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OBJECTIVE: Clubfoot is one of the most common congenital deformities affecting the musculoskeletal system. The main conservative treatment for clubfoot includes the Ponseti's and Kite's methods. This study aimed to perform a comparative evaluation of the effectiveness of the Ponseti and Kite approaches for the conservative treatment of clubfoot. METHODS: One hundred children with clubfoot abnormality, visiting the Martagão Gesteira Hospital, were divided in two groups. The first group received conservative treatment using the Kite method and the second group received conservative treatment using the Ponseti method. At the end of the treatment, both groups were evaluated based on the Pirani classification to verify whether there had been an adequate correction of the deformity. The variables studied included correction of deformity, age, sex, and laterality. RESULTS: The effectiveness of the Kite and Ponseti methods of conservative treatment showed significant statistical differences. CONCLUSION: The present study shows that the efficacy of conservative treatment using the Ponseti method was 18% higher than that of the Kite method. Level of evidence II, randomized clinical trial.
OBJETIVO: O Pé Torto Congénito Idiopático (PTC I) é um dos defeitos congénitos mais comuns, que envolvem o sistema músculo-esquelético, cujos principais tratamentos conservadores são através do método de Kite e de Ponseti. A presente pesquisa busca uma avaliação comparativa da eficácia dos métodos de Ponseti e de Kite, no tratamento conservador do PTC I. METODOLOGIA: Foram tratadas 100 crianças portadoras da deformidade do PTC I, no Hospital Infantil Martagão Gesteira, sendo divididas em dois grupos. No primeiro grupo foi instituído o tratamento conservador pelo método de Kite e no segundo grupo foi instituído o tratamento conservador pelo método de Ponseti. Ao final do tratamento, ambos os grupos foram avaliados com base na classificação de Pirani para verificar se houve correção adequada da deformidade. Foram estudadas as variáveis de correção da deformidade, idade, sexo e lateralidade. RESULTADOS: Houve diferença estatisticamente significante entre os métodos de tratamento conservador de Kite e de Ponseti. CONCLUSÃO: O presente estudo demonstrou que a eficácia do tratamento conservador pelo método de Ponseti foi superior em 18% em relação ao método do Kite. Nível de evidência II, ensaio clínico randomizado.
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To compare transcranial sonography (TCS) findings in patients with predominantly neurological Wilson's disease (WD) to those from controls, and to correlate TCS data with the clinical profile of WD. Patients with WD (n=40/f=18) and healthy, matched controls (n=49/f=20) were assessed in terms of TCS, serum copper and iron parameters, and clinical scales, such as the Unified Wilson's Disease Rating Scale (UWDRS), Addenbrooke's Cognitive Examination-Revised (ACE-R), Mini Mental State Examination (MMSE), and Beck Depression Inventory. Lenticular nuclei and substantia nigra echogenic area cut-off values clearly differentiated WD patients from controls (area under the curve: 95.4% and 79.4%). Substantia nigra echogenic area was significantly larger in male than in female patients (p=0.001). Compared with controls, patients showed hyperechogenicity also in thalami and midbrain tegmentum/tectum; third ventricle width was increased and midbrain axial area was reduced. In the WD group, male gender correlated with substantia nigra echogenic area (r=0.515, p=0.0007) and serum ferritin levels (r=0.479, p=0.002); lenticular nuclei hyperechogenicity correlated with dystonia (r=0.326, p=0.04) and dysarthria (r=0.334, p=0.035); third ventricle width correlated with dystonia (r=0.439 p=0.005), dysarthria (r=0.449, p=0.004), parkinsonism (r=0.527, p<0.001), UWDRS neurological and total scores (both r=0.504, p=0.0009), MMSE (r=-0.496, p=0.001), and ACE-R (r=-0.534, p=0.0004). Lenticular nuclei echogenic area allowed highly accurate discrimination between patients and controls. The gender differences in substantia nigra echogenicity and iron metabolism are of interest in further studies in WD. TCS reflects different dimensions of WD pathology clearly differentiable from healthy controls and correlating with various clinical characteristics of WD.
