Identification of the enamelin (g.8344delG) mutation in a new kindred and presentation of a standardized ENAM nomenclature

The amelogenesis imperfectas (Al) area geneticatly heterogeneous group of diseases that result in defective development of tooth enamel. Although X-linked, autosomal. dominant and autosomal. recessive forms of Al have been clinically characterized, only two genes (AMELX and ENAM) have been associated with Al. To date, three enamelin (ENAM) mutations have been identified. These mutations cause phenotypically diverse forms of autosomal. dominant Al. Detailed phenotype-genotype correlations have not been performed for autosomal. dominant Al due to ENAM mutations. We identified a previously unreported kindred segregating for the ENAM mutation, g.8344delG. Light and electron microscopy analyses of unerupted permanent teeth show the enamel is markedly reduced in thickness, Lacks a prismatic structure and has a laminated appearance. Taken together these histological features support the enamelin protein as being critical for the development of a normal. enamel. thickness and that it Likely has a role in regulating c-axis crystallite growth. Because there is growing molecular and phenotypic diversity in the enamelin defects, it is critical to have a nomenclature and numbering system for characterizing these conditions. We present a standardized nomenclature for ENAM mutations that will allow consistent reporting and communication. (C) 2003 Elsevier Science Ltd. All rights reserved.

Ultrastructure of developing tooth plates in the Australian lungfish, Neoceratodus forsteri (Osteichthyes : Dipnoi)

While the lungfish dentition is partially understood as far as morphology and light microscopic structure is concerned, the ultrastructure is not. Each tooth plate is associated with a dental lamina that develops from the inner layer of endodermal cells that form the oral epithelium. Dentines, bone and cartilage of the jaws differentiate from mesenchyme cells aggregating beneath the oral endothelium. Enamel, in the developing and in the mature form, has similarities to that of other early vertebrates, but unusual characters appear as development proceeds. Ameloblasts are capable of secreting enamel, and, with mononuclear osteoclasts, of remodelling the bone below the tooth plate. The forms of dentine, all based largely on an extracellular matrix of collagen and mineralised with biological apatite, differ from each other and from the underlying bone in the ultrastructure of associated cells and in the mineralised extracellular matrices produced. Cell processes emerging from the odontoblasts and from the osteoblasts vary in length, degree of branching and of anastomoses between the processes, although all of the cell types have large amounts of rough endoplasmic reticulum. Mineralisation of the extracellular matrices varies among the enamel, dentines and bone in the tooth plate. In addition, the development of the hard tissues of the tooth plates indicates that many of the similarities in fine structure of the dentition in lungfish, to tissues in other fish and amphibia, apparent early in development, disappear as the dentition matures. (C) 2003 Elsevier Ltd. All rights reserved.

Growth hormone receptor and insulin-like growth factor-I immunoreactivity in osteoclast-like cells during tooth eruption in the toothless (Osteopetrotic) rat following treatment with colony-stimulating factor-1

In the toothless (tl/tl) osteopetrotic rat, teeth form but fail to erupt. Treatment of tl/tl rats with colony-stimulating factor-1 (CSF-1) activates bone resorption by osteoclasts, permits tooth eruption, and up-regulates the immunoreactivity of bone marrow mononuclear cells to growth hormone receptor (GHr) and insulin-like growth factor (IGF)-I. This study examined the distribution of tartrate-resistant acid phosphatase (TRAP) and immunoreactivity for GHr and IGF-I in osteoclast-like cells located on the alveolar bone margin, adjacent to the lower first molar crown, in 14-day-old normal and tl/tl rats, following treatment with CSF-1. Osteoclast-like cells demonstrated a positive reaction for TRAP, GHr, and IGF-I in all groups. However, in tl/tl tissue, osteoclast-like cells were generally negative for GHr. There was no significant difference in the total number of TRAP, GHr, and IGF-I-positive osteoclast-like cells on the adjacent bone margin in normal, normal treated with CSF-1, and tl/tl rats. CSF-1 treatment of the tl/tl rat significantly increased the total number of osteoclast-like cells, which were positive for TRAP (p < 0.001), GHr (p < 0.05) and IGF-I (P < 0.01).

Modeling, control and simulation of full-power converter wind turbines equipped with permanent magnet synchronous generator

In this paper, two wind turbines equipped with a permanent magnet synchronous generator (PMSG) and respectively with a two-level or a multilevel converter are simulated in order to access the malfunction transient performance. Three different drive train mass models, respectively, one, two and three mass models, are considered in order to model the bending flexibility of the blades. Moreover, a fractional-order control strategy is studied comparatively to a classical integer-order control strategy. Computer simulations are carried out, and conclusions about the total harmonic distortion (THD) of the electric current injected into the electric grid are in favor of the fractional-order control strategy.

A Pitch Control Malfunction Analysis for Wind Turbines with Permanent Magnet Synchronous Generator and Full-power Converters: Proportional Integral Versus Fractional-order Controllers

A transient analysis for two full-power converter wind turbines equipped with a permanent magnet synchronous generator is studied in this article, taking into consideration, as a new contribution to earlier studies, a pitch control malfunction. The two full-power converters considered are, respectively, a two-level and a multi-level converter. Moreover, a novel control strategy based on fractional-order controllers for wind turbines is studied. Simulation results are presented; conclusions are in favor of the novel control strategy, improving the quality of the energy injected into the electric grid.

In permanent transit: discourses and maps of the intercultural experience

Instituto Politécnico do Porto. Instituto Superior de Contabilidade e Administração do Porto

Are severe occlusal problems more frequent in permanent than deciduous dentition?

