Thinning of the Corpus Callosum and Cerebellar Atrophy is Correlated with Phenotypic Severity in a Family

Sanjeev Rajakulendran,Coro Paisán-Ruiz,Henry Houlden 대한신경과학회 2011 Journal of Clinical Neurology Vol.7 No.2

Background:Mutations in the spatacsin gene are associated with spastic paraplegia type 11(SPG11), which is the most-common cause of autosomal recessive hereditary spastic paraplegia. Although SPG11 has diverse phenotypes, thinning of the corpus callosum is an important feature. Case Report:Clinical, genetic, and radiological evaluations were undertaken in a large family from Gujarat in North India with hereditary spastic paraplegia, whose affected members presented with varying degrees of spasticity, ataxia, and cognitive impairment. The clinical severity and the degree of corpus callosum and cerebellar atrophy varied among the four affected individuals in the family. Genetic testing of the affected members revealed recessive mutations in the spatacsin gene, consistent with a diagnosis of SPG11. Conclusions;We believe that the extent of corpus callosum thinning and cerebellar atrophy is correlated with disease severity in affected patients. The addition of extrapyramidal features in the most-affected members suggests that SPG11 exhibits considerable phenotypic heterogeneity.

Automatic Shadow Removal Algorithm for VOP, DWT Based Watermarking Algorithm for VOP and Generation of Super Resolved VOP

Alwyn R. Pais,John D‟Souza,Ram Mohana Reddy,P.Hari Krishna 보안공학연구지원센터 2011 International Journal of Multimedia and Ubiquitous Vol.6 No.3

‘한국교회의 복음화와 토착화 현실, 그리고 미래전망’에 대한 논평 1

배형진(Jacob Pais SVD) 한국그리스도사상연구소 2011 한국그리스도사상 Vol.- No.19

Um autor português do século XIX ainda próximo de nós A novelística de Camilo Castelo Branco

( Maria João Pais Do Amaral ) 한국포르투갈-브라질학회 2005 포르투갈-브라질 연구 Vol.2 No.1

The main purpose of this paper is to present a 19th century Portuguese novelist whose works are not as well known abroad as those of Camões, Pessoa or the Nobel Prize winner Saramago. Nonetheless, he is one of the most representative names of Portuguese Romanticism and, perhaps, one of the most genuine Portuguese writers of all times. Moreover, his works are a precious testimony of Portuguese 19th century’s major historical and social conflicts. Entering Camilo Castelo Branco’s fictional world can, thus, be regarded as one of the best ways of being acquainted with Portuguese history, culture and people. But it’s also a means of having knowledge of an extraordinary story-teller, whose adventurous plots and varied characters representing the worst and the best of mankind attract our attention - anytime, anywhere. The universality of Camilo’s novels partially lies on the fact that, although events take place in Portugal more than one century ago, although characters live and love, struggle and die in a distant country, at a distant time, they are all brought to life by the powerful words of an author who puts them before the reader’s eyes as if they were real people, living nearby or appearing daily on our TV screens. After all, the romantic technique of the feuilleton, which Camilo and most of his contemporary European fellow-writers mastered, is not that different from contemporary TV episodes writing technique. Causing strong feelings and emotions, moving readers or spectators into tears or laughter, sympathy or animadversion, hate or compassion, are crucial ingredients to catch any public’s attention and keep it alive. As a journalist and professional writer, Camilo was well aware of it. That’s why the author never shows indifference towards any character or event of the stories he tells. He makes comments and judgments. He addresses the reader as a friend and leads him to share his points of view. He talks passionately in order to make the reader feel passion. He claims he actually met some of the characters, or witnessed some of the events. For that reason, his autobiographical data also play an important role, as well as contemporary socio-political and historical events. Needless to say, passionate love stories between young people from different social or economical backgrounds constitute the central element of this overwhelming universe, in which individual happiness and collective destinies prove to be inseparable. As a matter of fact, Camilo’s love stories are more than mere love stories because the heroes’ adventures and misfortunes originate in a world where money meant more than human beings and marriage had nothing to do with love. These things are an essential part of the accurate portrayal of Portuguese 19th century bourgeois status that the author depicts in his works, accusing society of being dominated by materialism, prejudice and hypocrisy. Ironically, while suiting the bourgeois readers’ demands, who appreciated adventurous love stories and moral lessons, Camilo went further in his pedagogical labour and pointed his finger at those who made it possible for him to earn a living… And, although he didn’t believe in fiction’s persuasive power, he never stopped praising those things he considered true values: compassion, solidarity, forgiveness, fraternity… in one word, and above all, love. These are the main values his heroes fight for - and also any reader will recognize as the noblest human values.