Assuntos
Núcleo Caudado/diagnóstico por imagem , Corpo Estriado/diagnóstico por imagem , Degeneração Hepatolenticular/diagnóstico por imagem , Substância Negra/diagnóstico por imagem , Ultrassonografia Doppler Transcraniana/métodos , Adulto , Feminino , Degeneração Hepatolenticular/fisiopatologia , Humanos , Masculino , Curva ROC , Adulto JovemRESUMO
The isolation of neutralizing monoclonal antibodies (nmAbs) against the Zika virus (ZIKV) might lead to novel preventative strategies for infections in at-risk individuals, primarily pregnant women. Here we describe the characterization of human mAbs from the plasmablasts of an acutely infected patient. One of the 18 mAbs had the unusual feature of binding to and neutralizing ZIKV despite not appearing to have been diversified by affinity maturation. This mAb neutralized ZIKV (Neut50 ~ 2 µg/ml) but did not react with any of the four dengue virus serotypes. Except for the expected junctional diversity created by the joining of the V-(D)-J genes, there was no deviation from immunoglobulin germline genes. This is a rare example of a human mAb with neutralizing activity in the absence of detectable somatic hypermutation. Importantly, binding of this mAb to ZIKV was specifically inhibited by human plasma from ZIKV-exposed individuals, suggesting that it may be of value in a diagnostic setting.
Assuntos
Anticorpos Neutralizantes/imunologia , Anticorpos Antivirais/imunologia , Mutação em Linhagem Germinativa , Epitopos Imunodominantes/imunologia , Infecção por Zika virus/imunologia , Zika virus , Sequência de Aminoácidos , Anticorpos Monoclonais , Anticorpos Neutralizantes/genética , Anticorpos Antivirais/genética , Citometria de Fluxo , Humanos , Imunoglobulina G , Masculino , Pessoa de Meia-Idade , Infecção por Zika virus/sangue , Infecção por Zika virus/virologiaRESUMO
OBJECTIVE: Quantitative data are reported on rapid eye movement (REM) sleep behavior disorder (RBD) in a cohort of predominantly neurological Wilson's disease (WD). METHODS: A total of 41 patients with WD and 41 healthy, age- and gender-matched controls were studied by conducting face-to-face interviews, neurological and clinical examinations, laboratory tests, and WD- and RBD-specific scales. Video-polysomnography and quantification of REM sleep without atonia (RWA) were conducted in 35 patients and 41 controls. RESULTS: Patients with WD showed significantly worse sleep quality, less sleep efficiency, increased wakefulness after sleep onset, and more arousals compared to healthy controls. Five patients with WD (four women) fulfilled the diagnostic criteria for RBD with significantly higher values in RWA, RBD Questionnaire-Hong Kong, and RBD Screening Questionnaire compared to patients with WD without RBD. In three patients with WD, RBD had manifested before any other symptom that could be attributed to WD. Percentage of RWA was significantly lower in WD without RBD than in WD with RBD, but still significantly increased compared to controls. CONCLUSIONS: RBD can be comorbid with WD. RWA is commonly present in WD, both in the presence or absence of clinical RBD. A causal connection is possible, though retrospective determination of RBD onset and the low number of patients do not allow a definitive conclusion at this point. However, screening for WD in idiopathic RBD is available at low cost and is recommended. Early-stage copper chelation therapy provides a highly effective treatment to prevent further WD manifestations and might also control the comorbid RBD.
Assuntos
Degeneração Hepatolenticular/complicações , Transtorno do Comportamento do Sono REM/complicações , Adolescente , Adulto , Feminino , Degeneração Hepatolenticular/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Polissonografia , Transtorno do Comportamento do Sono REM/fisiopatologia , Inquéritos e QuestionáriosRESUMO
Objective. Violent dream content and its acting out during rapid eye movement sleep are considered distinctive for rapid eye movement sleep behaviour disorder (RBD). This study reports first quantitative data on dreaming in a cohort of patients with treated Wilson's disease (WD) and in patients with WD with RBD. Methods. Retrospective questionnaires on different dimensions of dreaming and a prospective two-week home dream diary with self-rating of emotions and blinded, categorical rating of content by an external judge. Results. WD patients showed a significantly lower dream word count and very few other differences in dream characteristics compared to age- and sex-matched healthy controls. Compared to WD patients without RBD, patients with WD and RBD reported significantly higher nightmare frequencies and more dreams with violent or aggressive content retrospectively; their prospectively collected dream reports contained significantly more negative emotions and aggression. Conclusions. The reduction in dream length might reflect specific cognitive deficits in WD. The lack of differences regarding dream content might be explained by the established successful WD treatment. RBD in WD had a strong impact on dreaming. In accordance with the current definition of RBD, violent, aggressive dream content seems to be a characteristic of RBD also in WD.
