39 research outputs found
The Villa-Lobos’ Cirandas for piano: from the children’s cultural universe to important works of the piano repertoire
Neste trabalho busca-se entender o tratamento dado por Villa-Lobos às melodias do cancioneiro infantil utilizadas nas Cirandas Terezinha de Jesus, Xô xô passarinho e Fui no Tororó, através análises entonacionais das melodias homônimas arranjadas no Guia Prático e também das próprias Cirandas. Essas análises são fundamentadas nos conceitos do musicólogo russo B. Asafiev e permitiram entender o universo cultural e histórico relacionado a essas três peças, que integram o conjunto de 16 Cirandas, obra importante no repertório para piano do século XXThis work investigates the Villa-Lobos’ treatment to the melodies used in Cirandas Terezinha de Jesus, Xô xô passarinho and Fui no Tororó, through intonational analyzes of the homonymous melodies arranged in the Guia Prático and also by the analyzes of the Cirandas themselves. These analyzes are based on the concepts of Russian musicologist B. Asafiev and allowed to understand the cultural and historical universe related to these three pieces, which are part of the 16 Cirandas, important work for piano of the twentieth century.
 
1º Caderno de Ponteios para piano de Camargo Guarnieri: uma concepção da totalidade da obra
Neste artigo, busca-se demonstrar que o 1º Caderno de Ponteios de Camargo Guarnieri, pode ser percebido como uma totalidade e não somente como dez peças avulsas.
Para tal utiliza-se a contextualização histórica, a análise musical, a descrição da imagem artística ( segundo H. Neuhaus) de cada Ponteio e um suporte epistemológico que abarca as noções de totalidade, sistema, unidade complexa, partes, interação, organização, emergências presentes no trabalho de Edgar Morin O Metodo 1
Rett Syndrome: Crossing the Threshold to Clinical Translation
Lying at the intersection between neurobiology and epigenetics, Rett syndrome (RTT) has garnered intense interest in recent years, not only from a broad range of academic scientists, but also from the pharmaceutical and biotechnology industries. In addition to the critical need for treatments for this devastating disorder, optimism for developing RTT treatments derives from a unique convergence of factors, including a known monogenic cause, reversibility of symptoms in preclinical models, a strong clinical research infrastructure highlighted by an NIH-funded natural history study and well-established clinics with significant patient populations. Here, we review recent advances in understanding the biology of RTT, particularly promising preclinical findings, lessons from past clinical trials, and critical elements of trial design for rare disorders
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Convergent cerebrospinal fluid proteomes and metabolic ontologies in humans and animal models of Rett syndrome
MECP2 loss-of-function mutations cause Rett syndrome, a neurodevelopmental disorder resulting from a disrupted brain transcriptome. How these transcriptional defects are decoded into a disease proteome remains unknown. We studied the proteome of Rett cerebrospinal fluid (CSF) to identify consensus Rett proteome and ontologies shared across three species. Rett CSF proteomes enriched proteins annotated to HDL lipoproteins, complement, mitochondria, citrate/pyruvate metabolism, synapse compartments, and the neurosecretory protein VGF. We used shared Rett ontologies to select analytes for orthogonal quantification and functional validation. VGF and ontologically selected CSF proteins had genotypic discriminatory capacity as determined by receiver operating characteristic analysis in Mecp2(-/y) and Mecp2(−/+). Differentially expressed CSF proteins distinguished Rett from a related neurodevelopmental disorder, CDKL5 deficiency disorder. We propose that Mecp2 mutant CSF proteomes and ontologies inform putative mechanisms and biomarkers of disease. We suggest that Rett syndrome results from synapse and metabolism dysfunction
Lack of observed tolerance to diazepam nasal spray (Valtoco®) after long-term rescue therapy in patients with epilepsy: Interim results from a phase 3, open-label, repeat-dose safety study.
