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Nelson Syndrome Medication

  • Author: Thomas A Wilson, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: Dec 11, 2015
 

Medication Summary

Because most patients who develop Nelson syndrome have had bilateral adrenalectomy, they need to have adrenal steroid replacement.

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Glucocorticoids

Class Summary

Steroid hormones with multiple activities are used for replacement therapy in patients with adrenal insufficiency or for the therapy of many diverse diseases.

Hydrocortisone (Hydrocortone, Hydrocort, Cortef, Solu-Cortef)

 

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.

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Mineralocorticoids

Class Summary

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 (Florinef)

 

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.

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Dopamine receptor agonist

Class Summary

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 (Dostinex)

 

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.

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Somatostatin analogs

Class Summary

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 (Sandostatin)

 

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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Contributor Information and Disclosures
Author

Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, American College of Endocrinology

Disclosure: Nothing to disclose.

Antony Lafferty, MB, BCh 

Antony Lafferty, MB, BCh is a member of the following medical societies: Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Lynne Lipton Levitsky, MD Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor of Pediatrics, Harvard Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Received grant/research funds from Eli Lilly for pi; Received grant/research funds from NovoNordisk for pi; Received consulting fee from NovoNordisk for consulting; Partner received consulting fee from Onyx Heart Valve for consulting.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Angelo P Giardino, MD, MPH, PhD Professor and Section Head, Academic General Pediatrics, Baylor College of Medicine; Senior Vice President and Chief Quality Officer, Texas Children’s Hospital

Angelo P Giardino, MD, MPH, PhD is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, Harris County Medical Society, International Society for the Prevention of Child Abuse and Neglect, Ray E Helfer Society

Disclosure: Received grant/research funds from Health Resources and Services Administration (HRSA) Integrated Community Systems for CSHCN Grant for other; Received advisory board from Baxter Healthcare Corporation for board membership.

Acknowledgements

Vardhini Desikan, MBBS, MPH Pediatric Endocrinology Fellow, Department of Pediatrics, State University of New York at Stony Brook

Vardhini Desikan, MBBS, MPH is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

References
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