Gigantism and Acromegaly Medication

  • Author: Alicia Diaz-Thomas, MD, MPH; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: May 18, 2012
 

Medication Summary

Analogs of somatostatin are the most effective medical therapies for GH excess. Dopamine-receptor agonists are best used as adjuvant treatments.

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Somatostatin analogs

Class Summary

Like natural somatostatin, octreotide inhibits the secretion of GH, insulin, and glucagon. After an intravenous administration, basal serum GH, insulin, and glucagon levels fall. Octreotide also inhibits PRL release by means of vasoactive intestinal peptide (VIP)-mediated and TRH-mediated secretion of PRL. Octreotide is used to treat acromegaly and several hormone-secreting tumors.

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Octreotide (Sandostatin, Sandostatin LAR)

 

Effectively lowers serum GH and IGF-I concentrations; may shrink tumor. Forty times more potent than native somatostatin to inhibit GH secretion. Available in immediate-release (Sandostatin) or long-acting depot (Sandostatin LAR) form.

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Dopamine agonists

Class Summary

Dopamine-receptor agonists are other pharmacologic options. However, these drugs are effective in only a few patients. Cabergoline is well tolerated.

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Bromocriptine (Parlodel)

 

Dopamine agonist most often used to treat GH and PRL excess. Safe when administered to a child for extended period.

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Cabergoline (Dostinex)

 

Potent dopamine agonist with prolonged duration of action. Inhibits PRL secretion more than bromocriptine.

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GH-receptor antagonist

Class Summary

This novel class of drugs have demonstrated efficacy to suppress GH and IGF-I levels.

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Pegvisomant (Somavert, B2036-PEG)

 

Recombinant DNA analog of human GH structurally altered to act as GH receptor antagonist. Selectively binds to GH receptors on cell surfaces, blocking endogenous GH binding. Thus interferes with GH signal transduction, decreasing levels of IGF-I, IGFBP-3, and acid-labile subunit.

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Contributor Information and Disclosures
Author

Alicia Diaz-Thomas, MD, MPH  Assistant Professor of Pediatrics, University of Tennessee Health Science Center, Memphis

Alicia Diaz-Thomas, MD, MPH is a member of the following medical societies: American Academy of Clinical Endocrinology, Endocrine Society, and Tennessee Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Melanie Shim, MD  Clinical Instructor, Department of Pediatrics, Division of Pediatric Endocrinology, University of California at Los Angeles School of Medicine

Melanie Shim, MD is a member of the following medical societies: American Diabetes Association and Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Phyllis W Speiser, MD  Chief, Division of Pediatric Endocrinology, Steven and Alexandra Cohen Children's Medical Center of New York; Professor of Pediatrics, Hofstra-North Shore LIJ School of Medicine at Hofstra University

Phyllis W Speiser, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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.

Barry B Bercu, MD  Professor, Departments of Pediatrics, Molecular Pharmacology and Physiology, University of South Florida College of Medicine, All Children's Hospital

Barry B Bercu, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Federation for Clinical Research, American Medical Association, American Pediatric Society, Association of Clinical Scientists, Endocrine Society, Florida Medical Association, Pediatric Endocrine Society, Pituitary Society, Society for Pediatric Research, Society for the Study of Reproduction, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

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.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Robert J Ferry Jr MD to the development and writing of this article.

References

  1. Izzard AS, Emerson M, Prehar S, et al. The cardiovascular phenotype of a mouse model of acromegaly. Growth Horm IGF Res. Oct 2009;19(5):413-9. [Medline].

  2. van der Lely AJ, Biller BM, Brue T, et al. Long-Term Safety of Pegvisomant in Patients with Acromegaly: Comprehensive Review of 1288 Subjects in ACROSTUDY. J Clin Endocrinol Metab. May 2012;97(5):1589-97. [Medline].

  3. Higham CE, Atkinson AB, Aylwin S, et al. Effective combination treatment with cabergoline and low-dose pegvisomant in active acromegaly: a prospective clinical trial. J Clin Endocrinol Metab. Apr 2012;97(4):1187-93. [Medline].

  4. Rix M, Laurberg P, Hoejberg AS, Brock-Jacobsen B. Pegvisomant therapy in pituitary gigantism: successful treatment in a 12-year-old girl. Eur J Endocrinol. Aug 2005;153(2):195-201. [Medline].