OBJECTIVE: Tolerance is a known consideration for maintenance use of benzodiazepines and other antiseizure drugs; however, clinical experience suggests that tolerance may not be anticipated with long-term intermittent use of benzodiazepines as rescue therapy. Diazepam nasal spray (Valtoco®) is a proprietary intranasal formulation approved for the acute treatment of intermittent, stereotypic episodes of frequent seizure activity (ie, seizure clusters, acute repetitive seizures) in patients with epilepsy aged ≥6 years. Reported here are exploratory analyses investigating whether there was evidence of development of tolerance in an interim analysis of a long-term, phase 3, open-label safety study of diazepam nasal spray.
METHODS: Patients and care partners were trained to administer 5, 10, 15, or 20 mg of diazepam nasal spray (age- and weight-based dosing), with a second dose administered 4-12 hours later if needed. A series of analyses were performed to assess evidence of tolerance using 2 equal, adjacent time periods and data for each patient to compare the proportion of events for which second doses of diazepam nasal spray (as a proxy for effectiveness) were administered in period 1 compared with period 2.
RESULTS: A total of 175 patients were enrolled at interim cutoff, and 158 were treated with diazepam nasal spray for 3370 seizure-cluster events. For 73.4% of patients, duration of exposure to diazepam nasal spray was ≥12 months. A total of 191 analyses were conducted; the proportion of analyses in which second doses in period 2 were lower than in period 1 was 72.8%. Only 5 analyses showed nominally statistically significant changes (P \u3c 0.05); this is fewer than expected by chance, and these differences were not directionally consistent. There was no safety signal with continued use.
CONCLUSIONS: These analyses found no statistical evidence of tolerance with the use of diazepam nasal spray over time based on use of a second dose in an initial period of the study compared with a subsequent period for each patient. These results are in agreement with prior studies of benzodiazepine rescue therapy
A interpretação das Cirandas de Villa-Lobos no âmbito da Teoria da Entonação de B. Asafiev
This work proposes a performance of Villa-Lobos’ 7 Cirandas. The B. Asafiev’s Theory of Intonation and Musical Form as a Process were used as the basis of the performance. For each Ciranda to be performed, was realized analytical and interpretative conception, entitled here as intonation analisys. In each conception the following items were treated: structuring of musical material, sound flow within time manifested through the Asafiev’s concepts of energy, impulse to the musical movement, musical movement and its conclusion, semantic and syntactic aspects immanent and emanating of the musical structures.Este trabalho propõe a execução de 7 Cirandas de Villa-Lobos, fundamentada nos conceitos formulados pelos trabalhos A Teoria da Entonação e Forma Musical como Processo de B. Asafiev, que concebia a música como a “arte do sentido entonado”. Para cada Ciranda a ser executada realiza-se uma análise entonacional, entendida como uma concepção analítica da peça, na qual os seguintes itens são abordados: a estruturação do material musical, conceitos asafianos de energia, impulso ao movimento musical, movimento musical e seu término, funções dos elementos simultâneos e sucessivos, os aspectos semânticos e sintáticos expressos no fluxo sonoro das peças, dentre outros
A teoria da entonação de B. Asafiev e a execução musical : concepções analíticas para a interpretação das cirandas de Villa-Lobos
Este trabalho investiga parte significativa da obra para piano de Villa-Lobos, as 16 Cirandas, com objetivo de apresentar concepções analíticas e interpretativas para a execução de cada uma das peças. Partindo da busca por um conhecimento que abordasse as questões semânticas e sintáticas, expressas no ato da execução musical, foram adotados, para as análises, os trabalhos de B. Asafiev, Teoria da Entonação e Forma Musical como Processo apresentados e contextualizados frente às teorias analíticas do século XX. Em cada concepção analítica das peças, neste trabalho, com o título de análises entonacionais, os itens a seguir foram tratados: a estruturação do material musical; o fluxo sonoro no tempo manifestado por intermédio dos conceitos “asafianos” de energia, impulso ao movimento musical, movimento musical e seu término, funções dos elementos e conjuntos sonoros simultâneos e sucessivos; os aspectos semânticos e sintáticos, imanentes e emanantes das estruturas musicais; a linguagem pianística de Villa-Lobos; aspectos do processo de execução, como