OBJECTIVE: To determine the prevalence and severity of occlusal problems in populations at the ages of deciduous and permanent dentition and to carry out a meta-analysis to estimate the weighted odds ratio for occlusal problems comparing both groups. METHODS: Data of a probabilistic sample (n=985) of schoolchildren aged 5 and 12 from an epidemiological study in the municipality of São Paulo, Brazil, were analyzed using univariate logistic regression (MLR). Results of cross-sectional study data published in the last 70 years were examined in the meta-analysis. RESULTS: The prevalence of occlusal problems increased from 49.0% (95% CI =47.4%-50.6%) in the deciduous dentition to 71.3% (95% CI =70.3%-72.3%) in the permanent dentition (p<0.001). Dentition was the only variable significantly associated to the severity of malocclusion (OR=1.87; 95% CI =1.43-2.45; p<0.001). The variables sex, type of school and ethnic group were not significant. The meta-analysis showed that a weighted OR of 1.95 (1.91; 1.98) when compared the second dentition period with deciduous and mixed dentition. CONCLUSIONS: In planning oral health services, some activities are indicated to reduce the proportion of moderate/severe malocclusion to levels that are socially more acceptable and economically sustainable.

Working hours, work-life conflict and health in precarious and "permanent" employment

OBJECTIVE: The expansion of precarious employment in OECD countries has been widely associated with negative health and safety effects. Although many shiftworkers are precariously employed, shiftwork research has concentrated on full-time workers in continuing employment. This paper examines the impact of precarious employment on working hours, work-life conflict and health by comparing casual employees to full-time, "permanent" employees working in the same occupations and workplaces. METHODS: Thirty-nine convergent interviews were conducted in two five-star hotels. The participants included 26 full-time and 13 casual (temporary) employees. They ranged in age from 19 to 61 years and included 17 females and 22 males. Working hours ranged from zero to 73 hours per week. RESULTS: Marked differences emerged between the reports of casual and full-time employees about working hours, work-life conflict and health. Casuals were more likely to work highly irregular hours over which they had little control. Their daily and weekly working hours ranged from very long to very short according to organisational requirements. Long working hours, combined with low predictability and control, produced greater disruption to family and social lives and poorer work-life balance for casuals. Uncoordinated hours across multiple jobs exacerbated these problems in some cases. Health-related issues reported to arise from work-life conflict included sleep disturbance, fatigue and disrupted exercise and dietary regimes. CONCLUSIONS:This study identified significant disadvantages of casual employment. In the same hotels, and doing largely the same jobs, casual employees had less desirable and predictable work schedules, greater work-life conflict and more associated health complaints than "permanent" workers.

Permanent vascular access in patients with end-stage renal disease, Brazil

OBJECTIVE: To assess factors associated with the establishment of permanent vascular access for patients with end-stage renal disease. METHODS: Cross-sectional study conducted in a nationally representative sample of Brazilian end-stage renal disease patients in dialysis and transplant centers during 2007. The sample comprised only patients who received hemodialysis as a primary therapy modality and reported the type of vascular access for their primary hemodialysis treatment (N=2,276). Data were from the TRS Project - "Economic and Epidemiologic Evaluation of Modalities of Renal Replacement Therapy in Brazil". Multiple logistic regression analysis was used to assess factors associated with the establishment of permanent vascular access in these patients. RESULTS: About 30% of the patients studied had an arteriovenous vascular access. The following factors were associated with a lower likelihood of having an arteriovenous vascular access as a primary type of access: time of hemodialysis start since the diagnosis of chronic renal failure < 1 year; shorter dialysis therapy; having no private health insurance; living in the central-western, northeastern and southeastern regions of Brazil; and living in the northern region plus having no private health insurance. In the final model there was found a positive association between the outcome and pre-dialysis care and no were association with socioeconomic and comorbidity variables. CONCLUSIONS: The study results showed that the focus should on pre-dialysis care to increase the establishment of an arteriovenous vascular access before starting hemodialysis in Brazil.

Association between tooth loss and obesity in Brazilian adults: a population-based study

OBJECTIVE: To examine the association between tooth loss and general and central obesity among adults. METHODS: Population-based cross-sectional study with 1,720 adults aged 20 to 59 years from Florianópolis, Southern Brazil. Home interviews were performed and anthropometric measures were taken. Information on sociodemographic data, self-reported diabetes, self-reported number of teeth, central obesity (waist circumference [WC] > 88 cm in women and > 102 cm in men) and general obesity (body mass index [BMI] ≥ 30 kg/m²) was collected. We used multivariable Poisson regression models to assess the association between general and central obesity and tooth loss after controlling for confounders. We also performed simple and multiple linear regressions by using BMI and WC as continuous variables. Interaction between age and tooth loss was also assessed. RESULTS: The mean BMI was 25.9 kg/m² (95%CI 25.6;26.2) in men and 25.4 kg/m2 (95%CI 25.0;25.7) in women. The mean WC was 79.3 cm (95%CI 78.4;80.1) in men and 88.4 cm (95%CI 87.6;89.2) in women. A positive association was found between the presence of less than 10 teeth in at least one arch and increased mean BMI and WC after adjusting for education level, self-reported diabetes, gender and monthly per capita income. However, this association was lost when the variable age was included in the model. The prevalence of general obesity was 50% higher in those with less than 10 teeth in at least one arch when compared with those with 10 or more teeth in both arches after adjusting for education level, self-reported diabetes and monthly per capita family income. However, the statistical significance was lost after controlling for age. CONCLUSIONS: Obesity was associated with number of teeth, though it depended on the participants' age groups.