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Cushing's Syndrome Treatment

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Treatment Î ES recommends that clinicians discuss and offer age-appropriate vaccinations to CS patients—particularly influenza, H. zoster, and pneumococcal vaccinations—due to an increased risk of infection. (US) First-line Treatment Options Î ES recommends initial resection of primary lesion(s) underlying Cushing's disease (CD), ectopic and adrenal (cancer, adenoma, and bilateral disease) etiologies, unless surgery is not possible or unlikely to significantly reduce glucocorticoid excess. (1|⊕⊕⊕⊕) • ES recommends unilateral resection by an experienced adrenal surgeon for all cases of benign unilateral disease. (1|⊕⊕⊕ ) • ES recommends localizing and resecting ectopic ACTH-secreting tumors with node dissection as appropriate. (1|⊕⊕⊕⊕) • ES recommends transsphenoidal selective adenomectomy (TSS) by an experienced pituitary surgeon as the optimal treatment for CD in pediatric and adult patients. (1|⊕⊕⊕⊕) ▶ ES recommends measuring serum sodium several times during the first 5–14 days after transsphenoidal surgery. (1|⊕⊕ ) ▶ ES recommends assessing free thyroxine and prolactin within 1–2 weeks of surgery, to evaluate for overt hypopituitarism. (1|⊕⊕ ) ▶ ES recommends obtaining a postoperative pituitary MRI within 1–3 months of successful TSS. (US) • ES recommends surgical resection of bilateral adrenal disorders (1|⊕⊕ ), and suggest medical therapy to block aberrant hormone receptors for bilateral macronodular adrenal hyperplasia (BMAH) (2|⊕⊕ ). Remission and Recurrence After Surgical Tumor Resection Î ES suggests an individualized management approach based on whether the post-operative serum cortisol values categorize the patient as hypocortisolism, hypercortisolism, or eucortisolism. (US) Î ES recommends additional treatments in patients with persistent overt hypercortisolism. (1|⊕⊕⊕⊕) Î ES recommends measuring late-night salivary or serum cortisol in patients with eucortisolism after TSS, including those cases where eucortisolism was established by medical treatment before surgery. (1|⊕⊕ ) Î ES recommends using tests to screen for hypercortisolism to assess for recurrence in patients with ACTH-dependent CS. (1|⊕⊕⊕ ) Glucocorticoid Replacement and Discontinuation, and Resolution of Other Hormonal Deficiencies Î ES recommends that hypocortisolemic patients receive glucocorticoid replacement and education about adrenal insufficiency after surgical remission. (1|⊕⊕⊕⊕)

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