  5. Weber DC, Momjian S, Pralong FP, Meyer P, Villemure JG, Pica A. Adjuvant or radical fractionated stereotactic radiotherapy for patients with pituitary functional and nonfunctional macroadenoma. Radiat Oncol. Dec 8 2011;6:169. [Medline]. [Full Text].

  6. Abe T, Tara LA, Ludecke DK. Growth hormone-secreting pituitary adenomas in childhood and adolescence: features and results of transnasal surgery. Neurosurgery. Jul 1999;45(1):1-10. [Medline].

  7. Ali O, Banerjee S, Kelly DF, Lee PD. Management of type 2 diabetes mellitus associated with pituitary gigantism. Pituitary. 2007;10(4):359-64. [Medline].

  8. Barkan AL, Burman P, Clemmons DR, et al. Glucose homeostasis and safety in patients with acromegaly converted from long-acting octreotide to pegvisomant. J Clin Endocrinol Metab. 2005;90:5684-5691. [Medline].

  9. Bera TK, Liu XF, Yamada M, Gavrilova O, Mezey E, Tessarollo L, et al. A model for obesity and gigantism due to disruption of the Ankrd26 gene. Proc Natl Acad Sci U S A. 2008;105(1):270-5. [Medline].

  10. Bonapart IE, van Domburg R, ten Have SM, et al. The 'bio-assay' quality of life might be a better marker of disease activity in acromegalic patients than serum total IGF-I concentrations. Eur J Endocrinol. 2005;152:217-224. [Medline].

  11. Cazabat L, Libe R, Perlemoine K, et al. Germline inactivating mutations of the aryl hydrocarbon receptor-interacting protein gene in a large cohort of sporadic acromegaly: mutations are found in a subset of young patients with macroadenomas. Eur J Endocrinol. Jul 2007;157(1):1-8. [Medline].

  12. Clemmons DR, Chihara K, Freda PU, et al. Optimizing control of acromegaly: integrating a growth hormone receptor antagonist into the treatment algorithm. J Clin Endocrinol Metab. Oct 2003;88(10):4759-67. [Medline].

  13. Cozzi R, Attanasio R, Barausse M, et al. Cabergoline in acromegaly: a renewed role for dopamine agonist treatment?. Eur J Endocrinol. Nov 1998;139(5):516-21. [Medline].

  14. Davoodi J, Kelly J, Gendron NH, MacKenzie AE. The Simpson-Golabi-Behmel syndrome causative glypican-3, binds to and inhibits the dipeptidyl peptidase activity of CD26. Proteomics. Jun 2007;7(13):2300-10. [Medline].

  15. Duncan E, Wass JA. Investigation protocol: acromegaly and its investigation. Clin Endocrinol (Oxf). Mar 1999;50(3):285-93. [Medline].

  16. Eugster EA, Pescovitz OH. Gigantism. J Clin Endocrinol Metab. Dec 1999;84(12):4379-84. [Medline].

  17. Ezzat S, Serri O, Chik CL et al. Canadian consensus guidelines for the diagnosis and management of acromegaly. Clin Invest Med. 2006;29:29-39. [Medline].

  18. Fuqua JS, Berkovitz GD. Growth hormone excess in a child with neurofibromatosis type 1 and optic pathway tumor: a patient report. Clin Pediatr (Phila). Dec 1998;37(12):749-52. [Medline].

  19. Gagel RF. Multiple endocrine neoplasia. In: Williams Textbook of Endocrinology. 9th ed. 1627-1649.

  20. Geffner ME, Nagel RA, Dietrich RB, Kaplan SA. Treatment of acromegaly with a somatostatin analog in a patient with McCune-Albright syndrome. J Pediatr. Nov 1987;111(5):740-3. [Medline].

  21. Giustina A, Barkan A, Casanueva FF, et al. Criteria for cure of acromegaly: a consensus statement. J Clin Endocrinol Metab. Feb 2000;85(2):526-9. [Medline].

  22. Herman V, Fagin J, Gonsky R, et al. Clonal origin of pituitary adenomas. J Clin Endocrinol Metab. Dec 1990;71(6):1427-33. [Medline].

  23. Herman-Bonert VS, Zib K, Scarlett JA, Melmed S. Growth hormone receptor antagonist therapy in acromegalic patients resistant to somatostatin analogs. J Clin Endocrinol Metab. Aug 2000;85(8):2958-61. [Medline].

  24. Holl RW, Bucher P, Sorgo W, et al. Suppression of growth hormone by oral glucose in the evaluation of tall stature. Horm Res. 1999;51(1):20-4. [Medline].