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Sonhos/psicologia , Degeneração Hepatolenticular/fisiopatologia , Adulto , Brasil , Estudos de Casos e Controles , Transtornos Cognitivos , Sonhos/fisiologia , Emoções , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/psicologia , Polissonografia , Estudos Prospectivos , Transtorno do Comportamento do Sono REM , Estudos Retrospectivos , Autorrelato , Sono REM/fisiologia , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: To describe characteristics of REM sleep behavior disorder in Wilson's disease. METHOD: Questionnaire-based interviews (patients and relatives), neurological examinations, two-week prospective dream-diary, video-polysomnography, transcranial sonography, MRI. RESULTS: Four Wilson's disease cases with REM sleep behavior disorder were described; three had REM sleep behavior disorder as initial symptom. All showed mesencephalic tegmental/tectal sonographic hyperechogenicities and two presented ponto-mesencephalic tegmental MRI hyperintensities. CONCLUSION: This first description of REM sleep behavior disorder in Wilson's disease in literature documents REM sleep behavior disorder as a possible presenting symptom of Wilson's disease and adds further evidence to the parallelism of Parkinson's disease and Wilson's disease in phenotype and brainstem topography, which ought to be further studied. REM sleep behavior disorder has prognostic relevance for neurodegeneration in α-synucleinopathies. In Wilson's disease, usefulness of early diagnosis and treatment are already well established. REM sleep behavior disorder in Wilson's disease offers a possible theoretical model for potential early treatment in this extrapyramidal and brainstem paradigm syndrome, previewing the possibility of neuroprotective treatment for REM sleep behavior disorder in "pre-clinical" Parkinson's disease.
Assuntos
Degeneração Hepatolenticular/fisiopatologia , Transtorno do Comportamento do Sono REM/fisiopatologia , Adulto , Diagnóstico Precoce , Feminino , Degeneração Hepatolenticular/tratamento farmacológico , Degeneração Hepatolenticular/patologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Doenças Neurodegenerativas/fisiopatologia , Exame Neurológico , Polissonografia , Transtorno do Comportamento do Sono REM/tratamento farmacológico , Transtorno do Comportamento do Sono REM/patologia , Inquéritos e Questionários , Ultrassonografia Doppler Transcraniana , Adulto JovemRESUMO
ABSTRACT Objective: Clubfoot is one of the most common congenital deformities affecting the musculoskeletal system. The main conservative treatment for clubfoot includes the Ponseti's and Kite's methods. This study aimed to perform a comparative evaluation of the effectiveness of the Ponseti and Kite approaches for the conservative treatment of clubfoot. Methods: One hundred children with clubfoot abnormality, visiting the Martagão Gesteira Hospital, were divided in two groups. The first group received conservative treatment using the Kite method and the second group received conservative treatment using the Ponseti method. At the end of the treatment, both groups were evaluated based on the Pirani classification to verify whether there had been an adequate correction of the deformity. The variables studied included correction of deformity, age, sex, and laterality. Results: The effectiveness of the Kite and Ponseti methods of conservative treatment showed significant statistical differences. Conclusion: The present study shows that the efficacy of conservative treatment using the Ponseti method was 18% higher than that of the Kite method. Level of evidence II, randomized clinical trial.
