Albright Syndrome Medication

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Sep 21, 2011
 

Medication Summary

A variety of medications may be required to correct various endocrine and metabolic derangements. Some of these include medroxyprogesterone acetate, testolactone, bromocriptine, propylthiouracil, ergocalciferol, and calcitonin. A qualified endocrinologist should conduct therapy.

Feuillan et al[37] reported on a pilot study of letrozole treatment for precocious puberty in girls with the MAS. Bisphosphonate therapy may have a role in the treatment of fibrous dysplasia.[38] Somatostatin analogs are useful in some, but not all, cases. The GH receptor antagonist pegvisomant can be useful in normalizing insulinlike growth factor-I levels.[2]

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Hormones

Class Summary

Given to correct endocrine disorders associated with sexual precocity manifestations (98% of cases), such as pubarche, menarche, and thelarche.

Medroxyprogesterone (Provera)

 

Progestins stop endometrial cell proliferation, allowing organized sloughing of cells after withdrawal. Typically does not stop acute bleeding episode but produces normal bleeding episode following withdrawal.

Testolactone (Teslac)

 

Synthetic peripheral aromatase inhibitor that blocks production of estradiol and estrone from testosterone and androstenedione.

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Ergot alkaloids

Class Summary

Some agents have dopaminergic properties that inhibit prolactin secretion.

Bromocriptine (Parlodel)

 

Semisynthetic ergot alkaloid derivative; strong dopamine D2-receptor agonist; partial dopamine D1-receptor agonist; indicated for amenorrhea/galactorrhea secondary to hyperprolactinemia in the absence of primary tumor.

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Antithyroid agents

Class Summary

Used in the palliative treatment of hyperthyroidism.

Propylthiouracil (PTU)

 

Derivative of thiourea that inhibits organification of iodine by thyroid gland. Blocks oxidation of iodine in thyroid gland, thereby inhibiting thyroid hormone synthesis; inhibits T4 to T3 conversion (advantage over other agents).

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Metabolic agents

Class Summary

Agents (eg, vitamin D) are indicated to correct deficiencies leading to hypoparathyroidism. Agents (eg, calcitonin) are indicated to treat hypercalcemia and prevent bone loss.

Ergocalciferol (Calciferol, Drisdol)

 

Stimulates absorption of calcium and phosphate from small intestine and promotes release of calcium from bone into blood.

Calcitonin (Miacalcin, Osteocalcin)

 

Lowers elevated serum calcium level in patients with primary hyperparathyroidism. Expect a higher response when serum calcium levels are high. Onset of action is approximately 2 h following injection and activity lasts for 6-8 h. May lower calcium levels for 5-8 d by approximately 9% if given q12h. IM route is preferred at multiple injection sites with dose >2 mL.

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Optigenex Consulting fee Independent contractor

Specialty Editor Board

Eleanor E Sahn, MD  Director, Division of Pediatric Dermatology, Associate Professor, Departments of Dermatology and Pediatrics, Medical University of South Carolina

Eleanor E Sahn, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Southern Medical Association

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, D. Stanton Whittaker Jr, MD, to the development and writing of this article.

References
  1. Weinstein LS, Liu J, Sakamoto A, Xie T, Chen M. Minireview: GNAS: normal and abnormal functions. Endocrinology. Dec 2004;145(12):5459-64. [Medline].

  2. Chanson P, Salenave S, Orcel P. McCune-Albright syndrome in adulthood. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:453-62. [Medline].

  3. Robey PG, Kuznetsov S, Riminucci M, Bianco P. The role of stem cells in fibrous dysplasia of bone and the Mccune-Albright syndrome. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:386-94. [Medline].

  4. Diaz A, Danon M, Crawford J. McCune-Albright syndrome and disorders due to activating mutations of GNAS1. J Pediatr Endocrinol Metab. Aug 2007;20(8):853-80. [Medline].

  5. Ozono K. GNAS1 gene abnormality in pseudohypoparathyroidism I a. Clin Calcium. Aug 2007;17(8):1214-9. [Medline].

  6. Wasniewska M, Matarazzo P, Weber G, Russo G, Zampolli M, Salzano G, et al. Clinical presentation of McCune-Albright syndrome in males. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:619-22. [Medline].

