Disorders of the adrenal cortex

Cushing’s syndrome/Addison’s disease

Cushing’s syndrome
Etiology and pathophysiology
-a spectrum of clinical abnormalities caused by excess corticosteroids, particularly
glucocorticoids
-Several conditions can cause Cushing’s syndrome
-Iatrogenic administrartion of exogenous cortisol
-endogenous Cusing’s syndrome-85% due to an ACTH-secreting pituitary
tumor(Cushing’s disease)
-adrenal tumors
-ectopic ACTH production by tumors outside the hypothalamic-pituitary -adrenal
axis
usually the lung or pancreas
-Cushings disease and primary adrenal tumors are more commmon in women 20-40
years of age
ectopic ACTH production is more common in men

Clinical manifestations
Can be seen in most body systems and are related to excess levels of
cortiscosteroids
-weight gain, accumulation of fat in the trunk, face, and cervical area
-transient weight gain from sodium and water retention may be present because of
the mineralcorticoid effects
-glucose intolerance-due to insulin resistance and increased gluconeogenesis
-Protein wasting-catabolic effects of cortisol on peripheral tissue
-muscle weakness in the extremities
-Loss of protein matrix in the bone-osteoporosis
-vertebral and compression fractures-chronic back pain
-loss of collagen-weak thin skin
-easy bruising
-catabolic processes dominate and wound healing is delayed
-mood disturbances(irritability, anxiety, euphoria)
-insomnia, irrationality, and psychosis may occur
-hypertension
-acne
-menstrual disorders in women, hirsutism in women and gynecomastia and
impotence in men, more commonly seen with adrenal tumors

Clincal presentation
• history and physical examination
• centripedal obesity or generalized obesity
• moon facies(fullness of the face) with facial plethora
• purplish-red striae which are usually depressed below the skin
surface on the abdomen breast or buttocks
• hirsutism
• menstrual disorders
 • hypertension
• unexplained hypokalemia

Diagnostic Studies

-Granulocytosis
-lymphocytopenia
-eosinopenia
-hyperglycemia
-glycosuria
-hypercalcuria
-osteoporosis
-hypokalemia
-alkolosis
-Plasma ACTH may be low, normal or elevated depending upon the exact problem
-Normal or High ACTH indicate ACTH dependent Cushing’s syndrome
-Low or undectable ACTH indicate an adrenal or exogenous etiology
-If cushing’s syndrome is suspected-a 24hour urine collection for free cortisol and a
low-dose dexamethasone suppression test is done

Collaborative Care
Cushing’s Disease
-Transphenoidal surgical removal of the pituitary adenoma
-Adrenalectomy is indecated for adrenal tumors or hyperplasia
-If surgery is suspected the client should be brought to an optimal level of
health
>glucose should be under control
>vit A replacement to aid in wound healing
>high protein meal plan to correct protein depletion
Drug Therapy
• In inoperable cases or in cases in which residual disease remains, treatment
with
-mitotane -suppresses cortisol production, alters peripheral metabolism of
cortisol and decreases plasma and urine corticosteroid levels
-Other drugs used include
>Nizoral
>cytadren
• If cushing’s syndrome has developed as a result of prolonged administration
of glucocorticoids(eg. Cortisol), one or more of the following alternatives may
be tried
-gradual discontinuance of glucocorticoid therapy
-reduction of the glucocorticoid dose
-conversion to an alternate-day regimen
-Gradual tapering of glucocorticoids is necessary to avoid potentially life-
threatening adrenal insufficiency

Nursing Management
• Review subjective and objective data
 Nursing Diagnosis
 – Risk for infection r/t lower resistance to stress and suppression of immune
system
– Risk for injury, fracture r/t decreased muscle strength, fatigue, osteoporosis
and increased protein catabolism
– Altered nutrition more than body requirements r/t increased appetite, high
caloric foods, decreased activity
– Self esteem disturbance r/t altered body image, emotional lability
– Impaired skin integrity r/t excess corticosteroids, immobility, and altered skin,
fragility as manifested by edema, thin fragile skin and impaired healing

