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.

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.

El plan de lengua y cohesión social en Catañunya: primeros datos de una investigación = Language and Social Cohesion Plan in Catalonia: Initial research data

Los programas de inmersión lingüística han constituido y constituyen dentro del Sistema Educativo catalánla principal forma para que el alumnado de lengua familiar no-catalana aprenda una nueva lengua, el catalán,sin que, en su proceso de aprendizaje, vea mermado ni el desarrollo de su propia lengua ni su rendimientoacadémico. El éxito de la inmersión lingüística en las décadas anteriores ha sido frecuentemente utilizado comouno de los argumentos orientativos para justificar la política lingüística que se sigue en la escolarización de lainfancia extranjera. Sin embargo, los resultados obtenidos por investigaciones recientes parece que no avalanempíricamente dicho argumento. Este artículo analiza dichos resultados y expone, a partir del Plan para laLengua y Cohesión Social puesto en marcha por el Departamento de Educación de la Generalitat de Cataluña,cuáles son los retos que se presentan a su Sistema Educativo dentro del nuevo marco que supone el aumento de ladiversidad cultural y lingüística en la actual sociedad catalana

Benjamin's conception of language and Adorno's aesthetic theory

According to the theory of language of the young Benjamin, the primary task of language isn't the communication of contents, but to express itself as a "spiritual essence" in which also men take part. That conception according to which language would be a medium to signification of something outside it leads to a necessary decrease of its original strength and is thus denominated by Benjamin bürgerlich. The names of human language are remainders of an archaic state, in which things weren't yet mute and had their own language. Benjamin suggests also that all the arts remind the original language of things, as they make objects "speak" in form of sounds, colors, shapes etc. That relationship between arts as reminders of the "language of things" and the possible reconciliation of mankind with itself and with nature has been developed by Theodor Adorno in several of his writings, specially in the Aesthetic Theory, where the artwork is ultimately conceived as a construct pervaded by "language" in the widest meaning - not in the "bourgeois" sense.

Conference Proceedings. Assessing Language and (Inter) cultural Competences in Higher Education. Evaluation des compétences langagières et (inter) culturelles dans l’enseignement supérieur

This epublication contains papers that were presented at the conference “Assessing Language and (Inter) cultural Competences in Higher Education” which took place at the University of Turku (Finland) on 30.8.1.9.2007. The online proceedings may be downloaded and used provided the source is acknowledged.

Ambivalent self-understanding? : change, language and boundaries in the Shetland Islands (1970–present)

Avhandlingen berör språkets roll på Shetlandsöarna från år 1970 till idag och shetländarnas självbild som shetländare och skottar. Öarna, som ligger i Storbritanniens nordligaste del, beskrivs ofta som kulturellt annorlunda och unika. Även om shetländarna uppfattar sig själva som annorlunda, har utomstående betraktare tidvis tonat ner, tidvis betonat öarnas kulturella särdrag. Shetlandsöarna utgör ett intressant undersökningsobjekt, eftersom shetländarnas uppfattningar om sig själva som en särskild grupp har genomgått en förändring under de fyra senaste årtiondena. Den ekonomiska högkonjunktur som var en konsekvens av oljefynden i Nordsjön och 1990-talets politiska förändringar på öarna och i Skottland har båda påverkat de sätt på vilka Shetland beskrivs och förstås. I den förändrade samhällssituationen tvingades shetländarna omvärdera sin relation till det skotska fastlandet. Man var också tvungen att hitta nya svar på frågan vilka shetländarna är och på vilka sätt man borde värna om den verkliga eller föreställda kulturella autonomin. Avhandlingens syfte var att undersöka och analysera de sätt på vilka de samhälleliga förändringarna har påverkat shetländarnas självförståelse, särskilt de uppfattningar som är kopplade till språket. Undersökningen visar att många av dagens uppfattningar om en kulturell särart kan spåras till slutet av 1800-talet och tiden efter andra världskriget. Som avhandlingen visar har språkhistorien spelat en viktig roll i den process i vilken shetländarna har särskilt sig som en separat grupp. Språkets betydelse kan förstås korrekt endast om man i stället för att betrakta shetländarna som en etnisk grupp betraktar Shetlandsöarna som en relativt sett autonom beslutför region. I detta fall fungerar dialekten som en symbol för regional samhörighet.