RESUMO Objetivo: O Pé Torto Congénito Idiopático (PTC I) é um dos defeitos congénitos mais comuns, que envolvem o sistema músculo-esquelético, cujos principais tratamentos conservadores são através do método de Kite e de Ponseti. A presente pesquisa busca uma avaliação comparativa da eficácia dos métodos de Ponseti e de Kite, no tratamento conservador do PTC I. Metodologia: Foram tratadas 100 crianças portadoras da deformidade do PTC I, no Hospital Infantil Martagão Gesteira, sendo divididas em dois grupos. No primeiro grupo foi instituído o tratamento conservador pelo método de Kite e no segundo grupo foi instituído o tratamento conservador pelo método de Ponseti. Ao final do tratamento, ambos os grupos foram avaliados com base na classificação de Pirani para verificar se houve correção adequada da deformidade. Foram estudadas as variáveis de correção da deformidade, idade, sexo e lateralidade. Resultados: Houve diferença estatisticamente significante entre os métodos de tratamento conservador de Kite e de Ponseti. Conclusão: O presente estudo demonstrou que a eficácia do tratamento conservador pelo método de Ponseti foi superior em 18% em relação ao método do Kite. Nível de evidência II, ensaio clínico randomizado.
RESUMO
INTRODUCTION: The act of swallowing depends on a complex and dynamic process which uses common structures to the act of breathing; respiratory problems can cause swallowing difficulties. AIM: To assess the swallowing pharyngeal phase in patients with chronic cough. METHOD: Retrospective study with 15 patients of both genders, patients with chronic cough and risk factors for aspiration defined by the pneumologic diagnosis. The patients were submitted to anamnesis on complaints related to swallowing, chewing and breathing, or related to food and to videofluoroscopic examination. RESULTS: It was observed that 33.3% had normal and functional swallowing, being the last one of most prevalence. The mild dysphagia was observed in 20% of the patients, the mild to moderate dysphagia in 6.7% of them. In relation to the Rosenbek scale, 73.3% of patients presented degree 1, 6.7% presented degrees 2 and 3, and 13.3% presented degree 8. The most found pathology was the chronic cough with 40%, followed by asthma with 20%; 69.2% of patients presented stasis and of these, five used protection maneuvers, of these, seven were effective and only three were used in the presence of stasis. The most used maneuver was the multiple swallowing, being effective in 100%. CONCLUSION: There are peculiarities in the patients' swallowing with chronic cough that, although not presenting complaints relating to swallowing, it presents an important aspiration risk due to the presence of changes in breathing pattern that can intervene in the coordination between breathing and swallowing, which is essential to protect the lower airway.
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RESUMO: Objetivo: analisar os efeitos do exercício respiratórios na biomecânica da deglutição de sujeitos normais. Métodos: o exercício muscular respiratório em sujeitos normais foi aplicado por sete dias consecutivos por meio de incentivador respiratório a fluxo (três séries de dez repetições para inspiração e expiração). A biomecânica da deglutição foi avaliada por videofluoroscopia, utilizando variáveis temporais (tempo de transição faríngea) e visuoperceptuais (número de deglutições, resíduos em seios piriformes e valéculas, penetração/aspiração). Para análise estatística foi aplicado o Teste de Wilcoxon, Igualdade de Duas Proporções e Kappa. Resultados: foram avaliadas 16 jovens do sexo feminino com média de idade de 21,2±3,4 anos. Nas variáveis visuoperceptuais observou-se concordância quase perfeita entre os avaliadores (p<0,001), bem como na temporal (p = 1,00). Após o período de treinamento houve redução no tempo de transição faríngea (p=0,02). Conclusão: o uso de incentivador respiratório a fluxo influenciou significantemente na biomecânica da deglutição, principalmente na redução do tempo de transição faríngea.
ABSTRACT: Purpose: to analyze the effects of the respiratory exercise in the biomechanics of swallowing in normal subjects. Methods: the muscle respiratory exercise in normal subjects was performed for seven consecutive days by the incentive spirometer flow (three sets of ten repetitions for inspiration and expiration).The biomechanics of swallowing was evaluated by video fluoroscopy through temporal variables (pharyngeal transition time) and visual-perceptual (number of swallows, waste in the pyriform sinuses and vallecula, penetration/aspiration). For statistical analysis, the Wilcoxon test, Equality of Two Proportions and Kappa were used. Results: 16 young women with a mean age of 21,2±3,4 years were evaluated. In visual-perceptual variables it was observed almost perfect agreement between evaluators (p<0,001) and in temporal variable (p=1,00). After the training period there was a significant reduction in the pharyngeal transition time (p=0,02). Conclusion: the use of incentive spirometer flow significantly influenced the biomechanics of swallowing, especially in reducing the pharyngeal transition time.