  25. Keil MF, Stratakis CA. Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics. Expert Rev Neurother. Apr 2008;8(4):563-74. [Medline].

  26. Kirschner LS, Carney JA, Pack SD, et al. Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex. Nat Genet. Sep 2000;26(1):89-92. [Medline].

  27. Kunwar S, Wilson CB. Pediatric pituitary adenomas. J Clin Endocrinol Metab. Dec 1999;84(12):4385-9. [Medline].

  28. Lissett CA, Peacey SR, Laing I, et al. The outcome of surgery for acromegaly: the need for a specialist pituitary surgeon for all types of growth hormone (GH) secreting adenoma. Clin Endocrinol (Oxf). Nov 1998;49(5):653-7. [Medline].

  29. Malan V, De Blois MC, Prieur M, et al. Sotos syndrome caused by a paracentric inversion disrupting the NSD1 gene. Clin Genet. Jan 2008;73(1):89-91. [Medline].

  30. Melmed S. Medical progress: Acromegaly. N Engl J Med. Dec 14 2006;355(24):2558-73. [Medline].

  31. Melmed S, Casanueva F, Cavagnini F, Chanson P, Frohman LA, Gaillard R, et al. Consensus statement: medical management of acromegaly. Eur J Endocrinol. Dec 2005;153(6):737-40. [Medline].

  32. Melmed S, Casanueva F, Cavagnini F, et al. Consensus statement: medical management of acromegaly. Eur J Endocrinol. 2005;153:737-740. [Medline].

  33. Miyazaki R, Yoshida T, Sakane N, et al. Acromegalic gigantism with low serum level of growth hormone and elevated serum insulin-like growth factor-I. Intern Med. Mar 1995;34(3):183-7. [Medline].

  34. Moran A, Pescovitz OH. Long-term treatment of gigantism with combination octreotide and bromocriptine in a child with McCune-Albright syndrome. Endocr J. 1994;2:111-113.

  35. Mussig K, Gallwitz B, Honegger J, et al. Pegvisomant treatment in gigantism caused by a growth hormone-secreting giant pituitary adenoma. Exp Clin Endocrinol Diabetes. Mar 2007;115(3):198-202. [Medline].

  36. Nanto-Salonen K, Koskinen P, Sonninen P, Toppari J. Suppression of GH secretion in pituitary gigantism by continuous subcutaneous octreotide infusion in a pubertal boy. Acta Paediatr. Jan 1999;88(1):29-33. [Medline].

  37. Orme SM, McNally RJ, Cartwright RA, Belchetz PE. Mortality and cancer incidence in acromegaly: a retrospective cohort study. United Kingdom Acromegaly Study Group. J Clin Endocrinol Metab. Aug 1998;83(8):2730-4. [Medline].

  38. Ray M, Malhi P, Bhalla AK, Singhi PD. Cerebral gigantism with West syndrome. Indian Pediatr. Jul 2003;40(7):673-5. [Medline].

  39. Schmidt H, Kammer B, Grasser M, Enders A, Rost I, Kiess W. Endochondral gigantism: a newly recognized skeletal dysplasia with pre- and postnatal overgrowth and endocrine abnormalities. Am J Med Genet A. Aug 15 2007;143(16):1868-75. [Medline].

  40. Schwartz TH, Stieg PE, Anand VK. Endoscopic transsphenoidal pituitary surgery with intraoperative magnetic resonance imaging. Neurosurgery. 2006;58:44-51. [Medline].

  41. Sotos JF. Overgrowth. Hormonal Causes. Clin Pediatr (Phila). Nov 1996;35(11):579-90. [Medline].

  42. Stratakis CA, Carney JA, Lin JP, et al. Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2. J Clin Invest. Feb 1 1996;97(3):699-705. [Medline].

  43. Thapar K, Kovacs K, Stefaneanu L, et al. Overexpression of the growth-hormone-releasing hormone gene in acromegaly-associated pituitary tumors. An event associated with neoplastic progression and aggressive behavior. Am J Pathol. Sep 1997;151(3):769-84. [Medline].

 
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Image shows the coauthor with a statue of Robert Wadlow, who was called the Alton giant. He was the tallest person ever recorded and was 8 feet 11 inches tall at the time of his death.
Photograph shows a 12-year-old boy with McCune-Albright syndrome. His growth-hormone excess manifested as tall stature, coarse facial features, and macrocephaly.
 
 
 
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