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CUSHING'S SYNDROME

DESCRIPTION

Cushing's syndrome is an endocrine disorder caused by excess adrenal hormones. Children who have been treated for any problem necessitating prolonged use of cortisone drugs will develop a form of Cushing's syndrome. These children require greatly increased cortisone treatment during any physiological crisis, such as serious injury or severe infection. The adrenal gland (located over the kidney) and pituitary gland (at the base of the brain) are involved. Cushing's syndrome is more common in adults than children and more common in females than males.
Appropriate health care includes: physician's monitoring of general condition and medications, including consultation with an endocrinologist; surgery (sometimes) to remove ACTH-producing tumors from the pituitary or to remove adrenal-gland tumors; hospitalization for high-voltage radiation treatment of the pituitary gland (sometimes); hospitalization following any crisis such as a broken bone or severe infection.

SIGNS & SYMPTOMS
Round face and puffy eyes; ruddy red complexion; growth of facial hair in females; fat accumulation over the upper back and trunk, accompanied by red "stretch marks"; high blood pressure; mental and emotional changes, including psychosis; menstrual changes, including cessation of, increased, or irregular periods; enlarged clitoris; diabetes mellitus; peptic ulcers; osteoporosis; low resistance to infection.

CAUSES
Symptoms and signs result from overproduction of the cortisone-like hormone produced by the adrenal glands. The overproduction may result from: a tumor in the adrenal glands; a pituitary tumor, causing production of excessive ACTH (adreno-cortico-tropic-hormone), which the pituitary gland produces to stimulate adrenal glands to secrete hormones; prolonged use of cortisone drugs.

RISK FACTORS
Prolonged use of ACTH for treatment of pituitary cancer.

PREVENTING COMPLICATIONS OR RECURRENCE

If use of ACTH or cortisone is necessary for other disorders, such as asthma, arthritis, kidney disease, or Addison's disease, your child should take the lowest dose possible for the shortest time. Consult your doctor.

BASIC INFORMATION

MEDICAL TESTS

  • Pictures taken before symptoms begin are helpful in noting changes in appearance; medical history and physical exam by a doctor; laboratory blood and urine studies of white-blood-cell counts, pituitary and adrenal-gland function and hormone levels; X-rays of the pituitary and adrenal glands.
  • Special studies that may include ultrasonography, CAT or CT scan, MRI, and radionuclide scan (See Glossary for all).

    POSSIBLE COMPLICATIONS

    Bone fractures due to osteoporosis; pituitary tumor, if adrenal glands are removed (rare); emergencies caused by injury, such as a broken bone or severe infection.

    PROBABLE OUTCOME

  • If caused by an adrenal-gland tumor, the disorder is curable with removal of the tumor or glands. Lifelong, carefully monitored drug therapy is essential if the glands are removed.
  • If caused by a pituitary tumor, the disorder is curable with removal of the tumor, but tumors may recur.
  • If caused by prolonged use of cortisone drugs or ACTH, the condition may improve if these are withdrawn gradually under medical supervision.

    TREATMENT

    HOME CARE

  • Learn all you can about this condition and its treatment. You must often monitor your child's reactions to medications. Discontinuing drugs suddenly is dangerous.
  • Have your child wear a Medic-Alert bracelet or pendant (See Glossary).
  • Protect your child from fractures. Accident-proof your home. Urge your child to wear seat belts in autos and not to take risks.

    MEDICATION
    Your doctor may prescribe: drugs, such as aminoglutethimide or mitotane, to suppress adrenal-gland function; cortisone drugs, if adrenal glands must be removed surgically; drugs to replace pituitary hormones (sometimes); anti-hypertensive drugs to lower blood pressure; calcium supplements to treat osteoporosis.

    ACTIVITY
    No restrictions. The child's energy will increase once treatment begins.

    DIET & FLUIDS
    A low-salt diet (See Appendix 29) is sometimes necessary. Consult your doctor.

    OK TO GO TO SCHOOL?

    Yes, when condition is stable.

    CALL YOUR DOCTOR IF

  • Your child has symptoms of Cushing's syndrome.
  • Your child has signs of infection, such as fever, chills, muscle aches, headache, and dizziness. ‡
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