Príncipes encobertos e princesas enamoradas: do disfarce por amor no teatro de Gil Vicente

Maria João Pais do Amaral 한국 포르투갈-브라질 학회 2008 포르투갈-브라질 연구 Vol.5 No.1

The main object of this essay is love disguise in two plays of Gil Vicente, Portuguese dramatist who developed his theatrical activity between 1502 and 1536 under the patronage of the monarchs Manuel I and João III. Widely spread in 16th century European literature, the motif of love disguise has its origins in chivalry romances, which are one of the many diverse sources of Gil Vicente's court theatre and also one of the dramatist's first spectators' favourite kind of books. I analyse it in Comédia do Viúvo and Tragicomédia de D. Duardos, where the protagonists, princes Rosvel and Duardos, hide their true identity and social rank from their beloved in order to prove the authenticity of their affection. In a first moment, I compare the way each of these two princes uses love disguise, reflecting on the function and meaning of the obvious differences this comparative analysis brings into light. In a second moment, I relate those differences with both the dramatic genre each of these two plays belongs and the conception of love that is on the basis of the stories enacted in Viúvo and Duardos, which, although originating in courtly love conventions, goes far beyond it. Finally, and because Gil Vicente's theatre is an art that strongly depends on circumstances, the date on which these spectacles were presented for the first time before the Portuguese king and court, as well as the purposes they were meant to fulfil, are also taken into consideration in this reflection on the innovative use of love disguise motif by one of the most important Portuguese dramatists of all times. The main object of this essay is love disguise in two plays of Gil Vicente, Portuguese dramatist who developed his theatrical activity between 1502 and 1536 under the patronage of the monarchs Manuel I and João III. Widely spread in 16th century European literature, the motif of love disguise has its origins in chivalry romances, which are one of the many diverse sources of Gil Vicente's court theatre and also one of the dramatist's first spectators' favourite kind of books. I analyse it in Comédia do Viúvo and Tragicomédia de D. Duardos, where the protagonists, princes Rosvel and Duardos, hide their true identity and social rank from their beloved in order to prove the authenticity of their affection. In a first moment, I compare the way each of these two princes uses love disguise, reflecting on the function and meaning of the obvious differences this comparative analysis brings into light. In a second moment, I relate those differences with both the dramatic genre each of these two plays belongs and the conception of love that is on the basis of the stories enacted in Viúvo and Duardos, which, although originating in courtly love conventions, goes far beyond it. Finally, and because Gil Vicente's theatre is an art that strongly depends on circumstances, the date on which these spectacles were presented for the first time before the Portuguese king and court, as well as the purposes they were meant to fulfil, are also taken into consideration in this reflection on the innovative use of love disguise motif by one of the most important Portuguese dramatists of all times.

