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.

An Overview Of British Automotive Industry: Implications From History and Recommendations for Future

This thesis is a literary research, which aims to uncover reasons for the downfall of automotive industry in the United Kingdom in the late 20th century. First, there is a short review on the history of the industry in the UK and then there are more present cases presented in the form of BMW-Rover and Tata-Jaguar Land Rover. Finally, the thesis suggests some ideas to which the UK should work towards in order to ensure future competitiveness. The automotive industry in the United Kingdom is one of the oldest in the world, but as the end of last millennium was approaching it was not doing too well. Industry that was still flourishing in the mid-century was soon heading down river and by the end of the century all large English car manufacturers had either closed down or were forced under foreign ownership. The thesis suggests possible targets for future prospects from the literary review and from the conclusions made. These are to ensure the continuity of the industry and the competitiveness on an international level. The suggestions are for long term and are mainly focused around research and development of renewable energy forms.

SOIL ORGANIC CARBON, CARBON STOCK AND THEIR RELATIONSHIPS TO PHYSICAL ATTRIBUTES UNDER FOREST SOILS IN CENTRAL AMAZONIA

ABSTRACT The soil carbon under Amazonian forests has an important roles in global changing, making information on the soil content and depths of these stocks are considerable interest in efforts to quantify soil carbon emissions to the atmosphere.This study quantified the content and soil organic carbon stock under primary forest up to 2 m depth, at different topographic positions, at Cuieiras Biological Reserve, Manaus/ ZF2, km 34, in the Central Amazon, evaluating the soil attributes that may influence the permanence of soil carbon. Soil samples were collected along a transect of 850 m on topographic gradient Oxisol (plateau), Ultisol (slope) and Spodosol (valley). The stocks of soil carbon were obtained by multiplying the carbon content, soil bulk density and trickiness of soil layers. The watershed was delimited by using STRM and IKONOS images and the carbon contend obtained in the transects was extrapolated as a way to evaluate the potential for carbon stocks in an area of 2678.68 ha. The total SOC was greater in Oxisol followed by Spodosol and Ultisol. It was found direct correlations between the SOC and soil physical attributes. Among the clay soils (Oxisol and Ultisol), the largest stocks of carbon were observed in Oxisol at both the transect (90 to 175.5 Mg C ha-1) as the level of watershed (100.2 to 195.2 Mg C ha-1). The carbon stocks under sandy soil (Spodosol) was greater to clay soils along the transect (160-241 Mg C ha-1) and near them in the Watershed (96.90 to 146.01 Mg C ha-1).

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.

Recovery boilers - history and future

Recovery boilers are built all over the world. The roots of recovery technology are longer than the roots of recovery boilers. But it wasn’t until the invention of recovery boilers before the Second World War that the pulping technology was revolutionalized. This led to long development of essentially the same type of equipment, culminating into units that are largest biofuel boilers in the world. Early recovery technology concentrated on chemical recovery as chemicals cost money and if one could recycle these chemicals then the profitability of pulp manufacture would improve. For pulp mills the significance of electricity generation from the recovery boiler was for long secondary. The most important design criterion for the recovery boiler was a high availability. The electricity generation in recovery boiler process can be increased by elevated main steam pressure and temperature or by higher black liquor dry solids as well as improving its steam cycle. This has been done in the modern Scandinavian units.

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.

Vegetational history and geochronology of several sites in South Southwestern Ontario with discussion on mastodon extinction in Southern Ontario /

This study has three purposes: to establish a chronologically controlled vegetational history for a number of sites in south Southwestern Ontario; to utilize the resulting data to support and/or add to the current understanding of Quaternary geology and stratigraphy, and the glacial and postglacial history of the Great Lakes in south Southwestern Ontario; and to attempt to propose a possible explanation for the extinction of the mastodon in Southern Ontario. Palynological and geochronological analyses were conducted on material collected from eleven sites (east to west): Verbeke Mastodon Site, Woloshko Mastodon Site, Walker Pond II, Pond Mills I, Lake Hunger Bog, Bouckaert Site. Mabee Site, Cornell Bog. Colles Lake I, Folden Mastodon Site and Forest Pond. Individual geochronologically controlled (where possible) vegetational histories were reconstructed for each of the sites investigated. The results of the individual studies, when considered in overview. indicated the existance of an established closed boreal forest throughout south Southwestern Ontario by 10,000 years B.P. This evidence for a significant climatic change coincident throughout south Southwestern Ontario supports the proposed age of 10,000 years B.P. for the Pleistocene/Holocene Boundary (Terasmae, 1972). Remnant patches of 'open spruce parkland' persisted in small local 'wet' areas. It was in these areas that the mastodon was restricted during early Holocene time. With continued encroachment by the surrounding boreal forest, possibly speeded up by this browser's destructive feeding habits, the spruce enclaves shrank and the mastodon became extinct in south Southwestern Ontario. The results of this thesis basically support Dreimanis' (1967, 1968) proposed 'Environmental-Climatic' theory for mastodon extinction. It is suggested that increased dryness during the present interglacial compared to the climate of earlier interglacials may be the key to unravelling the problem of mastodon extinction in eastern North America.

History and Theorizing: The Case of Winemaking in the Ontario Wine Industry

Despite the fact that theorization has been established as an important social mechanism in a variety of contexts, little research has explicated how this process works. I argue that theorization entails strategic constructions of history, or rhetorical history, in order to persuade audiences of legitimacy. I examine the role that history plays in actors’ theorizations of the Ontario wine industry as world class. By conducting a rhetorical analysis of the newsletters and websites of Ontario wineries, I find that various themes of history are routinely employed to re-theorize the industry as one that produces world class wines. In general, the findings suggest that the narratives of Ontario wineries tap into global repertoires of fine wine to portray the continuity of current practices with those of Old World winemaking. In addition, wineries sometimes tap into local histories to convey a sense of uniqueness, but they also obscure the history of poor winemaking in the region.

Economic security & reconstruction: utility, history and practice in post-conflict environments

Please consult the paper edition of this thesis to read. It is available on the 5th Floor of the Library at Call Number: Z 9999 P65 D53 2007

The American Weekly Messenger; or Register of State Papers, History and Politics for 1813 - 1814 Volume 1

Register of state papers, history, and politics for the years 1813 - 1814.