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Polyostotic Fibrous Dysplasia (McCune Albright Syndrome)

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FIBROUS DYSPLASIA OF BONE affecting several or many bones. When associated with melanotic pigmentation of the skin and endocrine disorders, it is known as Albright's syndrome. (From Dorland, 28th ed)

Also Known As:

McCune Albright Syndrome; Albright Syndrome; Syndrome, Albright's; Syndrome, McCune-Albright; Albright's Syndrome; Fibrous Dysplasia, Polyostotic; Albrights Syndrome; Dysplasia, Polyostotic Fibrous; Dysplasias, Polyostotic Fibrous; Fibrous Dysplasias, Polyostotic; Polyostotic Fibrous Dysplasias; McCune-Albright Syndrome

Networked: 320 relevant articles (13 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Musculoskeletal Diseases: 179
- Bone Diseases: 3566
  - Developmental Bone Diseases: 198
    - Osteochondrodysplasias: 551
      - Fibrous Dysplasia of Bone: 53
        Polyostotic Fibrous Dysplasia: 320
        Albright's hereditary osteodystrophy: 212
        Chromosome 2q37 deletion syndrome: 11

Related Diseases

1.	Precocious Puberty
2.	Gynecomastia
3.	Neoplasms (Cancer)
4.	Cushing Syndrome
5.	Pain (Aches)

Experts

1.	Collins, Michael T: 8 articles (01/2014 - 11/2002)
2.	Eugster, Erica A: 7 articles (01/2016 - 07/2003)
3.	Stratakis, Constantine A: 5 articles (02/2016 - 07/2004)
4.	Sultan, Charles: 5 articles (12/2015 - 12/2006)
5.	Cherman, Natasha: 4 articles (06/2008 - 11/2002)
6.	Weinstein, Lee S: 4 articles (12/2006 - 06/2002)
7.	Lala, R: 4 articles (08/2002 - 02/2000)
8.	Matarazzo, P: 4 articles (08/2002 - 02/2000)
9.	Pals-Rylaarsdam, Robin: 3 articles (06/2014 - 07/2006)
10.	Jacobson, Jill D: 3 articles (01/2013 - 01/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Polyostotic Fibrous Dysplasia:

