Nelson Syndrome Medication
- Author: Thomas A Wilson, MD; Chief Editor: Stephen Kemp, MD, PhD more...
Because most patients who develop Nelson syndrome have had bilateral adrenalectomy, they need to have adrenal steroid replacement.
Steroid hormones with multiple activities are used for replacement therapy in patients with adrenal insufficiency or for the therapy of many diverse diseases.
Hydrocortisone is the glucocorticoid of choice for replacing cortisol. Its short half-life necessitates a twice-daily dose schedule. The morning dose should be the largest, administering approximately two thirds of the total daily dose immediately after waking. A second dose is usually administered in the early afternoon. Longer-acting glucocorticoids are not recommended until growth is complete because they can compromise growth. In children who have stopped growing and in adults, prednisone and dexamethasone can be administered. Dexamethasone has the advantage of daily dosing but is unsuitable for long-term replacement therapy in children.
Mineralocorticoid replacement is necessary to maintain circulatory volume, blood pressure, and prevent hyperkalemia and hyponatremia in patients that have mineralocorticoid deficiency. Inadequate mineralocorticoid replacement results in increased glucocorticoid requirement that may make the patient cushingoid.
Fludrocortisone acetate possesses very little glucocorticoid activity at recommended doses. The dose requirement is determined by measuring blood pressure (hypertension indicates overreplacement) and supine plasma renin activity (PRA). Suppression of PRA indicates overreplacement and elevation indicates underreplacement. Dosages vary considerably among individuals and must be tailored to the individual patient. They can vary from 50-500 mcg/d. Dose adjustment is typically not required for acute illness, although some physicians advocate increasing the dose for severe GI illnesses.
Dopamine receptor agonist
Cabergoline is a long-acting dopamine receptor agonist that has a high affinity for D2 receptors. It has been used as an oral medication for the treatment of hyperprolactinemia. Cabergoline has been reported in 2 case reports to control serum adrenocorticotropin (ACTH) concentrations with appreciable tumor shrinkage or disappearance in Nelson syndrome.
Cabergoline acts on dopamine receptors, with a high affinity to the D2 receptor. It inhibits growth hormone secretion and has a multitude of other endocrine and nonendocrine effects, including inhibition of glucagon, VIP, and GI peptides.
Octreotide, like natural somatostatin, inhibits growth hormone secretion, insulin secretion, and glucagon secretion. Following intravenous administration, basal serum growth hormone, insulin, and glucagon levels are lowered. It also inhibits prolactin secretion via vasoactive intestinal peptide–mediated and thyrotropin-releasing hormone–mediated secretion of prolactin. Used to treat acromegaly and hormone-secreting tumors. When used in Nelson syndrome, octreotide is only useful as a means of controlling serum ACTH concentrations until definitive treatment is undertaken because it does not result in appreciable tumor shrinkage.
Octreotide acts primarily on somatostatin receptor subtypes II and V. It inhibits growth hormone secretion and has a multitude of other endocrine and nonendocrine effects, including inhibition of glucagon, VIP, and GI peptides.
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