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image/svg+xml Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao Closed Access logo, derived from PLoS Open Access logo. This version with transparent background. http://commons.wikimedia.org/wiki/File:Closed_Access_logo_transparent.svg Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao Reumatología Clínica...arrow_drop_down
image/svg+xml Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao Closed Access logo, derived from PLoS Open Access logo. This version with transparent background. http://commons.wikimedia.org/wiki/File:Closed_Access_logo_transparent.svg Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao
Reumatología Clínica (English Edition)
Article . 2014 . Peer-reviewed
License: Elsevier TDM
Data sources: Crossref
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Polyostotic fibrous displasia: A case report

Authors: Carlos F. Meneses; Antonio Egües; J. Belzunegui; Miren Uriarte;

Polyostotic fibrous displasia: A case report

Abstract

A 37-year-old woman with a fibrous polyostotic dysplasia (FPD) of the left femur, tibia and foot was diagnosed at 11 years of age. At the onset she presented mechanical pain of the left hip and later a stress fracture of the femur for which she was treated with surgery, pamidronate and zolendronate. Pathology confirmed the diagnosis. Phosphocalcic metabolism was normal. X-rays (Fig. 1) showeda left femurwitha «sheperd’s staff »deformity, a thin bone cortex and expansive radiolucent lesions. The left tibia (Fig. 2) was curved and had a thin cortex. Feet (Fig. 3) were thickened at the first right metatarsal and phalange with radiolucent and sclerotic areas. A computed tomographyof the tibia (Fig. 4) observed a ground glass matrix, with heterogeneous intramarrow images. FPD is a rare anomaly of skeletal development. A mutation in the GNAS1 gene has been detected,1 producing alterations in osteoplastic maturation and abnormal fibrous tissue deposit.2 There are two variants: monostotic and polyostotic.3 Lesions are localized on the epiphysis, metaphysis or diaphysis. Themonostotic variant is more prevalent, diagnosed during the patient’s youthand less symptomatic. It affects the ribs, femur, tibia, jawbone and humerus.4 The polyostotic form is observed in 30% of cases. It is usually diagnosed during the patients’ infancy. It affects the cranium, face, pelvis, spine and shoulder. It is associated to the McCune-Albright syndrome in 2% of cases (FPD, skin pigmentation and early puberty).2 It leads to dysmetria, gait abnormalities, mechanical pain and stress fractures.5 FPD diagnosis is radiological, rarely requiring a bone biopsy. The prognosis depends on the extension and degree of bone affection, age at

Related Organizations
Keywords

Adult, Humans, Female, Fibrous Dysplasia, Polyostotic, Tomography, X-Ray Computed

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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
3
Average
Average
Average
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