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Objective To describe characteristics of REM sleep behavior disorder in Wilson’s disease. Method Questionnaire-based interviews (patients and relatives), neurological examinations, two-week prospective dream-diary, video-polysomnography, transcranial sonography, MRI. Results Four Wilson’s disease cases with REM sleep behavior disorder were described; three had REM sleep behavior disorder as initial symptom. All showed mesencephalic tegmental/tectal sonographic hyperechogenicities and two presented ponto-mesencephalic tegmental MRI hyperintensities. Conclusion This first description of REM sleep behavior disorder in Wilson’s disease in literature documents REM sleep behavior disorder as a possible presenting symptom of Wilson’s disease and adds further evidence to the parallelism of Parkinson’s disease and Wilson’s disease in phenotype and brainstem topography, which ought to be further studied. REM sleep behavior disorder has prognostic relevance for neurodegeneration in α-synucleinopathies. In Wilson’s disease, usefulness of early diagnosis and treatment are already well established. REM sleep behavior disorder in Wilson’s disease offers a possible theoretical model for potential early treatment in this extrapyramidal and brainstem paradigm syndrome, previewing the possibility of neuroprotective treatment for REM sleep behavior disorder in “pre-clinical” Parkinson’s disease. .
Objetivo Descrever características do transtorno comportamental do sono REM (TCSR) na doença de Wilson (DW). Método Aplicação de entrevistas, vídeo-polissonografia, sonografia transcraniana (STC), ressonância magnética (RM), diário de sonhos. Resultados Descrevemos quatro casos de DW com TCSR. Três apresentaram o TCSR como primeira manifestação. Todos mostraram hiperecogenicidades mesencefálicas na STC, dois apresentaram hiperintensidades ponto-mesencefálicas na RM. Conclusão Esta é a primeira descrição do TCSR na DW. Relatamos o TCSR como um sintoma inicial da DW. Acrescentamos prova para o paralelismo entre a doença de Parkinson e DW, com relação aos fenótipos e localização das lesões cerebrais. Nas alfa-sinucleinopatias, o TCSR tem relevância prognóstica quanto à neurodegeneração. Na DW, já conhecemos a importância de diagnóstico e tratamento precoces. O TCSR na DW oferece um modelo para antecipar o tratamento desta síndrome de acometimento dos núcleos basais e tronco, vislumbrando a possibilidade de tratamento neuroprotetor para a fase “pré-clínica” da DP. .
Assuntos
Adulto , Feminino , Humanos , Masculino , Adulto Jovem , Degeneração Hepatolenticular/fisiopatologia , Transtorno do Comportamento do Sono REM/fisiopatologia , Diagnóstico Precoce , Degeneração Hepatolenticular/tratamento farmacológico , Degeneração Hepatolenticular/patologia , Imageamento por Ressonância Magnética , Exame Neurológico , Doenças Neurodegenerativas/fisiopatologia , Polissonografia , Transtorno do Comportamento do Sono REM/tratamento farmacológico , Transtorno do Comportamento do Sono REM/patologia , Inquéritos e Questionários , Ultrassonografia Doppler TranscranianaRESUMO
Introduction: The act of swallowing depends on a complex and dynamic process which uses common structures to the act of breathing; respiratory problems can cause swallowing difficulties. Aim: To assess the swallowing pharyngeal phase in patients with chronic cough. Method: Retrospective study with 15 patients of both genders, patients with chronic cough and risk factors for aspiration defined by the pneumologic diagnosis. The patients were submitted to anamnesis on complaints related to swallowing, chewing and breathing, or related to food and to videofluoroscopic examination. Results: It was observed that 33.3% had normal and functional swallowing, being the last one of most prevalence. The mild dysphagia was observed in 20% of the patients, the mild to moderate dysphagia in 6.7% of them. In relation to the Rosenbek scale, 73.3% of patients presented degree 1, 6.7% presented degrees 2 and 3, and 13.3% presented degree 8. The most found pathology was the chronic cough with 40%, followed by asthma with 20%; 69.2% of patients presented stasis and of these, five used protection maneuvers, of these, seven were effective and only three were used in the presence of stasis. The most used maneuver was the multiple swallowing, being effective in 100%. Conclusion: There are peculiarities in the patients' swallowing with chronic cough that, although not presenting complaints relating to swallowing, it presents an important aspiration risk due to the presence of changes in breathing pattern that can intervene in the coordination between breathing and swallowing, which is essential to protect the lower airway...
