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.

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.

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.

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

The Discipline of Economics and Economic Cycles

In This Article, It Is Argued That the Long International, Financial and Economic Cycle (50-60 Years) Is More Than a Statistical Aberration, and Is the Result of Institutional Political, Financial and Economic Conditions Which Are Recurrent. It Is Proposed As an Hypothesis That the Breakdown of International Monetary Systems Is At the Origin of Hte Financial and Economic Long Cycle. Such a Breakdown Starts a Process of Unexpected Inflation, of Balance of Payments Imbalances and of International Indebtedness in a Key-Currency. the Last Stage of This Process Is Characterized by Disinflation, a Speculative Stock Market Boom and a Period of Debt-Liquidation Which Negatively Affect the Real Side of the Economy. Without an International and Automatic Mechanism to Correct the Financial and Economic International Imbalances, the World Economy Runs the Risk of Getting More and More Unstable Until the Turning Point. International Monetary Cooperation Could Contribute in Alleviating the Negative Spillovers Accompanying Hte Adjustment of These Imbalances.

LMS software study and a practical application in language and literature didactics. 'Aplicación práctica en Didáctica de la lengua y la literatura del software LMS'.

Resumen en español

Towards an interface of language and literature. 'Hacia una interfaz de la Lengua y la Literatura'.

Se analiza la conveniencia de usar textos simplificados o en su formato original para propositos pedagógicos dentro de la enseñanza de la segunda lengua. El autor considera que ambas clases de textos (simplificados u originales) pueden formar el nivel intermedio de aprendizaje de lenguas extranjeras.

AQA GCSE English language and literature. Foundation.

Recurso aprobado por AQA, ha sido escrito para apoyar a los estudiantes en la preparación del General Certificate of Secondary Education (GCSE) inglés lengua y literatura inglesas. Todas las unidades son totalmente compatibles con recursos digitales. Estructurado en diecisiete unidades: las siete primeras ayudarán con los exámenes, las unidades restantes con evaluaciones controladas. Incluye secciones de gramática, ortografía y puntuación.

AQA GCSE English language and literature. Higuer.

Recurso aprobado por AQA, ha sido escrito para apoyar a los estudiantes en la preparación del General Certificate of Secondary Education (GCSE) inglés lengua y literatura inglesas. Todas las unidades son totalmente compatibles con recursos digitales. Estructurado en diecisiete unidades: las siete primeras ayudarán con los exámenes, las unidades restantes con evaluaciones controladas. Incluye secciones de gramática, ortografía y puntuación.

AQA GCSE English language and literature. Teacher's resource book. Higher and foundation.

Recurso para el profesor con directrices detalladas y consejos prácticos para los estudiantes que preparan el General Certificate of Secondary Education (GCSE) AQA de lengua y literatura inglesas. Estructurado en diecisiete unidades, las siete primeras preparan los exámenes y las restantes incluyen unidades con evaluaciones controladas.

La London School of Economics and Political Science.

Historia de la evolución de la famosa escuela de ciencias políticas y económicas, London School of Economics and Political Sciencie, centrándonos en las actividades puramente académicas desde su nacimiento, y en las labores llevadas a cabo por los sucesivos directores: W. A. Hewins, Halford J. Mackinder, W. Pember Reeves, Sir William Beveridge y A. M. Carr-Saunders.

Investigation and literature review of unilateral hearing loss

Literature and research were gathered and analyzed to determine the impact UHL has on a child’s education, speech and language development. The effects of early intervention and amplification were also analyzed.

Libraries and literature in Rome

A chapter of 6,000 words on books, literary culture, and public and private libraries in the ancient city of Rome

Integrating socio-economics and ecology: a taxonomy of quantitative methods and a review of their use in agro-ecology

Answering many of the critical questions in conservation, development and environmental management requires integrating the social and natural sciences. However, understanding the array of available quantitative methods and their associated terminology presents a major barrier to successful collaboration. We provide an overview of quantitative socio-economic methods that distils their complexity into a simple taxonomy. We outline how each has been used in conjunction with ecological models to address questions relating to the management of socio-ecological systems. We review the application of social and ecological quantitative concepts to agro-ecology and classify the approaches used to integrate the two disciplines. Our review included all published integrated models from 2003 to 2008 in 27 journals that publish agricultural modelling research. Although our focus is on agro-ecology, many of the results are broadly applicable to other fields involving an interaction between human activities and ecology. We found 36 papers that integrated social and ecological concepts in a quantitative model. Four different approaches to integration were used, depending on the scale at which human welfare was quantified. Most models viewed humans as pure profit maximizers, both when calculating welfare and predicting behaviour. Synthesis and applications. We reached two main conclusions based on our taxonomy and review. The first is that quantitative methods that extend predictions of behaviour and measurements of welfare beyond a simple market value basis are underutilized by integrated models. The second is that the accuracy of prediction for integrated models remains largely unquantified. Addressing both problems requires researchers to reach a common understanding of modelling goals and data requirements during the early stages of a project.