Cushing Syndrome

• ● Overproduction of cortisol from the adrenal cortex

  ● Two Possible Reasons: usually results from increased ACTH production due to a pituitary tumor or from a tumor of the adrenal cortex

  ● The peak age is 6-7 years old but it may occur as early as infancy.
• DIAGNOSIS

  ● The manifestations are all because of the high cortisol effect.

  1. Dexamethasone Suppression Test

  ● If without syndrome, giving dexamethasone (a glucocorticoid) will lower cortisol. It will not fall in children with adrenal tumor because the tumor continues to stimulate the adrenal gland to secrete cortisol
• 2. Cosyntropin (ACTH) Administration

  ● Normally, cortisol will rise but in patient with an adrenal tumor, the gland is already at full capacity so no more cortisol elevation will occur with ACTH administration in a child with cushing syndrome.

  3. CT Scan or Ultrasound

  ● Reveals enlarged adrenal or pituitary gland

  ○ Usually because of the tumor.
• ASSESSMENT

  ● Some children taking high doses of synthetic corticosteroids (e.g. prednisone) may develop the same symptoms in Cushing Syndrome. That’s why sometimes the side effects of these drugs are said to have a Cushingoid appearance.

  1. Increased glucose production causes fat to accumulate in cheeks, chin, and trunk, causing a moon-faced, stocky appearance.

  ● This also lead to polyuria as glucose finds its way out of the body through the urine

  2. Catabolic effects cause protein and muscle wasting, making extremities thin.

  ● Give children a higher risk for osteoporosis.
• 3. Steroids suppresses immune system leaving children susceptible to infection.

  4. Causes vasoconstriction, and extreme hypertension can occur.

  5. Hyperpigmentation occurs from melanin stimulating properties of ACTH

  ● High ACTH stimulation causes the child's face to be unusually red, especially the cheeks.
• 6. Abnormal masculinization or feminization may occur because of the overproduction of androgen and estrogen.

  7. Purple striae resulting from collagen deficit and appear on hips, abdomen, and thighs.

  8. Growth ceases, so short stature results.
• MANAGEMENT

  ● Treatment: surgical removal of the causative tumor

  ● Prognosis: depends on whether the tumor is benign or malignant

  ○ Carcinoma Type: tends to metastasize rapidly

  1. If a part of the adrenal gland is removed, the child will need cortisol replacement indefinitely.

  ● Watch out for signs of shock if epinephrine secretion is decreased from the adrenal medulla

  ○ The body’s ability to maintain blood pressure is severely compromised, and severe hypotension can result.

  2. If a part of the pituitary gland is removed, replacement of all pituitary hormones may be necessary.