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      Crecimiento mandibular bilateral: un caso de querubismo Translated title: Bilateral swelling of the jaws: a case of querubism

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          Abstract

          Resumen El querubismo es una displasia ósea benigna de la infancia caracterizada por un crecimiento mandibular bilateral progresivo. Presenta herencia autosómica dominante y es más frecuente en varones. Las primeras manifestaciones aparecen en torno a los dos años de edad, con un crecimiento óseo acelerado a los 8-9 años e interrupción espontánea tras la pubertad. El diagnóstico se basa en la clínica, radiología y anatomía patológica. El tratamiento es controvertido, incluyendo tratamiento médico o quirúrgico. Presentamos el caso de un varón de nueve años que consultó por crecimiento mandibular bilateral, requiriendo finalmente extirpación quirúrgica de las lesiones.

          Translated abstract

          Abstract Cherubism is a benign bone dysplasia characterized by bilateral progressive enlargement of the jaws. It is an autosomal dominant disease and boys are more affected than girls. The first signs of manifestation of the disease are generally observed at about two years of age, followed by accelerated growth from 8-9 years of age and spontaneous interruption after puberty. The diagnosis is based on clinical, radiographic and histopathologic findings. Treatment is a controversial issue and therapy strategies include surgical treatment and medical treatment. We describe the case of a nine-year-old boy with bilateral swelling of the jaws who required surgical treatment.

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          Cherubism: A Case Report with Surgical Intervention.

          Cherubism is a rare benign, autosomal-dominant hereditary fibro-osseous condition predominantly affecting the jaws. Symmetrical cyst-like expansions of the jaws cause the characteristic facial swellings. The disease is often associated with severe malposition of teeth. The gene for cherubism is src homology 3 binding protein 2 (SH3BP2) located on chromosome 4p16.3. The repeated experience of this self-limiting disorder in affected individuals published in the medical literature has resulted in a wait-and-see strategy regarding therapeutic options. Indeed, cessation and regression of even large bone expansions can be expected in early adulthood. Nevertheless, severe facial disfiguring and functional impairment can make surgical intervention necessary. This report details the surgical procedures carried out in a patient with progressive and disfiguring jaw expansions at the end of adolescence, the mutation of SH3BP2 gene, and the limited effect of surgically assisted orthodontic tooth movement in a patient with disease-associated impaired tooth development and tooth eruption.
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            Cherubism: a case report

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              Querubismo, un caso clínico

              El Querubismo es una alteración osteofibrosa benigna que los huesos de la cara originada por un funcionamiento anormal de osteoblastos y osteoclastos que ocasiona reemplazo de tejido óseo por matriz fibrosa y hueso inmaduro que como consecuencia desarrolla un crecimiento progresivo no doloroso de la mandíbula, dando un aspecto redondeado a la cara. Caso clínico: Se prresenta un caso de Querubismo en una paciente de 11 años de edad, con cuadro de 8 años de evolución de aumento progresivo del volumen facial, el cuadro clínico y radiológico de Querubismo fue confirmado por el análisis molecular que detecto una mutación de novo en el gen SH3BP2.
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                Author and article information

                Journal
                pap
                Pediatría Atención Primaria
                Rev Pediatr Aten Primaria
                Asociación Española de Pediatría de Atención Primaria (Madrid, Madrid, Spain )
                1139-7632
                September 2021
                : 23
                : 91
                : 301-303
                Affiliations
                [1] Madrid orgnameHospital Universitario La Paz orgdiv1Servicio de Urgencias de Pediatría España
                [2] Tenerife orgnameHospital Universitario Nuestra Señora de la Candelaria orgdiv1Servicio de Pediatría España
                Article
                S1139-76322021000300011 S1139-7632(21)02309100011
                283f724a-649e-4678-8c3d-01e798b627bf

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

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                Figures: 0, Tables: 0, Equations: 0, References: 10, Pages: 3
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                SciELO Spain

                Categories
                Notas Clínicas

                Cherubism,Giant cell tumor,Crecimiento mandibular,Displasia ósea,Querubismo,Tumor de células gigantes,Mandibular swelling,Bone dysplasia

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