Metamorfoses do conto: a propósito de Short Movies de Gonçalo M. Tavares

Maria João Pais do Amaral 한국 포르투갈-브라질 학회 2015 포르투갈-브라질 연구 Vol.12 No.3

The objective of this essay is to demonstrate that the texts included in Short Movies are innovative short stories that imitate cinema procedures in order to serve the purposes of a markedly contemporary practice of writing aimed at slight transformations of traditional literary forms. The extreme brevity of these texts, the ironic detachment of the narrator regarding its unnamed characters and its few but often harsh events are part of that goal. So is the way the narrator brings into broad light some of the darker aspects of human nature: by giving a new life to the ancient literary technique known as ante oculos, he places what happens before our eyes as a camera would, showing it as live images through realistic, accurate and often dynamic, but also fragmentary, descriptions. Images and fragments also contribute to break the Aristotelian canons. Yet, the main features that define the short story as a literary genre are still recognizable in most of these short movies, including those where nothing seems to happen or where part of what happens remains unknown. As to the ironic detachment of the narrator, it is not a cruel indifference sign but rather a way of keeping neutrality – and, above all, another effective means of leading the reader to reflect on a disturbing and shocking reality, in which random, fear, hunger, aggressiveness, struggle for domination and other primary instincts play a more decisive role in human life than we are usually willing to admit. El objetivo de este ensayo es demostrar que los textos incluidos en Short Movies son cuentos innovadores que imitan los procedimientos del cine a fin de servir los propósitos de una práctica de escritura contemporánea interesada en ligeras transformaciones de las formas literarias tradicionales. La extrema brevedad de estos textos, el distanciamiento irónico del narrador con respecto a sus personajes anónimos y sus pocos pero a menudo crueles eventos son parte de ese objetivo. También lo es la forma en que el narrador pone en plena luz algunos de los aspectos más sombríos de la naturaleza humana: dando una nueva vida a la antigua técnica literaria conocida como ante oculos, coloca lo que sucede ante nuestros ojos, mostrándolo como imágenes en directo mediante descripciones realistas, precisas y a menudo dinámicas, sino también fragmentarias. Imágenes y fragmentos contribuyen a infringir los cánones aristotélicos. Sin embargo, las principales características que definen el cuento como género literario son aún reconocibles en la mayoría de estos textos, incluyendo aquellos donde parece que nada sucede o donde se desconoce parte de lo que sucede. En cuanto al distanciamiento irónico del narrador, no es un signo de indiferencia cruel, sino más bien una forma de mantener la neutralidad – y, sobre todo, otro medio eficaz de conducir al lector a reflexionar sobre una realidad inquietante, en la que el azar, el miedo, el hambre, la agresividad, la lucha por la dominación y otros instintos primarios juegan un papel decisivo en la vida humana.

Psychological aspects in children and parents of children with chronic kidney disease and their families

Aier Alemsungla,Pais Priya,Raman Vijaya 대한소아청소년과학회 2022 Clinical and Experimental Pediatrics (CEP) Vol.65 No.5

Pediatric chronic kidney disease (CKD) is a chronic illness that affects the overall quality of life of patients during childhood. This article highlights the psychological and social burden of CKD in patients and their families. Patients with CKD and their families require comprehensive treatment for psychosocial problems. Therefore, it is crucial for pediatricians to screen for these issues and refer patients and their families for therapy. Tools that are short, easy to administer, and easy to score, such as the Pediatric Quality of Life Inventory or the Childhood Depression Inventory, can be utilized during routine clinical appointments. Reducing the negative impact of CKD on the family will improve the well-being and coping skills of patients and their families.

Poster Session:PS 0184 ; Endocrinology : Multiple Endocrine Neoplasia Type 2B - A Late Presentation

( Ines Vendrell ),( Filipe Filipe ),( Helena Luna Pais ),( Joana Martins ),( Catarina Espirito Sabto ),( Antonio Pedro Machado ),( Paulo Cantiga Duarte ),( Jose Braz Nogueira ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: Multiple Endocrine Neoplasia (MEN) is a rare condition which occurs 1-2/100.000 people and results from rearranged during transfection (RET) protooncogene mutations. MEN B is a particularly infrequent subtype of MEN (5% of all MENs) that usually presents during childhood with pseudo-hirschprung`s disease and skeletal abnormalities (marfanoid habitus). Medullary thyroid carcinoma occurs in almost all individuals and phaeochromocitoma can also be present. MEN 2B is an autosomal dominant disease. Yet, less than 50% of the patients have a family history (de novo mutations), as many perish before reproductive age. The authors describe the case of a 33-year-old male who presented with hypertension, tachycardia and high blood sugar levels. He had a history of cryptorchidism and bowel obstruction both surgically corrected during childhood. Moreover at the age of 30 he was diagnosed with ruptured diverticulitis and underwent a total colectomy (specimen showed reactive lymphoid hyperplasia and multiple diverticula). This patient had the classic marfanoid habitus and mucosal neuromas (lips and tongue), which prompted urine cathecolamine and metanephrine dosing. Abdominal CT scan showed bilateral adrenal masses (25mm and 77mm diameter) compatible with phaeochromocytoma. Given the phenotype, serum calcitonin dosing (over 2000 pg/mL), thyroid ultrasound (multiple nodules) and biopsy were performed, confi rming medullary thyroid carcinoma. Genetic testing reveled a germline point mutation in RET (p.M918T) known to be associated with MEN 2B. The patient later underwent total thyroidectomy with lymphadenectomy and bilateral adrenalectomy. Conclusions: Even in the presence of its classical features, MEN 2B is a challenging diagnosis. This case emphasizes the complexity of this disease and the need for recognizing this rare, yet potentially lethal condition.

