CUSHING’S SYNDROME
Assessment
The most common feature of a person with Cushing’s Syndrome is having that
cushingoid appearance. Due to the overproduction of cortisol by the adrenal cortex,
there is an increased glucose production. Fats are deposited in a certain character
for a person with Cushing’s syndrome. The usual sites of fat deposits are in the trunk
(central) - thus the truncal obesity and pendulous abdomen, in the cheeks
(cervicodorsal fat pads) - which is responsible for that “moon face” -, and in the neck
(buffalo hump). In extrahepatic tissues, cortisol increases the catabolic effect on
peripheral tissues. It inhibits amino acid uptake and protein synthesis leading to
protein wasting; hence, the thin extremities. Further, cortisol also inhibits collagen
formation leading to thin, weak integumentary tissues. The capillaries are more
visible. And because of the increasing size of the adipose tissues, purple striae in the
abdomen is observed. Aside from that, loss of collagenous support around small
blood vessels leads to the person susceptible to rupture or easy bruising. Cortisol is
not the only hormone that is affected, androgen, which is also produced by the
adrenal cortex is affected. Thus, increased androgens causes the body to develop
more body and facial hair (hirsutism), acne, changes in voice, recession of hairline,
and clitoral hypertrophy. Virilization or the masculinization of female is evident - lack
of breast development, increased muscle bulk, and hirsutism. One of the effects of
glucocorticoids is it inhibits growth. So another feature of the cushingoid appearance
is short stature.
Cushing syndrome is often suspected as the cause of obesity in children. Some
obese children do have elevated levels of plasma corticosteroids, a fact that
complicates the diagnosis. However, these elevated levels of corticosteroids are
secondary to the obesity; they are not the cause. Children with natural obesity are
generally tall; those with Cushing syndrome are short.
To confirm the diagnosis for Cushing’s syndrome is to have the child undergo the
dexamethasone suppression test. If given to a normal child, plasma level of adrenal
cortisol will fall. However, for children with adrenocortical tumors, plasma level of
cortisol will not fall because the tumor affected the feedback mechanism of the
pituitary gland and thus it continues to secrete ACTH albeit given dexamethasone.
Treatment
Treatment to restore hormonal balance and reverse Cushing’s syndrome may
necessitate radiation, drug therapy, or surgery.
Radiation therapy
● Pituitary irradiation
○ Patients with pituitary-dependent Cushing’s syndrome with adrenal
hyperplasia and severe cushingoid symptoms (such as psychosis,
poorly controlled diabetes mellitus, osteoporosis, and severe
pathologic fractures) may require pituitary irradiation.
○ Radiation therapy may be recommended if surgery isn't an option, if
some of a pituitary tumor remains or comes back after surgery, or if the
tumor causes symptoms that aren’t controlled with medicines.
Pharmacologic therapy
● Adrenal enzyme inhibitors
○ Metyrapone, aminoglutethimide, mitotane, and ketoconazole used to
reduce hyperadrenalism if syndrome is caused by ACTH secretion by a
tumor that cannot be eradicated
● Cortisol therapy
○ Cortisol therapy is essential during and after surgery, to help the
patient tolerate the physiologic stress imposed by the removal of the
pituitary or adrenals
Surgical therapy
Removal of the adrenal or pituitary gland offers a greater success rate for the
recovery of the patient.
● Transsphenoidal hypophysectomy
○ Surgical removal of the tumor by transsphenoidal hypophysectomy is
the treatment of choice if Cushing’s syndrome is caused by pituitary
tumors and has an 80% success rate.
