polyostotic fibrous dysplasia


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pol·y·os·tot·ic fi·brous dys·pla·si·a

the occurrence of lesions of fibrous dysplasia in multiple bones, commonly on one side of the body; may occur with areas of pigmentation and endocrine dysfunction (McCune-Albright syndrome).
Farlex Partner Medical Dictionary © Farlex 2012

McCune-Albright syndrome

An autosomal dominant condition (OMIM:174800) due to altered regulation of cAMP, endocrinopathy (e.g., hyperthyroidism) and hypophosphatemia.

Clinical findings
Precocious puberty, polyostotic (cystic fibrous dysplasia) spontaneous fractures at young age, café-au-lait spots on skin, ovarian cysts.
 
Lab
Cyclical 4–6-week fluctuations of plasma oestrogen; afflicted young girls have decreased gonadotropins, decreased response to LH-RH; increased testosterone, increased alkaline phosphatase.
 
Molecular pathology
Defects in GNAS, which encodes a G protein that modulates various membrane signalling cascasdes, cause McCune-Albright syndrome.

Management
Aromatase inhibitor testolactone.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

polyostotic fibrous dysplasia

Fibrous dysplasia affecting multiple bones, including, in some children, long bones as well as facial or cranial bones. See: McCune-Albright syndrome
See also: dysplasia
Medical Dictionary, © 2009 Farlex and Partners
References in periodicals archive ?
The distribution of bones in polyostotic fibrous dysplasia is often unilateral and monomelic.
(5) This syndrome is characterized by the triad of polyostotic fibrous dysplasia, ipsilateral skin pigmentation, and precocious puberty.
A special type of polyostotic fibrous dysplasia is McCune-Albright syndrome, which is associated with precocious sexual development and cafe au lait spots on the skin.
(4) Polyostotic fibrous dysplasia, along with endocrine abnormalities and cutaneous hyperpigmentation, is a component of Albright-McCune-Sternberg syndrome, a rare condition that primarily affects females.
It has been suggested that such lesions that are not associated with involvement of the extracranial skeleton represent a third type of polyostotic fibrous dysplasia. [3]
[1] There are two primary categories of the disease: monostotic fibrous dysplasia involves a single bone and represents 70% of all cases; polyostotic fibrous dysplasia involves multiple bones and makes up the remainder, [2] Also, approximately 3% of patients with fibrous dysplasia will experience McCune-Albright syndrome, in which the bony involvement is accompanied by skin lesions and endocrinopathy.