Cushing’s Syndrome

Cushing's syndrome is caused by long-term exposure to too much cortisol, a

hormone that the adrenal gland makes. The adrenal glands may overproduce
corticosteroids because of a problem in the adrenal glands or because of too
much stimulation from the pituitary gland. An abnormality in the pituitary
gland, such as a tumor, can cause the pituitary to produce large amounts of
corticotropin, the hormone that controls the production of corticosteroids
from the adrenal glands. Tumors outside the pituitary gland, such as small-
cell lung cancer, can produce corticotropin as well (a condition called
ectopic corticotropin syndrome). Corticotropin may also be produced by a
tumor called a carcinoid, which may occur almost anywhere in the body.
Sometimes a noncancerous tumor (adenoma) develops in the adrenal glands,
which causes them to overproduce corticosteroids. Adrenal adenomas are
extremely common. Half of all people have them by the age of 70. Only a
small fraction of adenomas produce excess hormone, however. Cancerous
tumors of the adrenal glands are very rare.

People who are obese, and have type-2 diabetes along with poorly-
controlled blood glucose and high blood pressure have an increased risk of
developing this disorder. Cushing's syndrome can also develop in people
who must take large doses of corticosteroids because of a serious medical
condition. Those who must take large doses have the same symptoms as
those who produce too much of the hormone. The symptoms can
occasionally occur even if the corticosteroids are inhaled, as for asthma, or
are used topically for a skin condition.

Pituitary Cushing’s syndrome occurs after puberty with equal frequency in

boys and girls. In adults, it has a greater frequency in women than men, with
most diagnosed between ages 20 and 50 years. The total incidence is about
10–15 million people per year. It is a disorder characterized by virilism,
upper body obesity with thin arms and legs, hyperglycemia, glucosuria,
hypertension, red moon face, vertigo, emotional lability, buffalo hump,
purple striae over obese areas, acne, female balding, blurry vision, pitting
ankle edema. In some cases osteoporosis and severe depression may also be
present. Women with Cushing’s syndrome usually have excess hair growth
on their face, neck, chest, abdomen, and thighs (hirsutism). Their menstrual
periods may become irregular or stop. Men may have decreased fertility
with diminished or absent desire for sex and, sometimes, erectile
dysfunction.

Although Cushing’s disease is rare, differential diagnosis can be difficult.

No existing test when used alone is accurate. When doctors suspect
Cushing's syndrome, they measure the level of cortisol, the main
corticosteroid hormone, in the blood. Normally, cortisol levels are high in
the morning and lower late in the day. In people who have Cushing's
syndrome, cortisol levels are very high throughout the day.

If the cortisol levels are high, doctors may recommend a dexamethasone

suppression test (LDDST–Low dose dexamethasone suppression test).
Dexamethasone suppresses the pituitary gland and should lead to
suppression of cortisol secretion by the adrenal glands. If Cushing's
syndrome is caused by too much pituitary stimulation, the level of cortisol
will fall to some extent, although not as much as in people who do not have
Cushing's syndrome. If Cushing's syndrome has another cause, the level of
cortisol will remain high. A high corticotropin level further suggests
overstimulation of the adrenal gland. Apart from this, some of the common
lab tests include 24-hour urinary free cortisol test, measurement of midnight
plasma cortisol or late-night salivary cortisol. Another test, the
dexamethasone-corticotropin-releasing hormone test, may be needed to
distinguish Cushing’s syndrome from other causes of excess cortisol.

Imaging tests may be needed to determine the exact cause, including a

computed tomography (CT) or magnetic resonance imaging (MRI) scan of
the pituitary or adrenal glands and a chest X-ray or CT scan of the lungs.
However, these tests may occasionally fail to find the tumor.

When overproduction of corticotropin is thought to be the cause, blood

samples may be taken from the veins that drain the pituitary to see if that is
the source.

If left untreated, Cushing’s syndrome can be fatal. Treatments differ

according to the cause; adrenocorticotropic hormone (ACTH) dependent
(pituitary or ectopic) or independent (an adrenal tumor) or iatrogenic (from
excessive steroid hormone use). If iatrogenic, depletion of steroid hormones
will be needed. If pituitary, the gland may need to be removed.
Cushing’s syndrome caused by ACTH production from solid tumors can
result in life threatening hypercortisolemia. Radiation, chemotherapy, or
surgery may be needed. After surgical removal of the adrenal glands most
symptoms of Cushing’s syndrome disappear, but psychological problems
could persist, despite successful treatment.

The fact that obesity is a prominent feature of Cushion’s syndrome has

brought about investigation as to whether hypercortisolism and its enzymatic
overactivity is the cause or a result of the obesity if still unclear.

Anyone who has Cushing’s disease is at risk for metabolic syndrome.

Chromic cortisol hypersecretion causes central obesity, hypertension, insulin
resistance, dyslipidemia, and prothrombotic state. Interventions must be
developed for these patients that include plans to manage elevated blood
glucose, diabetes, and cardiovascular disease.

For food & nutrition therapy:

• Weight management/glucose control (carb counting/calorie

controlled-diet)

• Management of metabolic syndrome with diet & exercise (carb

counting, cal controlled diet, exercise)

• Ensure foods high in calcium and potassium.

• Ensure adequate intake of protein if losses are excessive (e.g., 1 g

protein /kg or more)

• Restriction of sodium if steroids are used

• With glucocorticoid therapy osteoporosis and hypercalciuria are

common side effects—Large doses of Vit D may be necessary do not
use for extended periods of time because toxicity may occur.

References

Mahan, Escott-Stump: Krause’s Food, Nutrition & Diet Therapy, 12th Ed., WB Saunders 2008

Sylvia Escott-Stump: Nutrition Diagnosis Related Care, 6th Edition, Lippincott-Williams & Wilkins, 2007
http://www.merck.com/mmhe/sec13/ch164/ch164c.html

http://www.nlm.nih.gov/medlineplus/cushingssyndrome.html

http://www.endocrine.niddk.nih.gov/pubs/cushings/cushings.htm