Cases reported "Jaw Diseases"

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1/4. 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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keywords = cherubism
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2/4. cherubism: diagnosis, treatment, and comparison with central giant cell granulomas and giant cell tumors.

    An extensive case of cherubism in a 17-year-old girl with no apparent familial history is presented. The rationale for treatment is presented and is compared with previous reports that have advocated nontreatment. The clinical and histologic similarities of cherubism to central giant cell granuloma and the giant cell tumor of bone are also discussed.
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keywords = cherubism
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3/4. cherubism - new hypotheses on pathogenesis and therapeutic consequences.

    AIMS: The hereditary occurrence of cherubism indicates a probable genetic aetiology: a correlation with a mutation in the gene SH3BP2 has been demonstrated. A convincing concept of formal pathogenesis is not yet available. The study was aimed at advancing the understanding of the pathogenesis of cherubism by presenting a case study including genetic findings and an evaluation of the literature. RESULTS AND CONCLUSION: Because of its association with the development of the second and third molars, cherubism could be defined as a genetically determined alteration of tooth development. In this context, disturbed PTHrP - PTHrP receptor interaction induced by the mutation in SH3BP2 is discussed. The temporal and spatial determination of the clinical symptoms is explained by an interaction of SH3BP2-dependent signal transduction pathways with jaw morphogenesis (e.g. Hox-gene Msx-1). Because of the disease-induced lack of determination of the cap phase of the second and third molar, a spatial compartmentation, which is necessary for normal dental development, does not take place. This leads to dysregulation of mesenchymal bone building tissue areas, and to the development of giant cell granulomas with high osteoclastic activity. Because of the genetic determination of cherubism and the associated dedifferentiation of the diseased tissue, a surgical removal should be exclusively restricted to specific indications. Therefore an attitude of wait and see is preferred.
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keywords = cherubism
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4/4. Multiple central giant cell lesions with a Noonan-like phenotype.

    A small number of patients with the phenotypic features of noonan syndrome have also developed giant cell lesions of the jaws similar to those seen in cherubism. This case report describes an individual with the features of the recently described Noonan-like/multiple giant cell lesion syndrome. Previously reported cases and issues relative to diagnosis and management are considered.
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keywords = cherubism
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