McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia

McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia

Fibrous dysplasia (FD) is sometimes accompanied by extraskeletal manifestations that can include any combination of café-au-lait macules, hyperfunctioning endocrinopathies, such as gonadotropin-independent precocious puberty, hyperthyroidism, growth hormone excess, FGF23-mediated renal phosphate wasting, and/or Cushing syndrome, as well as other less common features. The combination of any of t...

McCune Albright Syndrome ( MAS ) - polyostotic fibrous dysplasia

Sinonasal disease in polyostotic fibrous dysplasia and McCune-Albright Syndrome.

OBJECTIVES/HYPOTHESIS To characterize the spectrum, symptoms, progression, and effects of endocrine dysfunction on sinonasal disease in polyostotic fibrous dysplasia (PFD) and McCune-Albright Syndrome (MAS). STUDY DESIGN Retrospective review. METHODS A prospectively followed cohort of subjects with PFD/MAS underwent a comprehensive evaluation that included otolaryngologic and endocrine eval...

Intramedullary rodding and bisphosphonate treatment of polyostotic fibrous dysplasia associated with the McCune-Albright syndrome.

The authors report the results of the management of five consecutive children with McCune-Albright syndrome. These children were treated with a combination of drug treatment (bisphosphonates) and surgical treatment with elongating intramedullary rods (Sheffield) for management of femoral and tibial lesions. This treatment was successful in all patients, as judged by improvement in their quality...

Fracture incidence in polyostotic fibrous dysplasia and the McCune-Albright syndrome.

UNLABELLED In patients with polyostotic fibrous dysplasia of bone, the peak incidence of fractures is during the first decade of life, followed by a decrease thereafter. Phosphaturia is associated with an earlier incidence and increased frequency of fractures. INTRODUCTION Fibrous dysplasia (FD) is a disorder involving either one (monostotic) or several bones (polyostotic FD [PFD] and sometim...