Safety of Nonoperative Management After Acute Diverticulitis

Javier Suarez Alecha,Sonia Amoza Pais,Xavi Batlle Marin,Begoña Oronoz Martinez,Enrique Balen Ribera,Concepción Yarnoz Irazabal 대한대장항문학회 2014 Annals of Coloproctolgy Vol.30 No.5

Purpose: The role of surgery in the management of diverticular disease after an episode of acute diverticulitis (AD) managed in a conservative form is evolving. Age, number of episodes of AD, type of episode, and symptoms after the episodes are factors related to the need for elective surgery. The aim of this study is to evaluate the safety of conservative management and the risk factors for emergency surgery after a first episode of AD managed without surgery. Methods: We retrospectively evaluated 405 patients diagnosed as having had a first episode of AD. Sixty-nine patients underwent emergency surgery on the first admission, and 69 patients had an elective operation in the follow-up (group A). The remaining 267 patients were managed initially without surgery (group B). Thirteen of these 267 patients needed a further urgent surgical procedure. Factors involved in the decision of elective surgery and the probability of emergency surgery after the first episode of AD managed without surgery were evaluated in relation to demographic factors, risk factors, presence of recurrences, and type of the first episode. Results: Patients, mean age was 62.7 years, 71 were aged less than 51, and 151 were males. The mean follow-up for patients with nonoperative management was 91.2 months. An elective operation was performed in 69 patients. Compared to patients in group B, those in group A more frequently had a first episode of complicated acute diverticulitis (CAD) (37.1% vs. 16.4%; P = 0.000) and were more likely to be smokers (46.3% vs. 19.3%; P = 0.000) and to suffer more than one episode of AD (42% vs. 26.9%; P = 0.027). Nonoperative management was chosen for 267 patients, but 13 patients needed an emergency operation later. In the multivariate analysis, we found a significant relation between the presence of CAD in the first episode and the need for emergency surgery. There were no differences in surgical mortality between the patients in the two groups, but patients treated with elective surgery had a higher rate of stoma than patients treated non-operatively (7.2% vs. 1.4%; P = 0.028); this difference was not observed in the subgroup of patients with CAD (15.3% vs. 6.8%; P = 0.458). Conclusion: After an episode of AD, nonoperative management is safe because fewer than 5% of patients will need an emergent procedure in a subsequent attack of AD. A first episode of CAD is the only risk factor for emergency surgery in patients managed conservatively.

Novel strategies targeting pathogen transmission reduction in insect vectors: Tsetse-transmitted trypanosomiasis control

Brian L. WEISS,Geoffrey M. ATTARDO,Roshan PAIS,Jingwen WANG,Serap AKSOY 한국곤충학회 2007 Entomological Research Vol.37 No.4

Insect vectors are essential for the transmission of important human diseases such as malaria, leishmaniasis, Chagas and sleeping sickness. Insects are also responsible for the transmission of agricultural diseases that affect livestock and crops. Traditionally, control of the vector populations has been an effective disease management strategy. Recently, vector control strategies have been fortified by research in insect biology and in insect-pathogen interactions as well as by the development of transgenic technologies. In addition to insect population reduction methods, disease control via selective elimination of pathogens in insects can now be explored. Here we explore the tsetse vectors of African trypanosomes and describe the application of recent knowledge gained in their symbiotic, reproductiveand vectorial biology to develop novel disease control strategies.