1.	Testolactone (Teslac)FDA Link 03/01/1998 - "Girls with McCune-Albright syndrome, given testolactone, respond similarly. " 06/01/1987 - "Short-term effects of testolactone compared to other treatment modalities on longitudinal growth and ovarian activity in a girl with McCune-Albright syndrome." 01/01/2004 - "The first-generation agent, testolactone, has been demonstrated to be tolerable and effective in the treatment of familial male precocious puberty, while mixed results with testolactone have been achieved in girls with McCune-Albright syndrome. " 10/30/1986 - "We conclude that testolactone is an effective treatment of precocious puberty in the McCune-Albright syndrome." 09/01/1993 - "Long-term testolactone therapy for precocious puberty in girls with the McCune-Albright syndrome."
2.	Gonadotropin-Releasing Hormone (GnRH)FDA Link 10/18/1984 - "Cyclical ovarian function resistant to treatment with an analogue of luteinizing hormone releasing hormone in McCune-Albright syndrome." 10/01/1984 - "Variable response to a long-acting agonist of luteinizing hormone-releasing hormone in girls with McCune-Albright syndrome." 10/01/1984 - "Six girls with McCune-Albright syndrome were treated for at least 2 months with the long-acting LHRH agonist D-Trp6-Pro9-NEt-LHRH, which previously was found to be an effective treatment for true precocious puberty. " 01/01/1998 - "GnRH analogues have been used with variable success for the treatment of precocious puberty in children with McCune-Albright syndrome (MAS). " 01/01/1998 - "Secondary central precocious puberty in a girl with McCune-Albright syndrome responds to treatment with GnRH analogue."
3.	Aromatase (CYP19)IBA 06/01/2007 - "Our objective was to assess the effectiveness of the potent, third-generation aromatase inhibitor letrozole in decreasing pubertal progression in girls with MAS and to assess the response of indices of bone turnover associated with the patients' polyostotic fibrous dysplasia. " 12/01/1991 - "One of these girls appeared to benefit from the aromatase inhibitor testolactone, which is effective in suppressing precocious puberty in girls with the McCune-Albright syndrome." 07/01/2008 - "The aromatase inhibitor anastrozole is ineffective in the treatment of precocious puberty in girls with McCune-Albright syndrome." 10/30/1986 - "Treatment of precocious puberty in the McCune-Albright syndrome with the aromatase inhibitor testolactone." 06/01/1985 - "We used an aromatase inhibitor, testolactone, to treat precocious puberty in a girl with McCune-Albright syndrome. "
4.	pamidronate (APD)FDA LinkGeneric 06/01/2015 - "Treatment of Polyostotic Fibrous Dysplasia of the Thoracic Spine with Intravenous Pamidronate: Result from 9 Months Follow Up." 07/01/2010 - "Pamidronate for fibrous dysplasia due to McCune Albright Syndrome." 04/01/2009 - "Long-term pamidronate treatment of polyostotic fibrous dysplasia of bone: A case series in young adults." 01/01/2006 - "Pamidronate treatment of polyostotic fibrous dysplasia: failure to prevent expansion of dysplastic lesions during childhood." 10/01/2003 - "Effect of pamidronate treatment in children with polyostotic fibrous dysplasia of bone."
5.	Estrogens (Estrogen)FDA Link 10/01/2015 - "Precocious pseudopuberty in girls occurs as a result of peripheral estrogen production, most commonly through an autonomous ovarian cyst or in patients with McCune Albright syndrome. " 09/01/2004 - "These medications have been used primarily in women with breast carcinoma and also in children with autonomous estrogen production, such as patients with McCune-Albright Syndrome. " 06/01/1988 - "It is likely that in this case of McCune-Albright syndrome precocious puberty was a result of ovarian estrogen secretion, while pubertal activation of the hypothalamus-pituitary axis was absent. " 06/01/1984 - "It appears that precocious puberty in McCune-Albright syndrome may result from ovarian estrogen secretion in the absence of normal pubertal activation of the hypothalamic-pituitary-ovarian axis." 12/01/1982 - "In girls, precocious puberty with very high circulating estrogen levels was observed as part of a McCune-Albright syndrome. "
6.	Tamoxifen FDA LinkGeneric 01/01/2006 - "Tamoxifen improved final height prediction in a girl with McCune-Albright syndrome: patient report and literature review." 09/01/2015 - "A 5-year and 3-month-old girl, diagnosed with McCune-Albright syndrome, came in with abnormal hair growth approximately 2 months after the initiation of tamoxifen. " 01/01/2015 - "Tamoxifen Improves Final Height Prediction in Girls with McCune-Albright Syndrome: A Long Follow-Up." 09/01/2015 - "Tamoxifen-associated hirsutism: an unusual side effect in a 5-year-old girl with McCune-Albright syndrome." 08/01/2008 - "Tamoxifen (Tam) has been used experimentally to treat boys with gynecomastia and girls with McCune-Albright syndrome. "
7.	fulvestrant (Faslodex)FDA Link 01/01/2012 - "Fulvestrant treatment of precocious puberty in girls with McCune-Albright syndrome." 01/01/2012 - "The objective of this study was to evaluate the safety and efficacy of fulvestrant (FaslodexTM), a pure estrogen receptor antagonist, in girls with progressive precocious puberty (PP) associated with McCune-Albright Syndrome (MAS). "
8.	Familial TestotoxicosisIBA 04/01/2012 - "Unfortunately, medical advances in the diagnosis of these disorders have not been reflected in the medical treatment of McCune-Albright syndrome and testotoxicosis. " 04/01/2012 - "In recent years, molecular advances have made remarkable progress in understanding the pathophysiology of some of these disorders, most notably in McCune-Albright syndrome and testotoxicosis. " 09/01/1987 - "[Pubertas praecox in McCune-Albright syndrome--case report and review of the literature]." 08/01/1954 - "[Albright's disease (syndrome of polyostotic fibrous dysplasia, pubertas praecox and multiple pigment spots)]." 11/01/2009 - "It may result from several different disorders including testotoxicosis, McCune-Albright syndrome, congenital adrenal hyperplasia, adrenal and gonadal tumours. "
9.	Alendronate (Alendronate Sodium)FDA LinkGeneric 01/01/2004 - "Oral alendronate is suggested to be a useful option in the treatment of polyostotic fibrous dysplasia." 01/01/2004 - "Oral alendronate treatment for polyostotic fibrous dysplasia: a case report." 09/01/2003 - "We report a case of polyostotic fibrous dysplasia in a three-year-old child who showed significant improvement in bone mineral density after treatment with oral alendronate." 03/01/2011 - "Alendronate therapy in polyostotic fibrous dysplasia presenting with pathologic fracture." 01/01/2004 - "We describe a patient with polyostotic fibrous dysplasia, who had complained of persistent pain for more than 20 years, that was treated with oral alendronate alone. "
10.	Aromatase InhibitorsIBA 02/01/2012 - "Although different in presentation, all three atypical cases of suspected McCune-Albright syndrome with gonadotropin-independent precocious puberty were successfully treated with aromatase inhibitors. " 09/01/1999 - "Treatment of progressive precocious puberty in patients with McCune-Albright syndrome (MAS) has traditionally been with aromatase inhibitors, such as testolactone. " 03/01/2012 - "Current data suggest that aromatase inhibitors are probably effective in the treatment of patients with aromatase excess syndrome or testotoxicosis, partially effective in Peutz-Jeghers and McCune-Albright syndrome, but probably ineffective in gynecomastia. " 03/01/2012 - "The availability of the selective aromatase inhibitors letrozole and anastrozole--currently approved as adjuvant therapy for breast cancer--have stimulated off-label use of aromatase inhibitors in pediatrics for the following conditions: hyperestrogenism, such as aromatase excess syndrome, Peutz-Jeghers syndrome, McCune-Albright syndrome and functional follicular ovarian cysts; hyperandrogenism, for example, testotoxicosis (also known as familial male-limited precocious puberty) and congenital adrenal hyperplasia; pubertal gynecomastia; and short stature and/or pubertal delay in boys. "

