Cushing Syndrome and Cushing Disease PDF

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Professor Rounds-Stanley...

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Cushing Syndrome and Cushing Disease Definition: Also called adrenocortical hyperfunction, is characterized by a group of symptoms resulting from excess blood levels of glucocorticoids (especially cortisol). Cushing disease is caused a pituitary tumor. Etiology/Pathophysiology:  Prolonged administration of glucocorticoid hormones  During infancy, most cases of endogenous Cushing syndrome are due to an adrenocortical tumor  Most common cause of endogenous Cushing syndrome in children older 7 is Cushing disease in which a pituitary tumor (adenoma) secretes excess ACTH  bilateral adrenal hyperplasia Clinical manifestations:  Obesity  Excessive weight gain is followed by slow linear growth  Child develops the characteristic cushingoid features that include rounded (moon) face w/ prominent cheeks  Additional manifestations include hirsutism, acne, deepening of the voice, and HTN  Older children may experience delayed puberty, irregular menstrual periods, headaches, weakness, pathologic fractures, emotional problems, and hyperglycemia Diagnostic tests:  Based on characteristic physical findings and lab values, including increased 24-hour urinary levels of free cortisol and elevated nighttime salivary cortisol levels  Child will also have an abnormal glucose tolerance test  Adrenal suppression test with an 11 p.m dose of dexamethasone reveals that adrenal cortisol output is not suppressed overnight  CT and MRI are used to detect the specific location of tumors in the adrenal and pituitary glands Clinical therapy:  Surgical removal of the pituitary adenoma is the current tx of choice  Irradiation of the pituitary is performed when surgical removal of the adenoma does not substantially reduce cortisol levels  Bilateral removal of the adrenal glands may be necessary in some cases to stop the excessive secretion  Lifelong hormone replacement is required when both adrenal glands are removed Nursing diagnoses:  Fluid Volume: Excess r/t elevated serum sodium and fluid retention  Infection, Risk for r/t surgical incision  Body Image, Disturbed r/t body changes  Anxiety (Child and Parent) r/t surgical procedure and serious disorder Nursing interventions:  Monitor VS, fluid status, nutritional status, and weight  Additional assessments include muscle strength and endurance during hospital play activities  Explain to child and parents that cushingoid appearance is reversible with tx  Elevate HOB 30 to promote effective breathing; prevent tension on the incision line during repositioning  Administration of cortisol replacement therapy is best early in the morning or every other day b/c it causes fewer symptoms and mimics the normal diurnal pattern of the cortisol secretion  Hydrocortisone (Cortef, Solu-cortef, cortisone acetate) is available in liquid, tablet, or injectable forms  Educate parents on crushing tablets and mixing with small amount of applesauce due to the bitter taste  Giving the dose at mealtimes and using antacids between meals helps reduce GI irritation (SE) of cortisone  Injectable form is usually administered when the child is vomiting, has diarrhea, or cannot take the oral medication  Failure to give medication when the child is ill may lead to severe illness and cardiovascular collapse...