Nursing Implementation
• Identify patients at risk for Cushing’s syndrome.
• Careful patient education on use of corticosteroids and potential
consequences
• Acute intervention
– Therapeutic interventions are focused on daily assessments for side
effects related to hormone and drug toxicity
• Vital signs q 4
• Daily weights
• signs and symptoms of infection
• location time and duration of abdominal pain
• signs and symptoms of abnormal thromboembolic phenomena
such as sudden chest pain, dyspnea or tachycardia
• capillary blood glucose monitoring
• bone pain or limitations of range of motion, especially the lower
back
• Changes in mental status, depression
Clients require a great deal of emotional support to deal with
– changes in appearance
• centripedal obesity
• multiple bruises
• hirusitism in women
• gynecomastia in men
– Body image changes may make the client feel unattractive
– 71% of clients surveyed with Cushing’s syndrome stated their lives had
been very adversely affected by this condition.

Preoperative/postoperative
If treatment involves surgical removal of a pituitary adenoma, an adrenal
tumor, or one or both adrenal glands
– Nursing care will focus on preoperative and postoperative care
– Client must be brought to optimal physical condition prior to surgery
• correct hyper/hypoglycemia
• increased protein in diet
• potassium supplements etc.
– Postoperatively clients must be watched closely duet to changes in
levels of hormone
– High doses of corticosteroids are administered postoperatively and
during surgery
– This ensures adequate response to the stressors
 – Glucocorticoids may be continued long-term or replacement may be
forever depending upon what the surgical procedure entails
– If glucoroticoid dosage is tapered too rapidly after surgery acute
adrenal insufficiency can occur
– Discharge instructions are based on the patient’s lack of endogenous
corticosteroids and resulting inability to react to stressors
phsysiologically
– Patients should always wear a medic alert bracelet
– Exposure to extremes in temp. emotional stress, and infection should
be avoided
– Lifetime replacement therapy is required by many patients after
surgery
– Pt. Education on medication administration is a priority

Complications
Acute adrenal insufficiency
– Addisonian crisis- a life threatening emergency caused by insufficient
adrenocortical hormones or a sudden sharp decrease in these
hormones
– it may occur during stress(eg. Infection, surgery, trauma, hemorrhage,
or psychologic distress)
– or following sudden withdrawal of cortociosteroid hormone
replacement therapy
– this is often done by the patient without knowledge of the importance
of replacement therapy

Severe manifestations of glucocorticoid and mineralcorticoid deficiencies

 Hypotension
 tachycardia
 dehydration
 hyponatremia
 hyperkalemia
 hypoglycemia
 fever
 weakness
 diarrhea
 abdominal pain

Diagnostic studies
• Clinical features of addison’s disease
• Diagnosis is made when cortisol levels are subnormal or fail to rise over basal
levels with an ACTH stimulation test
• hyperkalemia
• hypchloremia
• hyponatremia
• hypoglycemia
• anemia
• increased BUN
 • A failure of cortisol levels to rise in response to ACTH stimulation indicates
primary adrenal disease.

Collaborative Care
• Treatment of adrenocortical insufficiency is focused on management of the
underlying cause.
• Mainstay of treatment is replacement therpay with glucocorticoids and
mineralcorticoids
• hydrocortisone is the most commonly used form of replacement therapy
• Management of Addisonian crisis requires immediate glucocorticoid
replacement therapy
• treatment must be vigorous and directed toward shock management
• IV hydrocortisone 100 mg every 6 hours
• sodium, fluids and dextrose are necessary until blood pressure returns to
normal

Effects of corticosteroids
• Antiinflammatory action-
• Immunosppression-
• maintenance of normal blood pressure-
• Carbohydrate and protein metabolic effects-

Complications of Corticosteroid therapy

 Review patient teaching guide and uses of corticosteroids
 Review side effects
• susceptibility to infection
• blood pressure increased
• glucose intolerance
• protein depletion
• hypocalcemia
• decreased mucus production-increased risk of duodenal ulceration
• surgical client increased risk for evisceration-healing is delayed
• hypokalemia
• skeletal muscle atrophy
• suppression of pitu8itary ACTH synthesis
• mood and behavior changes-depression
• fat from extremities is redistributed to trunk and face