● Adrenalectomy
○ Adrenalectomy is the treatment of choice in patients with primary
adrenal hypertrophy. It is a surgical removal of one or both adrenal
glands. The procedure can be performed using an open incision or
laparoscopic technique
Nursing Diagnosis and Interventions
1. Disturbed Body Image related to altered physical appearance
● Assess for any changes in personal appearance caused by the cortisol
excess
○ Physical changes may include: moon face, buffalo hump,
excessive weight gain, red cheeks, hirsutism and
hyperpigmentation
● Encourage the client to verbalize feelings about the body image
changes
○ Expression of feelings can enhance the client’s coping
strategies
2. Impaired Skin Integrity related to impaired wound healing
● Assess the skin frequently to check for reddened areas, skin
breakdown, tearing or excoriation
○ Cushing’s disease causes thinning of the skin because cortisol
causes the breakdown of some proteins along with the
weakening of small blood vessels which allows it to be damaged
easily
● Assess the skin for signs of bruising
○ The accumulation of fat causes the skin to stretch which is
already thin and weakened due to cortisol action, causing it to
hemorrhage and stretch permanently
● Instruct the client about keeping the skin clean and moisturized
○ Excessive dryness or excess moisture increases the risk for skin
breakdown
3. Risk for injury related to weakness
● Discuss and assist client safety measures for ambulation and daily
activities
○ Cushing’s disease is also associated with loss of bone density
and development of osteoporosis
● Encourage the client to increase dietary intake of calcium and vitamin
D
○ Cushing’s disease causes bones to be fragile therefore, calcium
and vitamin D helps to strengthen bones
ADDISON’S DISEASE
- Primary adrenal insufficiency
- Hyposecretion of hormones, specifically the ones from the adrenal cortex
○ Decreased glucocorticoids like cortisol
○ Decreased mineralocorticoids like aldosterone
○ Decreased androgen hormones
- Relatively rare, occurring most often in adults 30-60 yrs old although it may
appear at any time
- Caused by autoimmune mechanisms and is more common in women
- Most common cause:
○ Autoimmune destruction of the adrenal cortex
- Other causes:
○ Infections such as TB, fungal infections, HIV
○ Infiltrative diseases such as amyloidosis, metastatic carcinoma
○ Bilateral adrenal haemorrhage
- Pathophysiology:
○ Characterized by inadequate corticosteroid and mineralocorticoid
synthesis and elevated serum ACTH levels
○ 90% of total adrenocorticoid tissue must be destroyed before clinical
manifestations are evident
○ Adrenal glands are smaller and may be misshaped
- Idiopathic Addison’s Disease (organ specific autoimmune adrenalitis)
○ Causes adrenal atrophy and hypofunction
○ Often associated with other autoimmune diseases such as
■ Autoimmune polyendocrine syndrome (APS)
- Addisonian Crisis
○ An acute exacerbation of their disease; medical emergency
Clinical Manifestations
Clinical Manifestations Pathophysiologic Mechanism
Weakness and easy fatigability Not known, may be related to
hypoglycaemia, hypotension, or
decreased metabolism of proteins
GI disturbances: anorexia, nausea, May be associated with Celiac disease or
vomiting, diarrhea, abdominal pain, electrolyte abnormalities
weight loss
Hypoglycemia manifested by fatigue, Absence of cortisol
mental confusion, apathy, and psychosis
Hyperpigmentation Elevations of ACTH that lead to
stimulation of melanocytes
Vitiligo Autoimmune destruction of melanocytes
Addisonian crisis: severe hypotension Combined effects of hypocortisolism,
and vascular collapse hypoaldosteronism, extracellular volume
depletion, and some precipitating
stressors; decreased vasomotor tone
caused by cortisol deficiency
Treatment
The treatment of Addison’s disease involves lifetime glucocorticoid and
mineralocorticoid replacement therapy, together with dietary modification and
correction of any underlying disorders. In case Addisonian crisis occurs immediate IV
infusion of sodium chloride is given.
Pharmacologic therapy
● Glucocorticoid
○ Hydrocortisone, prednisone or methylprednisolone to replace cortisol.
These hormones are given on a schedule to mimic the normal 24-hour
fluctuation of cortisol levels
○ Take with meal or snack
○ High-dose glucocorticoids may cause insomnia; immediate-release
formulation is typically administered in the morning to coincide with
circadian rhythm
● Mineralocorticoid
○ Fludocrtisone acetate
○ Take this medication by mouth with or without food
○ Stomach upset, headache, and menstrual changes (e.g.,
delayed/irregular/absent periods) may occur
Non-pharmacologic therapy
● High Sodium Diet with increase fluid intake
○ It is highly encouraged especially during heavy exercise or during hot
weather to restore any electrolyte imbalance.
○ The individual’s diet should include at least 150 mEq of sodium per
day.