Therapies and Procedures

1.	Ovariectomy (Oophorectomy) 01/01/2008 - "Restoration of ovulation after unilateral ovariectomy in a woman with McCune-Albright syndrome: a case report." 09/01/2007 - "Oophorectomy in McCune-Albright syndrome: a case of mistaken identity." 12/01/2015 - "Postpubertal Persistent Hyperestrogenemia in McCune-Albright Syndrome: Unilateral Oophorectomy Improved Fertility but Detected an Unexpected Borderline Epithelial Ovarian Tumor." 08/01/2010 - "McCune-Albright syndrome (MAS): early and extensive bone fibrous dysplasia involvement and "mistaken identity" oophorectomy." 09/01/2007 - "The objective of the study was to determine the incidence of oophorectomy in girls presenting with precocious puberty and vaginal bleeding who are subsequently diagnosed with McCune-Albright syndrome (MAS). "
2.	Adrenalectomy 01/01/2003 - "Patients from the former group had an open or laparoscopic adrenalectomy, or a bilateral adrenalectomy in case of the rare bilateral diseases, as McCune-Albright syndrome. " 06/01/2008 - "Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome." 06/01/2008 - "Adrenalectomy may be avoidable even in severe cases of Cushing syndrome associated with McCune-Albright syndrome." 07/01/1968 - "McCune-Albright's syndrome following adrenalectomy for Cushing's syndrome in infancy." 06/01/1999 - "Two cases of McCune-Albright syndrome (MAS) are reported who presented in the neonatal period with profound failure to thrive, cardio-respiratory distress, precocious puberty and Cushing's syndrome for which both underwent bilateral adrenalectomy. "
3.	Aftercare (After-Treatment) 12/01/2011 - "The clinical symptoms of children with McCune-Albright syndrome or CAH were alliaviated partly after treatment, and 7 cases were transformed to central precocious puberty. " 09/01/2003 - "We report a case of polyostotic fibrous dysplasia in a three-year-old child who showed significant improvement in bone mineral density after treatment with oral alendronate." 09/01/2010 - "To report the case of a patient with polyostotic fibrous dysplasia of the cranium who showed dramatic improvement after treatment with intravenous zoledronic acid. " 02/01/1993 - "Cyproterone acetate was tried under the diagnosis of McCune-Albright syndrome, and her secondary sexual characteristics disappeared after treatment."
4.	Osteotomy 07/01/2015 - "Children from 4 to 7 years of age with polyostotic fibrous dysplasia (PFD) may need internal fixation of the femur for either fracture or osteotomy. " 07/01/2015 - "Internal fixation after fracture or osteotomy of the femur in young children with polyostotic fibrous dysplasia." 06/01/1987 - "Multiple osteotomies with Zickel nail fixation for polyostotic fibrous dysplasia involving the proximal part of the femur." 09/01/2005 - "[Corrective osteotomy of the humerus using perivascular axillary anesthesia according to Weber in a patient suffering from McCune-Albright syndrome]." 01/01/2015 - "Double-level osteotomy and one-stage reconstruction with long intramedullary femoral nail to correct a severe proximal and diaphyseal femur deformity in a patient with polyostotic fibrous dysplasia: case report and literatures review."
5.	Decompression 10/01/1999 - "A case of atypical McCune-Albright syndrome requiring optic nerve decompression." 09/01/2014 - "Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome." 04/01/2008 - "Relief of severe retro-orbital pain and vision improvement after optic-nerve decompression in polyostotic fibrous dysplasia: case report and review of the literature." 05/01/2012 - "The syndromes involving the thyroid and parathyroid glands that have ocular manifestations and are rare include Mc Cune Albright syndrome wherein optic nerve decompression may occur due to fibrous dysplasia, primary hyperparathyroidism that may present as red eye due to scleritis and Ascher syndrome wherein ptosis occurs. "