dedilhados, movimentos de dedos, mãos e braços. Integrada à Teoria da Entonação, a execução musical é entendida como entonação de uma obra musical, ou seja, manifestação sonora originada das mãos e voz dos intérpretes, que comunica seu estado psicológico intelectual/emocional/semântico em um contexto cultural e histórico. Por sua vez, a música é entendida por Asafiev como “a arte do sentido entonado”. Conectado à sua contemporaneidade, Villa-Lobos entoa, nas Cirandas, um universo cultural por meio de entonações historicamente cristalizadas no consciente social brasileiro.This work investigates a substantial part of Villa-Lobos’ work for piano, the 16 Cirandas. It proposes analytical and interpretive conceptions to the performance of each musical piece. Searching for principles that would encompass the semantics and syntax expressed in the act of musical performance, B. Asafiev’s Intonation Theory and Musical Form as Process was used as the basis for analysis, being presented and contextualized with the analytical theories of the 20th century. In each analytical conception of the pieces, entitled here as intonation analyses, the following items were treated as a complex: structuring of the musical material; sound flow within time manifested through the Asafie’s concepts of energy, impulse to the musical movement, musical movement and its conclusion; functions of simultaneous and successive sound elements and sets; semantic and syntactic aspects immanent and emanating of musical structures; pianistic language of Villa-Lobos; aspects of the performing process such as fingering, finger, hand and arm movements. Musical performance, integrated to the Theory of Intonation, is understood as intonation of a work of music, thus, manifestation of sound originated in the hands and voice of the interpreters, which communicates their psychological/intellectual/emotional/semantic state in a historical and cultural context. According to Asafiev’s understanding, music is the “art of intoned meaning”. Villa-Lobos, in touch with his contemporaneity, intones in his Cirandas a cultural universe through historically crystallized intonations of the Brazilian social consciousness.Автор представляемого исследования изучает одно из самых значимых произведений Вилла-Лобоса – 16 Cirandas (Сирандас). Он предлагает анализ и различные интерпретационные концепции каждой музыкальной пьесы цикла. При поиске принципов, которые лежат в основе семантики и синтаксиса музыкального произведения, автор опирался в своем анализе на выдающиеся работы Б. Асафьева «Теория интонации» и «Музыкальная форма как процесс», критически представленные в этом исследовании в контексте других созданных в 20 веке музыкальных теорий. В анализе каждой из музыкальных пьес, называемом в работе «Интонационный анализ», как единый комплекс рассматривались следующие элементы: структурирование музыкального материала; звуковой поток в единицу времени в свете теории энергетики искусства Асафьева, импульсы музыкального движения, музыкальное движение, функции синхронных и последовательных звуковых элементов и их сочетаний; присущие музыкальной структуре семантические и синтаксические аспекты; пианистический язык Вилла-Лобоса; такие вопросы исполнительского искусства как аппликатура, туше, движения рук и плеч. Музыкальное исполнение, с точки зрения «Теории интонации», понимается как интонирование музыкального произведения, таким образом, звуковое воплощение происходит в руках и голосе интерпретатора, который передает свое психологическое/ интеллектуальное/эмоциональное/смысловое состояние в данном историческом и культурном контексте. В соответствии с пониманием Б. Асафьева, музыка – это «искусство интонируемого смысла». Вилла-Лобос, будучи неотъемлемой частью своего времени, интонирует в Сирандас культурные универсалии через исторически кристаллизованные интонации бразильского социального сознания
Growth charts for 22q11 deletion syndrome
The purpose of this work was to create growth curves specific to the 22q11.2 deletion syndrome. Growth parameters on 188 patients (86 females, 102 males) followed by a group of three dysmorphologists were collected by retrospective chart review. Growth charts for body mass, length/height, and head circumference were generated using a semi-parametric model with goodness-of-fit tests. The resulting charts show that between 25 and 50% of children with 22q11.2 deletion syndrome fall below the 2nd centile for the normal population for all growth parameters. Establishing norms of growth for 22q11.2 deletion syndrome allows the clinician to identify and investigate those children who deviate substantially from the growth profile of this condition