Tuberous sclerosis complex with oral manifestations: A case report and literature review

Introduction: Tuberous sclerosis complex (TSC) is a neurocutaneous syndrome produced by a number of genetic mutations. The disease is characterized by the development of benign tumors affecting different body systems. The most common oral manifestations of TSC are fibromas, gingival hyperplasia and enamel hypoplasia. Clinical Case: A 35-year-old woman diagnosed with TSC presented with a reactive fibroma of considerable size and rapid growth in the region of the right lower third molar. Discussion: In the present case the association of TSC with dental malpositioning gave rise to a rapidly evolving reactive fibroma of considerable diameter. Few similar cases can be found in the literature. Patients with TSC present mutations of the TSC1 and TSC2 genes, which intervene in cell cycle regulation and are important for avoiding neoplastic processes. No studies have been found associating TSC with an increased risk of oral cancer, though it has been shown that the over-expression of TSC2 could exert an antitumor effect. Careful oral and dental hygiene, together with regular visits to the dentist, are needed for the prevention and early detection of any type of oral lesion. The renal, pulmonary and cardiac alterations often seen in TSC must be taken into account for the correct management of these patients.

Is Self-employment a Way to Escape from Skill Mismatches?

During the last two decades, skill mismatches have become one of the most important issues of policy concern in the EU (European Commission, 2008). Hence, the literature has stressed the necessity to reduce skill mismatches. We contribute to this literature by analyzing the impact of the transition from salaried employment to self-employment on self-reported skill mismatches. To do so, we resort to the European Community Household Panel (ECHP) covering the period 1994–2001. Using panel data, we track individuals over time and measure their self-reported skill mismatch before and after the transition. Our empirical findings indicate not only that the average self-employee is less likely to declare being skill-mismatched but also that those individuals who transit from salaried employment to self-employment reduce their probability of skill mismatches after the transition. Keywords: Self-employment, skill mismatches, salaried employment. JEL Classification: L26, J24, B23 __________________________

Further insights in trichothiodistrophy: a clinical, microscopic, and ultrastructural study of 20 cases and literature review

Background: Trichothiodistrophy (TTD) is a rare autosomal recessive condition that is characterized by a specific congenital hair shaft dysplasia caused by deficiency of sulfur associated with a wide spectrum of multisystem abnormalities. In this article, we study clinical, microscopic, and ultrastructural findings of 20 patients with TTD with the aim to add further insights regarding to this rare condition. Additionally, analyses of our results are compared with those extracted from the literature in order to enhance its comprehensibility. Materials and Methods: Twenty cases of TTD were included: 7 from Mexico and 14 from Spain. Clinical, microscopic, scanning electron microscopy (SEM) studies and X-ray microanalysis (XrMa) were carried out in all of them. Genetic studies were performed in all seven Mexican cases. Patients with xeroderma pigmentosum and xeroderma pigmentosum/TTD-complex were excluded. Results: Cuticular changes and longitudinal crests of the hair shaft were demonstrated. These crests were irregular, disorganized, following the hair longest axis. Hair shaft sulfur deficiency was disposed discontinuously and intermittently rather than uniformly. This severe decrease of sulfur contents was located close to the trichoschisis areas. Only five patients did not show related disturbances. Micro-dolichocephaly was observed in five cases and represented the most frequent facial dysmorphism found. It is also remarkable that all patients with urologic malformations also combined diverse neurologic disorders. Moreover, three Mexican sisters demonstrated the coexistence of scarce pubic vellus hair, developmental delay, onychodystrophy, and maxillar/mandibullar hypoplasia. Conclusions: TTD phenotype has greatly varied from very subtle forms to severe alterations such as neurologic abnormalities, blindness, lamellar ichthyosis and gonadal malformations. Herein, a multisystem study should be performed mandatorily in patients diagnosed with TTD.

