Noninvasive and multidisciplinary approach to the functional and esthetic rehabilitation of amelogenesis imperfecta: a pediatric case report

Case Report. An 8-year-old girl with amelogenesis imperfecta (AI) reported unsatisfactory aesthetics, difficulty in mastication, and dental hypersensitivity. The intraoral examination observed mixed dentition, malocclusion in anteroposterior relationships, anterior open bite, and dental asymmetry. A hypoplastic form of AI was diagnosed in the permanent dentition. A multidisciplinary planning was performed and divided into preventive, orthopedic, and rehabilitation stages. Initially, preventive treatment was implemented, with fluoride varnish applications, in order to protect the fragile enamel and reduce the dental sensitivity. In the second stage, the patient received an interceptive orthopedic treatment to improve cross-relationship of the arches during six months. Finally, the rehabilitation treatment was executed to establish the vertical dimension. In the posterior teeth, indirect composite resin crowns were performed with minimally invasive dental preparation. Direct composite resin restorations were used to improve the appearance of anterior teeth. Follow-Up. The follow-up was carried out after 3, 6, 12, and 18 months. After 18 months of follow-up, The restoration of integrity, oral hygiene, and patient satisfaction were observed . Conclusion. Successful reduction of the dental hypersensitivity and improvement of the aesthetic and functional aspects as well as quality of life were observed.

Variable expressivity of osteogenesis imperfecta in a Brazilian family due to p.G1079S mutation in the COL1A1 gene

Osteogenesis imperfecta (OI) is a Mendelian disease with genetic heterogeneity characterized by bone fragility, recurrent fractures, blue sclerae, and short stature, caused mostly by mutations in COL1A1 or COL1A2 genes, which encode the pro-alpha 1(I) and pro-alpha 2(I) chains of type I collagen, respectively. A Brazilian family that showed variable expression of autosomal dominant OI was identified and characterized. Scanning for mutations was carried out using SSCP and DNA sequence analysis. The missense mutation c.3235G>A was identified within exon 45 of the COL1A1 gene in a 16-year-old girl diagnosed as having OI type I; it resulted in substitution of a glycine residue (G) by a serine (S) at codon 1079 (p.G1079S). The proband's mother had the disease signs, but without bone fractures, as did five of nine uncles and aunts of the patient. All of them carried the mutation, which was excluded in four healthy brothers of the patient's mother. This is the first description in a Brazilian family with OI showing variable expression; only one among seven carriers for the c.3235G>A mutation developed bone fractures, the most striking clinical feature of this disease. This finding has a significant implication for prenatal diagnosis in OI disease.

Do patients with osteogenesis imperfecta need individualized nutritional support?

Objective: Information regarding nutrition and body composition in patients diagnosed with osteogenesis imperfecta (OI) is scarce. In the present study, nutritional status, bone mineral density, and biochemical parameters of subjects with Of were evaluated. Methods: Patients with type I OI (n = 13) and type III OI (n = 13) and healthy controls (n = 8) were selected. Nutritional status and bone mineral density were assessed by a 3-d food diary and dual-energy X-ray absorptiometry at the lumbar spine, respectively. Body mass index, serum albumin, calcium, creatinine, cross-linked C-telopeptide, parathyroid hormone, and 25-hydroxivitamin D-3 were also evaluated. Results: Patients with OI had lower bone mineral density (P < 0.05 versus controls). Patients with type III OI had the highest body mass index (P < 0.05 versus patients with type I OI and controls) and the lowest lean body mass (P < 0.05 versus patients with type I OI and controls). In patients with OI, the number of fractures was positively correlated with body mass index (r = 0.581, P = 0.002) and the percentage of body fat (r = 0.451, P = 0.027) and negatively correlated to lean body mass (r = -0.523, P = 0.009). Even when taking dietary supplements, 58% and 12% of subjects with OI did not achieve the calcium and vitamin D recommendations, respectively. Conclusions: Body composition is a risk factor for bone fractures in subjects with OI. Individualized nutritional support is recommended not only to improve body composition but also to potentiate pharmacologic and physical therapies. (C) 2012 Elsevier Inc. All rights reserved.

Problemas de equilibrio en modelos de competencia espacial en redes

Premio Extraordinario, Área de Sociales y Jurídicas

El uso de textos de la Literatura de tradición oral en el desarrollo de la competencia comunicativa

Claves comunicativas perceptibles en una versión romancístiva del sur grancanario. Análisis de las interacciones entre los protagonistas de un romance que integra a su vez tres motivos romancísticos tradicionales:"La infantina encantada", "El caballero burlado" y "Don Bueso y la hermana cautiva"

Competencia entre orcas y pescadores de la piedra por el atún rojo en el Estrecho de Gibraltar

Máster en Gestión Sostenible de Recursos Pesqueros

Ensayo sobre la reforma del sector eléctrico : tecnología y competencia

Programa de doctorado: Economía

Desarrollo de la competencia discursiva e intercultural de sinohablantes en el ámbito universitario europeo.

[ES] Esta investigación intenta dar respuesta a una necesidad social e institucional de cómo desarrollar la competencia escrita de estudiantes sinohablantes universitarios. El objetivo final será la producción de textos argumentativos propios de un nivel universitari B1, correspondiente al Marco Común Europeo de Referencia de las lenguas: aprendizaje, enseñanza y evaluación (MCER) y el Plan Curricular del Instituto Cervantes (PCIC). La muestra la conforman seis estudiantes chinas pertenecientes a la Universidad Normal de Changchun, China, adscritas al Centro Confucio de la Universidad de Las Palmas de Gran Canaria, durante el período académico 2010-2011. Este trabajo propone una serie de factores para el proceso enseñanza-aprendizaje de alumnos chinos, aplicables al EEES y busca una mejora en la composición escrita de textos argumentativos, teniendo en cuenta factores como la motivación y la interculturalidad.