Assuntos
Humanos , Masculino , Feminino , Doença Crônica , Tosse , Fluoroscopia/métodos , Estudos Retrospectivos , Transtornos de Deglutição/etiologia , Fatores de RiscoRESUMO
O nitrogênio (N) aplicado na superfície do solo em vinhedos pode ser perdido por volatilização ou lixiviação, sendo uma das possíveis explicações para o baixo aproveitamento de N pelas videiras. O trabalho objetivou avaliar as perdas de N por volatilização e lixiviação do N mineral em solo cultivado com videira submetida à adubação nitrogenada. Foram instalados dois experimentos em um vinhedo de Cabernet Sauvignon, em Rosário do Sul (RS), Brasil. No experimento 1, foram instalados lisímetros no solo que recebeu a aplicação de 0, 40, 80 e 120kg de N ha-1 ano-1 na forma de ureia e, durante o período de agosto a dezembro de 2009, foi coletada a solução do solo para a análise de N mineral. No experimento 2, foram instaladas câmaras coletoras no solo que recebeu, em agosto de 2010, a aplicação de 0, 20, 40, e 80kg de N ha-1 ano-1 na forma de ureia, 40kg de N ha-1 ano-1 de composto orgânico e 40kg de N ha-1 ano-1 de ureia revestida com polímeros, para avaliar a volatilização de N-NH3 do momento da aplicação até 80 horas depois da aplicação. As maiores concentrações de nitrogênio mineral na solução lixiviada foram encontradas nas doses mais elevadas de fertilizante mineral aplicado e ao longo da brotação e florescimento das videiras, o que pode diminuir o aproveitamento do nutriente pelas plantas. Os maiores fluxos e perdas de amônia do solo cultivado com videira para a atmosfera aconteceram nas doses mais elevadas de nitrogênio mineral aplicado, especialmente, depois de 44 horas da sua aplicação sobre a superfície do solo. A aplicação de ureia revestida com polímeros e composto orgânico promoveram as menores perdas de amônia por volatilização, o que pode estimular o aproveitamento de nitrogênio pelas videiras.
Nitrogen (N) applied in vineyards may be lost through volatilization or lixiviation. Two experiments were carried out to evaluate the N losses through volatilization or lixiviation in vineyard submitted the N fertilization, at Sandy Typic Hapludalf soil in Southern Brazil. In the experiment 1, the treatments were applications of 0, 40, 80 and 120kg N ha-1, as urea form, and analyze N concentration in soil solution. The soil solution was collected by lysimeters during the cycle of the grapevines. In the experiment 2, the treatments were 0, 20, 40, and 80kg N ha-1, as urea form, 40kg ha-1 N as composed organic form and 40kg N ha-1 as urea covered with polymer to evaluate ammonia volatilization until 80 hours after N fertilization. The largest concentration of mineral nitrogen were detected in the leach solution from treatments of higher doses of mineral fertilizer, along the budding and along the flowering of grapevines and this factors may decrease the nutrient efficiency. The largest flows and losses of ammonia to the atmosphere from a soil cultivated with grapevines happened at treatments with highest doses of mineral nitrogen. The highest lose happened 44 hours after fertilizer application on soil surface. The application of urea coated with polymers and organic compounds showed the smallest ammonia losses by volatilization, which may improve nitrogen efficiency by grapevines.