Cases reported "Cherubism"

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1/38. Gingival fibromatosis combined with cherubism and psychomotor retardation: a rare syndrome.

    Gingival fibromatosis is frequently an isolated condition, but rarely associated with some uncommon syndromes. This paper describes an 11-year-old patient with pronounced gingival enlargement, cherubic facial appearance, and psychomotor retardation and discusses the major aspects of the case. The most striking finding orally was the presence of grossly hyperplastic gingiva, which completely covered all teeth except the occlusal surfaces of some teeth. The swelling in the lower part of the face and the appearance of sclera beneath the iris suggest cherubism. The diagnosis was confirmed by the detection of giant cell regenerative granuloma and perivascular eosinophilic particles and osteoclasts after biopsy of the mandible. In this case, surgery was the only effective way to treat the patient. A full-mouth gingivectomy procedure was performed under general anesthesia in 2 stages. The case was followed for 12 months and no recurrence was seen. An appropriate oral hygiene regimen was established.
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ranking = 1
keywords = mandible, lower
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2/38. cherubism: clinicopathologic features.

    A case of cherubism in 6-year-old boy is reported. He presented with bilateral symmetrical enlargement of the jaw in addition to medially dislocated premature teeth, narrow V-shaped palatal vault, and mild upward turning of the eyes. Radiographs showed multiloculated osteolysis in both the mandible and maxilla. histology revealed a non-neoplastic fibrous lesion, rich in multinucleated giant cells, consistent with giant-cell reparative granuloma. Since the original description of cherubism, various histologic interpretations have been proposed, particularly that of fibrous dysplasia. However, it should be emphasized that cherubism is a disease histologically indistinguishable from giant-cell reparative granuloma.
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ranking = 1.6764424598128
keywords = mandible, jaw
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3/38. Craniosynostosis in cherubism.

    cherubism is a rare autosomal dominant fibro-osseous disorder that affects almost exclusively maxilla and mandible. Extracranial skeletal involvement is rare. We report on three affected males in three generations. The youngest affected relative was examined at age 4 months. He also had craniosynostosis. His affected father and grandfather had cherubism and clubbing of the fingers. cherubism was mapped to region 4p16. Because of the associated cranio-synostosis, we excluded the FGFR3 gene as a candidate gene for cherubism.
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ranking = 0.99883904573319
keywords = mandible
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4/38. Orbital involvement in cherubism.

    cherubism is a rare, inherited condition characterized by fibro-osseous lesions of the maxilla and mandible. It has recently been localized to chromosome 4p16.3. The fullness of the lower half of the face and retraction of the lower lids gives the characteristic 'eyes raised to heaven' cherubic appearance. A case report of a 7-year-old girl with extensive orbital involvement of cherubism is presented. The patient underwent multidisciplinary surgery for the bony lesions, which extended from the maxillary antrum into adjacent structures, including extensive extraperiosteal orbital involvement. cherubism may have orbital manifestations including lower lid retraction, proptosis, diplopia, globe displacement and visual loss due to optic atrophy. Ophthalmologists should be aware of the syndrome, its ophthalmic features and overall management.
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ranking = 1.0023219085336
keywords = mandible, lower
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5/38. cherubism--clinical picture and treatment.

    cherubism is a rare, painless, disfigurating disease primarily affecting bones of the jaws. OBJECTIVE: To report on five patients with cherubism. The symptoms of the disease, methods of management and possible mode of inheritance are discussed and literature is reviewed. patients: The study involves five cherubs, members of one family. The diagnoses were based on history, physical examination, laboratory tests, X-ray parameters, and clinical follow-up. One member of the family had surgical intervention. The remaining cases were left for observation. RESULTS: Good aesthetic and long lasting effect was reached in the operated patient. CONCLUSIONS: Treatment is unnecessary unless functional or emotional disturbances develop. An autosomal recessive pattern of inheritance is suggested for these cases, although autosomal dominant transmission has been previously established.
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ranking = 0.67760341407962
keywords = jaw
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6/38. Orbital involvement in cherubism.

