Therapeutic approach to pediatric oral disorders.

The promotion of oral health in pediatrics stands as an institutional requirement in countries such as Italy where children's dental disorders still register a high incidence despite high levels of general health. Guidelines indicate the need to target a large age group and stress the relevance of parental education, whom pediatricians and dentists should address to. In this respect, teething is paradigmatic, due to the interactions between inflammation of the gingival mucosa, the possible associations with systemic symptoms as well as the psychological parental component.

Oculo-auriculo-vertebral spectrum with myopathy and velopharyngeal insufficiency. A case report with a non-branchiomeric muscle biopsy.

In the present paper we report on a case of oculo-auriculo-vertebral spectrum presenting fluorescence in situ hybridization and comparative genomic hybridization tests negative, hypotonia of some branchiomeric muscles (with velo-pharyngeal insufficiency, dysphagia and nasal voice) and non-branchiomeric muscles (with strabismus and limb hypotrophy). On the basis of the left quadriceps muscle biopsy, showing anisometry and prevalence of type 1 fibers, and on literature data, we underline the relevance of TBX1 gene (regulator of neural crest cells and activator of myogenic factors in branchiomeric muscles development) and of PAX3 gene (present in neural crest, inducing migration of these cells and reported in non-branchiomeric muscles). We conclude that the case of OAVS presented a generalized myopathy and we hypothesize that a cluster of genes strictly neural crest cells related, including TBX1 and PAX3, may be responsible of the branchiomeric and non-branchiomeric myopathy; alternatively, a regulatory mechanism abnormally common to OAVS and velo-cardio-facial syndrome could be present.

A modified intraoral resin mouthguard to prevent self-mutilations in lesch-nyhan patients.

Lesch-Nyhan syndrome, described in 1964 by Lesch and Nyhan, is a X-linked recessive disorder, occurring in 1 : 100000 to 1 : 380000 live births. LNS is characterized by a decrease in activity of hypoxanthine guanine phosphoribosyl transferase, an enzyme involved in purine metabolism, resulting in overproduction of uric acid. Hyperuricemia and neurological features including choreoathetoid spasticity, self-mutilation, and mental retardation clinically characterize this syndrome.

Cytogenetic analysis of gingival epithelial cells, as related to smoking habits and occurrence of periodontal disease.

Periodontal disease, progressing from gingivitis to periodontitis, affects the majority of the world population. Its pathogenesis is related to a complex interaction between environmental, microbial, genetic and other host factors, tobacco smoking being the most important environmental risk factor. Conflicting results are reported in the literature regarding the effects of smoking habits on cytogenetic damage in exfoliated oral cells.