  7. Osada H, Sakamoto R, Seki K, Sekiya S. Accelerated bone turnover in pregnant women with McCune-Albright syndrome. Gynecol Obstet Invest. 2005;60(2):102-7. [Medline].

  8. Matarazzo P, Lala R, Andreo M, Einaudi S, Altare F, Viora E, et al. McCune-Albright syndrome: persistence of autonomous ovarian hyperfunction during adolescence and early adult age. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:607-17. [Medline].

  9. Volkl TM, Dorr HG. McCune-Albright syndrome: clinical picture and natural history in children and adolescents. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:551-9. [Medline].

  10. Kapoor S, Gogia S, Paul R, Banerjee S. Albright's hereditary osteodystrophy. Indian J Pediatr. Feb 2006;73(2):153-6. [Medline].

  11. Sargin H, Gozu H, Bircan R, Sargin M, Avsar M, Ekinci G, et al. A case of McCune-Albright syndrome associated with Gs alpha mutation in the bone tissue. Endocr J. Feb 2006;53(1):35-44. [Medline].

  12. Leet AI, Wientroub S, Kushner H, Brillante B, Kelly MH, Robey PG, et al. The correlation of specific orthopaedic features of polyostotic fibrous dysplasia with functional outcome scores in children. J Bone Joint Surg Am. Apr 2006;88(4):818-23. [Medline].

  13. Hamadani M, Chaudhary AL. McCune-Albright syndrome. Med J Aust. Dec 4-18 2006;185(11-12):597. [Medline].

  14. Ozcan-Kara P, Mahmoudian B, Erbas B, Erbas T. McCune-Albright syndrome associated with acromegaly and bipolar affective disorder. Eur J Intern Med. Dec 2007;18(8):600-2. [Medline].

  15. Narayan RL, Maldjian PD. Restrictive lung disease and cor pulmonale secondary to polyostotic fibrous dysplasia. Int J Cardiol. Aug 22 2007;[Medline].

  16. Bhat MH, Bhadada S, Dutta P, Bhansali A, Mittal BR. Hyperthyroidism with fibrous dysplasia: an unusual presentation of McCune-Albright syndrome. Exp Clin Endocrinol Diabetes. May 2007;115(5):331-3. [Medline].

  17. Román R, López P, Johnson MC, Boric MA, Gallo M, Ponce C, et al. Sudden infant death syndrome and activating gnas1 gene mutations. Fetal Pediatr Pathol. Jul-Aug 2007;26(4):199-205. [Medline].

  18. Bajpai A, Greenway A, Zacharin M. Platelet dysfunction and increased bleeding tendency in McCune-Albright syndrome. J Pediatr. 2008;153:287-9. [Medline].

  19. Xavier SP, Ribeiro MC, Sicchieri LG, Brentegani LG, Lacerda SA. Clinical, microscopic and imaging findings associated to McCune-Albright syndrome: report of two cases. Braz Dent J. 2008;19:165-70. [Medline].

  20. Ohata Y, Yamamoto T, Mori I, Kikuchi T, Michigami T, Imanishi Y, et al. Severe arterial hypertension: a possible complication of McCune-Albright syndrome. Eur J Pediatr. 2009;168:871-6. [Medline].

  21. Arrigo T, Pirazzoli P, De Sanctis L, Leone O, Wasniewska M, Messina MF, et al. McCune-Albright syndrome in a boy may present with a monolateral macroorchidism as an early and isolated clinical manifestation. Horm Res. 2006;65(3):114-9. [Medline].

  22. Medow JE, Agrawal BM, Resnick D. Polyostotic fibrous dysplasia of the cervical spine: case report and review of the literature. Spine J. Nov-Dec 2007;7(6):712-5. [Medline].

  23. Lietman SA, Ding C, Levine MA. A highly sensitive polymerase chain reaction method detects activating mutations of the GNAS gene in peripheral blood cells in McCune-Albright syndrome or isolated fibrous dysplasia. J Bone Joint Surg Am. Nov 2005;87(11):2489-94. [Medline].