    PURPOSE: To demonstrate the clinical, radiologic, and histopathologic features of a patient with orbital involvement in cherubism that prompted surgical treatment. DESIGN: Single interventional case report. INTERVENTION: Findings of the ophthalmic evaluation, computed tomography (CT) scans, intraoperative examination, and light microscopy of the specimens were analyzed. MAIN OUTCOME MEASURES: Globe displacement, orbital bony lesions detected on CT scans, histopathology, and postoperative results were assessed. RESULTS: A 27-year-old female was seen with a slowly progressive superonasal globe displacement and a temporal orbital mass bilaterally of 6 years' duration. She had a history of cherubism, but her cheeks and jaws had a normal appearance instead of the bilateral fullness of the lower half of the face typical of the disease. CT scans demonstrated multicystic bony lesions arising from the orbital floors bilaterally. The masses were excised using an anterior transcutaneous transseptal orbitotomy. Histopathology demonstrated numerous giant cells in a fibrovascular stroma, confirming the clinical diagnosis of cherubism. Postoperative recovery was complete. CONCLUSIONS: Orbital involvement in cherubism may develop beyond puberty, after stabilization or regression of the lesions in the jaws. patients with cherubism should be routinely evaluated by an ophthalmologist.
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ranking = 1.3563677824261
keywords = jaw, lower
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7/38. cherubism and its charlatans.

    cherubism is a rare hereditary condition characterised by progressive cystic proliferation of the mandible and maxilla in childhood, followed by post-pubertal involution of the process and jaw remodelling in adulthood. Its name is derived from the cherubic appearance that results from the jaw hypertrophy. Here, we present the case of a young boy with cherubism, in the context of his pedigree, to illustrate the clinical characteristics and their variable expression. We also seek to distinguish cherubism from central giant cell granuloma and giant cell tumour of the jaws, with which it holds a false synonymity.
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ranking = 3.031649287972
keywords = mandible, jaw
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8/38. cherubism: clinical evidence and therapy.

    cherubism is a hereditary childhood disease of autosomal dominant inheritance that is more common in the male sex. Some cases have also revealed a mutation of the gene FGF-RIII (fibroblast growth factor receptor III). The clinical picture of cherubism is characterized by painless symmetrical swelling of the mandible and polycystic destruction of the mandibular bone structure. Complications include delayed dentition, dental root resorption, malalignment of teeth, and impacted teeth. The disease progression comes to a halt of its own accord at the end of puberty. Spontaneous recoveries have been observed. Histologically, multinuclear giant cells with osteoclastic activity, which correspond to a giant cell granuloma, are found in fibrous stroma. It is generally agreed that decisions about surgical intervention should be considered carefully. A case report describes the course of the disease and therapy of a female patient with a periorbital manifestation of cherubism. The latero-orbital enlargement of the intraorbital and latero-orbital region of the zygomatic bone resulted in displacement of the orbital contents with a motility disorder of the eyes. The motility disorder was eliminated by reduction of the diseased hard tissue as well as by carrying out a modeling orbitotomy and by reconstructing an anatomically correct localized and irregular bilateral osseous structure of the zygomatic bone in the intraorbital and latero-orbital region.
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ranking = 0.99883904573319
keywords = mandible
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9/38. cherubism in siblings: a case report.

    cherubism is a non-neoplastic bone disease characterized by clinically evident bilateral, painless enlargements of the jaws that are said to give the patient a cherubic appearance. cherubism may appear in solitary cases or in many members of the same family, often in multiple generations. On radiography, the lesions exhibit bilateral multilocular radiolucent areas. Histopathologic evaluation reveals proliferating fibrous connective tissue containing numerous multinucleated giant cells. Since the first description of this condition in 1933, almost 200 cases have been reported. We describe cherubism in 2 siblings and briefly review the literature on this subject.
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ranking = 0.67760341407962
keywords = jaw
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10/38. Novel mutation in the gene encoding c-Abl-binding protein SH3BP2 causes cherubism.

    cherubism is a rare autosomal dominant inherited condition caused by mutations in the c-Abl-binding protein SH3BP2. It is characterized by multiple cystic giant cell lesions of the jaw appearing in early childhood with stabilization and remission after puberty. In the present study, we used direct sequence analysis of the SH3BP2 gene of several individuals from a family with cherubism to search for additional SH3BP2 mutations resulting in cherubism. In affected relatives, we found a previously unreported G to A transition in exon 9 leading to a Gly to Arg substitution at amino acid position 420. G420R has been reported previously with a G to C transversion. To date there have been no disease causing mutations outside exon 9. Therefore, the amino acid sequence from positions 415 to 420 may represent a specific protein domain which, when disrupted, leads to the cherubism phenotype.
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ranking = 0.67760341407962
keywords = jaw
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