Further insights in trichothiodistrophy: a clinical, microscopic, and ultrastructural study of 20 cases and literature review

Background: Trichothiodistrophy (TTD) is a rare autosomal recessive condition that is characterized by a specific congenital hair shaft dysplasia caused by deficiency of sulfur associated with a wide spectrum of multisystem abnormalities. In this article, we study clinical, microscopic, and ultrastructural findings of 20 patients with TTD with the aim to add further insights regarding to this rare condition. Additionally, analyses of our results are compared with those extracted from the literature in order to enhance its comprehensibility. Materials and Methods: Twenty cases of TTD were included: 7 from Mexico and 14 from Spain. Clinical, microscopic, scanning electron microscopy (SEM) studies and X-ray microanalysis (XrMa) were carried out in all of them. Genetic studies were performed in all seven Mexican cases. Patients with xeroderma pigmentosum and xeroderma pigmentosum/TTD-complex were excluded. Results: Cuticular changes and longitudinal crests of the hair shaft were demonstrated. These crests were irregular, disorganized, following the hair longest axis. Hair shaft sulfur deficiency was disposed discontinuously and intermittently rather than uniformly. This severe decrease of sulfur contents was located close to the trichoschisis areas. Only five patients did not show related disturbances. Micro-dolichocephaly was observed in five cases and represented the most frequent facial dysmorphism found. It is also remarkable that all patients with urologic malformations also combined diverse neurologic disorders. Moreover, three Mexican sisters demonstrated the coexistence of scarce pubic vellus hair, developmental delay, onychodystrophy, and maxillar/mandibullar hypoplasia. Conclusions: TTD phenotype has greatly varied from very subtle forms to severe alterations such as neurologic abnormalities, blindness, lamellar ichthyosis and gonadal malformations. Herein, a multisystem study should be performed mandatorily in patients diagnosed with TTD.

Is the influence of quality of life on urban growth non-stationary in space? A case study of Barcelona

There are several determinants that influence household location decisions. More concretely, recent economic literature assigns an increasingly important role to the variables governing quality of life. Nevertheless, the spatial stationarity of the parameters is implicitly assumed in most studies. Here we analyse the role of quality of life in urban economics and test for the spatial stationarity of the relationship between city growth and quality of life.

Does visual impairment in a child or an adolescent affect their parents' quality of life? : A literature review

The purpose of this research was to determine whether the existing research shows that having a child with a visual impairment affects their parents' quality of life and, if it is thecase, in what way it does.

Is the wage curve formal or informal? Evidence for Colombia

The objective of this paper is to analyse the existente or not of a wage curve in Colombia, paying special attention to the differences between formal and informal workers, an issue that has been systematically ignored in the wage curve literature. The obtained results using microdata from the Colombian Continuous Household Survey (CHS) between 2002 and 2006 show the existence of a wage curve with a negative slope for the Colombian economy. Using information on metropolitan areas, the estimates of the elasticity of individual wages to local unemployment rates was -0.07, a value that is very close to those obtained for other countries. However, the disaggregation of statistical information for formal and informal workers has shown significant differences among both groups of workers. In particular, for the less protected groups of the labour market, informal workers (both men and women), a high negatively sloped wage curve was found. This result is consistent with the conclusions from efficiency wage theoretical models and should be taken into account when analysing the functioning of regional labour markets in developing countries.

Taxation of unhealthy consumption of food and drinks: An updated literature review

By means of a literature review this paper aims at shedding more light on the potentialities of unhealthy food/drink taxation in changing eating patterns and life styles and hence combating the obesity epidemic. One remarkable point that emerges when assessing the set of selected papers is the existence of a wide diversity of objectives, methodologies, settings and datasets, policies implemented and results achieved by all these studies, which undoubtedly adds complexity to any attempt to draw a general conclusion on fast food taxation. Most of the examined studies predict a rather modest fiscal impact on unhealthy food and drinks consumption and/or nutrition intake and consequently a poor result on weight loss and obesity, by the interplay of several factors among them the effects of cross-price elasticities.

Taxation of unhealthy consumption of food and drinks: An updated literature review

By means of a literature review this paper aims at shedding more light on the potentialities of unhealthy food/drink taxation in changing eating patterns and life styles and hence combating the obesity epidemic. One remarkable point that emerges when assessing the set of selected papers is the existence of a wide diversity of objectives, methodologies, settings and datasets, policies implemented and results achieved by all these studies, which undoubtedly adds complexity to any attempt to draw a general conclusion on fast food taxation. Most of the examined studies predict a rather modest fiscal impact on unhealthy food and drinks consumption and/or nutrition intake and consequently a poor result on weight loss and obesity, by the interplay of several factors among them the effects of cross-price elasticities.