Laparoscopy in uterine cervical cancer. Current state and literature review

Cervical cancer remains the most frequent gynecological tumor in Brazil and other developing countries. Minimally invasive techniques, especially laparoscopy, have been increasingly employed in such tumors. This article aims to describe the main applications of laparoscopy in the treatment and staging of cervical cancer. In the early stages, it is possible to provide a fertility-preserving surgery in the form of radical trachelectomy and, in a study protocol, the function-preserving surgery, avoiding parametrectomy and the associated morbidity. A fully laparoscopic radical hysterectomy is fairly standard in the literature and has the tendency to become the standard of care in early cases, for patients who want to bear no more children. In advanced stages, minimally invasive surgery can offer ovarian transposition, with intent to prevent actinic castration, without upsetting the time for the start of radiotherapy and chemotherapy. Staging laparoscopic surgery, including pelvic and para-aortic lymphadenectomy, has been the subject of studies, since it has the potential to modify the extension of radiotherapy depending on the extent of lymph node spread.

The Arctic Ocean between Greenland and Nova Zemla and between the 60th and 82d parallel of latitude from Swedish, Danish, British, Russian and German surveys and explorations up to 1871

Kartta kuuluu A. E. Nordenskiöldin kokoelmaan

Memory mixed with desire : a preliminary study of philosophy and literature in the works of Friedrich Nietzsche and Milan Kundera

Memory Mixed with Desire: A preliminary study of Philosophy and Literature in the works of Friedrich Nietzsche and Milan Kundera Robert Spinelli Brock University, Department of Philosophy This thesis studies intertextuality in the works of Friedrich Nietzsche and Milan Kundera through the primary themes of memory and forgetting. The thesis starts with two introductory chapters that delineate memory according to Nietzsche and Kundera respectively. From here, I move into a discussion of Nietzsche's Ubermensch as an example of the type of forgetting that Nietzsche sees as a cure for the overabundance of memory that has led to Christian morality. Next, I explore the Kunderan concept of kitsch as the polar opposite of what Nietzsche has sought in his philosophy, finishing the chapter by tying the two thinkers together in a Kunderan critique of Nietzsche. The thesis ends with a chapter devoted to the Eternal Return beginning with an exegesis of Nietzsche's idea and ending with a similar exegesis of Kundera's treatment of this thought. What I suggest in this chapter is that the Eternal Return might itself be a form of kitsch even in its attempt to revalue existence.

Singing Nature, Dancing Buddha: Zen, Language, and the Groundlessness of Silence

This thesis presents Zen experience as aesthetic in nature. This is done through an analysis of language, a central concern for Zen Buddhism. The thesis develops two modes of language at work in Zen: representational and indexical. What these modes of language entail, the kind of relations that are developed through their use, are explored with recourse to a variety of Zen platforms: poetry, the koan, zazen, music, and suizen. In doing so, a primacy of listening is found in Zen - a listening without a listener. Given this primacy of listening, silence comes to the forefront of the investigation. An analysis of John Cage's 4'33" provides this thesis with justification of the groundlessness of silence, and the groundlessness of subjectivity. Listening allows for the abyssal subject to emerges, which in tum allows for reality to present itself outside of the constitutive function of language.

“You Know What I Mean?”- Language and Cultural Retention in Luso-Canadian Mothers in the Greater Toronto Area

The Portuguese community is one of the largest diasporic groups in the Greater Toronto Area and the choice of retention and transmission of language and culture to Luso-Canadians is crucial to the development and sustainability of the community. The overall objective of this study is to learn about the factors that influence Luso-Canadian mothers’ inclination to teach Portuguese language and cultural retention to their children. To explore this topic I employed a qualitative research design that included in-depth interviews conducted in 2012 with six Luso-Canadian mothers. Three central arguments emerged from the findings. First, Luso-Canadian mothers interviewed posses a pronounced desire for their children to succeed academically, and to provide opportunities that their children that they did not have. Second, five of the mothers attempt to achieve this mothering objective partly by disconnecting from their Portuguese roots, and by disassociating their children from the Portuguese language and culture. Third, the disconnection they experience and enact is influenced by the divisions evident in the Portuguese community in the GTA that divides regions and hierarchically ranks dialects, and groups. I conclude that the children in these households inevitably bear the prospects of maintaining a vibrant Portuguese community in the GTA and I propose that actions by the community in ranking dialects influence mothers’ decisions about transmitting language and culture to their children.

Face to face with the mexicans : the domestic life, educational, social, and bussines ways statesmanship and literature, legendary and general history of the mexicana people.

UANL

Polyglot reader, and guide for traslation: consisting of a series of english extracts, with their traslation into French, German, Spanish, and Italian : the severeal parts designed to serve as mu tual keys

UANL

An ethnolinguistic study of the Yanesha’ (Amuesha) language and speech community in Peru’s Andean Amazon, and the traditional role of Ponapnora, a female rite of passage

Thesis written in co-mentorship with Richard Chase Smith Ph.D, of El Instituto del Bien Comun (IBC) in Peru. The attached file is a pdf created in Word. The pdf file serves to preserve the accuracy of the many linguistic symbols found in the text.