Osteofibrous Dysplasia (Kempson-Campanacci's Disease)

2019-10-01 07:55:04 | BioPortfolio


The aim of the present study is to obtain long term follow-up in patients with osteofibrous dysplasia, to assess natural history of the disease, late results of treatment and in particular the potential and risk of progression to adamantinoma.

Study Design


Osteofibrous Dysplasia


study of osteofibrous dysplasia features


Dept. of Pathology of IRCCS Istituto Ortopedico Rizzoli




Istituto Ortopedico Rizzoli

Results (where available)

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Published on BioPortfolio: 2019-10-01T07:55:04-0400

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Medical and Biotech [MESH] Definitions

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)

A condition resulting from congenital malformations involving the brain. The syndrome of septo-optic dysplasia combines hypoplasia or agenesis of the SEPTUM PELLUCIDUM and the OPTIC NERVE. The extent of the abnormalities can vary. Septo-optic dysplasia is often associated with abnormalities of the hypothalamic and other diencephalic structures, and HYPOPITUITARISM.

An autosomal dominant form of ectodermal dysplasia which is due to mutations in the gene for the EDAR RECEPTOR.

An X-linked form of ectodermal dysplasia which results from mutations of the gene encoding ECTODYSPLASIN.

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)

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