  24. Christoforidis A, Maniadaki I, Stanhope R. McCune-Albright syndrome: growth hormone and prolactin hypersecretion. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:623-5. [Medline].

  25. de Sanctis L, Delmastro L, Russo MC, Matarazzo P, Lala R, de Sanctis C. Genetics of McCune-Albright syndrome. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:577-82. [Medline].

  26. Esmaili J, Chavoshi M, Noorani MH, Eftekhari M, Assadi M. Late diagnosed polyostotic fibrous dysplasia. Bone scan, radiography and magnetic resonance imaging findings. Hell J Nucl Med. Jan-Apr 2010;13(1):65-6. [Medline].

  27. Defilippi C, Chiappetta D, Marzari D, Mussa A, Lala R. Image diagnosis in McCune-Albright syndrome. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:561-70. [Medline].

  28. Riminucci M, Robey PG, Bianco P. The pathology of fibrous dysplasia and the McCune-Albright syndrome. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:401-11. [Medline].

  29. Lala R, Matarazzo P, Andreo M, Marzari D, Bellone J, Corrias A, et al. Bisphosphonate treatment of bone fibrous dysplasia in McCune-Albright syndrome. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:583-93. [Medline].

  30. Mansoori LS, Catel CP, Rothman MS. Bisphosphonate Treatment in Polyostotic Fibrous Dysplasia of the Cranium: Case Report and Literature Review. Endocr Pract. Jun 21 2010;1-14. [Medline].

  31. Chan B, Zacharin M. Maternal and infant outcome after pamidronate treatment of polyostotic fibrous dysplasia and osteogenesis imperfecta before conception: a report of four cases. J Clin Endocrinol Metab. Jun 2006;91(6):2017-20. [Medline].

  32. Dunkel L. Use of aromatase inhibitors to increase final height. Mol Cell Endocrinol. Jul 25 2006;254-255:207-16. [Medline].

  33. Feuillan P, Calis K, Hill S, Shawker T, Robey PG, Collins MT. Letrozole treatment of precocious puberty in girls with the McCune-Albright syndrome; a pilot study. J Clin Endocrinol Metab. Apr 3 2007;[Medline].

  34. Messina MF, Arrigo T, Wasniewska M, Lombardo F, Crisafulli G, Salzano G, et al. Combined treatment with ketoconazole and cyproterone acetate in a boy with McCune-Albright syndrome and peripheral precocious puberty. J Endocrinol Invest. 2008;31:839-40. [Medline].

  35. Gesmundo R, Guanà R, Valfrè L, De Sanctis L, Matarazzo P, Marzari D, et al. Laparoscopic management of ovarian cysts in peripheral precocious puberty of McCune-Albright syndrome. J Pediatr Endocrinol Metab. May 2006;19 Suppl 2:571-5. [Medline].

  36. Verma RR, Paul A. Fibrous dysplasia of the fourth metacarpal: en-bloc resection and free metatarsal transfer. Orthopedics. Apr 2006;29(4):371-2. [Medline].

  37. Feuillan P, Calis K, Hill S, Shawker T, Robey PG, Collins MT. Letrozole treatment of precocious puberty in girls with the McCune-Albright syndrome: a pilot study. J Clin Endocrinol Metab. Jun 2007;92(6):2100-6. [Medline].

  38. DiMeglio LA. Bisphosphonate therapy for fibrous dysplasia. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:440-5. [Medline].

  39. Jayaraman M, Karikumar K, Verma A, Modi KD. Alendronate therapy in polyostotic fibrous dysplasia presenting with pathologic fracture. Am J Orthop (Belle Mead NJ). Mar 2011;3:E48-51. [Medline].

  40. Rastogi A, Bhadada SK, Bhansali A. Recurrent Femur Neck Fracture and Response to Bisphosphonates in Polyostotic Fibrous Dysplasia. Indian J Pediatr. Jul 2011;[Medline].

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Large café au lait patches around the shoulder in a child with McCune-Albright syndrome.
Lucency characteristic of polyostotic fibrous dysplasia in a patient with McCune-Albright syndrome.
 
 
 
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