenal glands affects one in 16,000 neonates congenitally. In adults, 8 in 100,000 people are affected[5] . Etiology Primary adrenal hypofunction is defined as occurring when more than 90% of both adrenal glands are destroyed[5,25] . The majority of cases (up to 80% of Addison’s disease cases) are caused by an autoimmune process in which circulating antibodies specifically “attack” adrenal tissue[5,25] . Autoimmune Addison’s disease occurs primarily in middle- aged females and gradually destroys the adrenal cortex, the outer layer of the adrenal glands[25] . In primary adrenal hypofunction, the adrenal glands may be the only glands affected. However, other endocrine glands may be affected as well, something that is referred to as polyendocrine deficiency syndrome[25] . Polyendocrine deficiency syndrome appears in two forms: type 1 and type 2[25] . Type 1 is inherited and occurs in children who, in addition to adrenal insufficiency, may also have[25] : ● ● Underactive parathyroid glands. ● ● Slowed sexual development. ● ● Pernicious anemia. ● ● Chronic fungal infections. ● ● Chronic hepatitis. Type 2 polyendocrine deficiency syndrome, sometimes referred to as Schmidt’s syndrome, is also inherited but usually affects young adults and includes[25] : ● ● Underactive thyroid gland. ● ● Slowed sexual development. ● ● Diabetes. ● ● Vitiligo (loss of pigment on areas of the skin). Other causes of primary adrenal hypofunction include[5,25] : ● ● Tuberculosis: Tuberculosis can destroy the adrenal glands. It was once the chief cause of primary adrenal insufficiency, but now accounts for only 10% to 15% of Addison’s diseases in developed countries. However, current research shows an increase in primary adrenal hypofunction due to tuberculosis of the adrenal glands and cytomegalovirus infection. Cytomegalovirus primarily affects people who have weakened immune systems. ● ● Malignancy of the adrenal glands. ● ● Surgical removal of the adrenal glands. ● ● Hemorrhage into the adrenal glands. ● ● Genetic defects. ● ● Medication induced adrenal hypofunction from such agents as antifungal medications and the anesthetic etomidate. The occurrence of secondary adrenal hypofunction may be traced to a temporary form of the disorder that occurs when long-term corticosteroid is discontinued. Such long-term therapy causes the adrenal glands to produce less of their hormones. Once the prescription corticosteroids are discontinued the adrenal glands may not resume production of their own hormones in a timely fashion. This can lead to secondary adrenal hypofunction[5,25] . Secondary adrenal hypofunction alert! Prescription corticosteroids should always be discontinued gradually over a period of time ranging from weeks to months to reduce the chances of adrenal insufficiency[25] . ● ● Surgical removal of pituitary tumors. ● ● Infections of the pituitary. ● ● Reduction of blood flow to the pituitary. ● ● Pituitary radiation for treatment of pituitary tumors or tumors of adjacent structures. ● ● Surgical removal of portions of the hypothalamus. ● ● Surgical removal of the pituitary gland. Adrenal crisis Adrenal insufficiency can sometimes lead to adrenal crisis, a critical deficiency of mineralocorticoids and glucocorticoids. Adrenal crisis is the most serious complication related to adrenal hypofunction. It can develop gradually or abruptly[5,11,25] . Adrenal crisis is most likely to develop in people who[5,11] : ● ● Fail to respond to hormone replacement therapy. ● ● Abruptly stop hormone or prescribed steroid therapy. ● ● Experience trauma, surgery, or other types of physiologic stress that exhaust the body’s provisions of glucocorticoids in someone who has adrenal hypofunction. ● ● Undergo bilateral adrenalectomy. ● ● Develop adrenal gland thrombosis following a severe infection (referred to as Waterhouse-Friderichsen syndrome). During adrenal crisis, there is a swift decline in the steroid hormones cortisol and aldosterone. This decline impacts the liver, stomach, and kidneys[11] . Adrenal crisis produces[5,25] : ● ● Significant weakness and fatigue. ● ● Abrupt severe pain in the lower back, abdomen, or legs. ● ● Severe nausea and vomiting. ● ● Dehydration. ● ● Hypotension. ● ● Loss of consciousness. Occasionally, adrenal crisis can cause patients to develop a high fever followed by hypothermia. Untreated adrenal crisis can lead to vascular collapse, renal shutdown, coma, and death[5] . Adrenal crisis requires immediate, life-saving interventions. An intravenous bolus of hydrocortisone must be administered followed by fluid resuscitation. Later doses of hydrocortisone are given intramuscularly or are diluted with dextrose in saline solutions and given intravenously until the patient stabilizes[5,25] . With swift, appropriate treatment, adrenal crisis usually resolves quickly. Patients may need maintenance doses of hydrocortisone to maintain stability[5] . Nursing consideration: Persons at risk for adrenal crisis should wear a medical alert bracelet and carry a corticosteroid injection with them at all times and be taught how to inject themselves. Nurses should teach the patients’ and their families and friends how to administer the injection as well in case the patient loses consciousness and is unable to inject him/herself[25] . Pathophysiology of adrenal insufficiency The adrenal glands, located just above the kidneys, produce cortisol and aldosterone. These hormones help regulate blood pressure, metabolism, and the way the body responds to stress. Adrenal hormones also help to produce androgens and estrogens[25] .