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Revista Médica del Uruguay

versión impresa ISSN 0303-3295versión On-line ISSN 1688-0390

Resumen

RIENZI, Tomás et al. Polyostotic fibrous dysplasia - McCune-Albright syndrome: a case report. Rev. Méd. Urug. [online]. 2021, vol.37, n.1, e701.  Epub 01-Mar-2021. ISSN 0303-3295.  http://dx.doi.org/10.29193/rmu.37.1.12.

Introduction:

polyostotic fibrous dysplasia is a low prevalence disease, and for this reason, it often goes undetected. When associated to endocrinopathies and/or hyperpigmented skin lesions we speak about McCune Albright syndrome.

Clinical case:

eight-year old school girl who presented pathological fracture of the left femur, which was diagnosed as polyostotic fibrous dysplasia. As it was accompanied by peripheral precocious puberty it constituted an indicative clinical picture of the so-called McCune Albright. Control and treatment were multidisciplinary. The traumatology team performed osteosynthesis with plaques and nails to fix the bone lesion, and evolution consolidated in a regular time frame (3 months). Upon six months follow-up, the girl has no pain and presents no functional repercussion in daily life. From the endocrinological perspective, the girl received precocious puberty treatment with aromatase inhibitors with the purpose of improving her final size and avoid psychological and emotional implications.

The study presents the characteristics of this condition, as well as its vital and functional prognosis.

Palabras clave : Polyostotic fibrous dysplasia; McCune-Albright syndrome; Peripheral precocious puberty.

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