Nelson Syndrome Medication

  • Author: Thomas A Wilson, MD; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Mar 5, 2012
 

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.

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

 

Glucocorticoid of choice for replacing cortisol. Short half-life necessitates bid 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.

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Fludrocortisone acetate (Florinef)

 

Possesses very little glucocorticoid activity at recommended doses. Dose requirement determined by measuring blood pressure (hypertension indicates overreplacement) and supine 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.

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Cabergoline (Dostinex)

 

Acts on dopamine receptors, with high affinity to the D2 receptor. Inhibits GH 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.

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Octreotide (Sandostatin)

 

Acts primarily on somatostatin receptor subtypes II and V. Inhibits GH 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, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

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.

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 Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Antony Lafferty, MB ChB, FRACP  Senior Lecturer of Pediatric Endocrinology, Monash University Department of Pediatrics, National Institutes of Health, Bethesda, MD, and Princess Margaret Hospital for Children, Perth, Western Australia

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Angelo P Giardino, MD, PhD, MPH  Associate Professor, Baylor College of Medicine; Chief Medical Officer, Texas Children's Health Plan; Chief Quality Officer, Medicine, Texas Children's Hospital

Angelo P Giardino, MD, PhD, MPH 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, Helfer Society, and International Society for Prevention of Child Abuse and Neglect

Disclosure: Bayer Honoraria Review panel membership; Pfizer Grant/research funds Independent contractor; MedImmune Honoraria Review panel membership; Teva Pharmacutical travel & honoraria Managed Care Advisory Panel; CIGNA Honoraria Physician Advisory Council

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, and Society for Pediatric Research

Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds Other; Eli Lily Grant/research funds PI; NovoNordisk Grant/research funds PI; NovoNordisk Consulting fee Consulting; Onyx Heart Valve Consulting fee Consulting

Merrily P M Poth, MD  Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences

Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  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, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

References

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