Eliminating Software Failures - A Literature Survey

Software faults are expensive and cause serious damage, particularly if discovered late or not at all. Some software faults tend to be hidden. One goal of the thesis is to figure out the status quo in the field of software fault elimination since there are no recent surveys of the whole area. Basis for a structural framework is proposed for this unstructured field, paying attention to compatibility and how to find studies. Bug elimination means are surveyed, including bug knowhow, defect prevention and prediction, analysis, testing, and fault tolerance. The most common research issues for each area are identified and discussed, along with issues that do not get enough attention. Recommendations are presented for software developers, researchers, and teachers. Only the main lines of research are figured out. The main emphasis is on technical aspects. The survey was done by performing searches in IEEE, ACM, Elsevier, and Inspect databases. In addition, a systematic search was done for a few well-known related journals from recent time intervals. Some other journals, some conference proceedings and a few books, reports, and Internet articles have been investigated, too. The following problems were found and solutions for them discussed. Quality assurance is testing only is a common misunderstanding, and many checks are done and some methods applied only in the late testing phase. Many types of static review are almost forgotten even though they reveal faults that are hard to be detected by other means. Other forgotten areas are knowledge of bugs, knowing continuously repeated bugs, and lightweight means to increase reliability. Compatibility between studies is not always good, which also makes documents harder to understand. Some means, methods, and problems are considered method- or domain-specific when they are not. The field lacks cross-field research.

Built environment and elderly population health: a comprehensive literature review

Global population aging over recent years has been linked to poorer health outcomes and higher healthcare expenditure. Policies focusing on healthy aging are currently being developed but a complete understanding of health determinants is needed to guide these efforts. The built environment and other external factors have been added to the International Classification of Functioning as important determinants of health and disability. Although the relationship between the built environment and health has been widely examined in working age adults, research focusing on elderly people is relatively recent. In this review, we provide a comprehensive synthesis of the evidence on the built environment and health in the elderly.

IS SELF-DECEPTION PRETENSE?

I assess Tamar Gendler's (2007) account of self-deception according to which its characteristic state is not belief, but imaginative pretense. After giving an overview of the literature and presenting the conceptual puzzles engendered by the notion of self-deception, I introduce Gendler's account, which emerges as a rival to practically all extant accounts of self-deception. I object to it by first arguing that her argument for abandoning belief as the characteristic state of self-deception conflates the state of belief and the process of belief-formation when interpreting David Velleman's (2000) thesis that belief is an essentially truth-directed attitude. I then call attention to the fact that Velleman's argument for the identity of motivational role between belief and imagining, on which Gendler's argument for self-deception as pretense depends, conflates two senses of 'motivational role'-a stronger but implausible sense and a weaker but explanatorily irrelevant sense. Finally, I introduce Neil Van Leeuwen's (2009) argument to the effect that belief is the practical ground of all non-belief cognitive attitudes in circum-stances wherein the latter prompt action. I apply this framework to Gendler's account to ultimately show that imaginative pretense fails to explain the existence of voluntary actions which result from self-deception.

Incidence of uterine post abortion infection at Hospital de Clínicas de Porto Alegre. Is prophylactic antibiotic necessary?

OBJECTIVE: To identify the incidence of pelvic infection after miscarriage undergoing uterine evacuation in a tertiary hospital in southern Brazil and to compare with the international literature.METHODS: we reviewed electronic medical records of the Hospital de Clinicas de Porto Alegre of all patients who underwent uterine evacuation for miscarriage between August 2008 and January 2012 were reviewed. We included all patients submitted to uterine curettage due to abortion and who had outpatient visits for review after the procedure. We calculated emographic and laboratory data of the study population, number needed for treatment (NNT) and number needed to harm (NNH).RESULTS: of the 857 revised electronic medical records, 377 patients were subjected to uterine evacuation for miscarriage; 55 cases were lost to follow-up, leaving 322 cases that were classified as not infected abortion on admission. The majority of the population was white (79%); HIV prevalence and positive VDRL was 0.3% and 2%, respectively. By following these 322 cases for a minimum of seven days, it was found that the incidence of post-procedure infection was 1.8% (95% CI 0.8 to 4). The NNT and NNH calculated for 42 months were 63 and 39, respectively.CONCLUSION: The incidence of post-abortion infection between August 2008 to January 2012 was 1.8% (0.8 to 4).