Polyostotic Fibrous Dysplasia (Subscribe)  

Abstract Fibrous dysplasia is a benign but slowly progressive disorder of bone in which normal cancellous bone is replaced by immature woven bone and fibrous tissue. Significant deformity and both acute and chronic visual impairment can result. A contemporary understanding of fibrous dysplasia, emphasizing the origins of visual impairment, indications for...

Author: Gabriel I Uwaifo, MBBS, Clinical and Research Attending, MedStar Clinical Research Center, Assistant Professor of Medicine and Endocrinology, The MedStar Research Institute and the Washington Hospital Center Coauthor(s): Nicholas J Sarlis, MBBS, MD, PhD, FACP, Medical Director, Department of Oncology-US Medical Affairs Department, Sanofi-Aventis Pharmaceuticals Background McCune-Albright syndrome (MAS) is defined as...

Alternative titles; symbols ALBRIGHT SYNDROME POLYOSTOTIC FIBROUS DYSPLASIA; PFD; POFD this phenotype is associated with mutations in the GNAS1 gene (139320). The McCune-Albright syndrome, usually caused by mosaicism for a mutation in the GNAS1 gene, is characterized by polyostotic fibrous dysplasia, pigment patches of the skin and endocrinologic abnormalities, including precocious puberty, thyrotoxicosis,...

Synonyms MAS Albright Syndrome Osteitis Fibrosa Disseminata Polyostotic, Fibrous Dysplasia PFD Precocious Puberty with Polyostotic Fibrosis and Abnormal Pigmentation POFD General Discussion McCune-Albright Syndrome (MAS) is a rare multisystem disorder characterized by (1) replacement of normal bone tissue with areas of abnormal fibrous growth (fibrous dysplasia); (2) patches of abnormal...

# polyostotic fibrous dysplasia
# precocious puberty (33% of females, rarely in males)
# cafe-au-lait spots (coast of Maine)

Osteitis Fibrosa Disseminata: "A disease of bone marked by thinning of the cortex and replacement of bone marrow by gritty fibrous tissue containing bony spicules, producing pain, disability, and gradually increasing deformity. Only one bone may be involved (FIBROUS DYSPLASIA, MONOSTOTIC) or several (FIBROUS DYSPLASIA, POLYOSTOTIC). (From Dorland, 28th ed)" 2 synonyms...

Definition of Polyostotic fibrous dysplasia Polyostotic fibrous dysplasia: A genetic disorder of bones, skin pigmentation and hormonal problems with premature sexual development. Also called McCune-Albright syndrome or the Albright syndrome. In the syndrome, there is bone disease with fractures and deformity of the legs, arms and skull; pigment patches of the...

McCune-Albright syndrome (polyostotic fibrous dysplasia), described in 1937 by Donovan James McCune and Fuller Albright, is a genetic disorder of bones, skin pigmentation and hormonal problems along with premature puberty. It is suspected when two of the three following features are present: (autonomous) endocrine hyperfunction such as precocious puberty Fibrous dysplasia...

Polyostotic Fibrous Dysplasia, The Alfred I. duPont Institute