INTRODUÇÃO: Em pacientes com doença hepática avançada, pode ocorrer diminuição das pressões inspiratória máxima (PImáx) e expiratória máxima (PEmáx), respectivamente. Nos pacientes submetidos a transplante de fígado (Tx) essas alterações são agravadas no pós-operatório imediato. Para nosso conhecimento, a importância da medida pré-operatória da força da musculatura respiratória na evolução pós-operatória ainda não foi investigada no Tx. OBJETIVO: Avaliar o efeito das medidas pré-operatórias de força da musculatura respiratória no resultado do Tx. MÉTODO: Foram estudados retrospectivamente 228 pacientes de ambos os sexos, sem limite de idade, submetidos a primeiro Tx eletivo, com enxerto proveniente de doador cadáver. As medidas de PImáx e PEmáx foram obtidas imediatamente antes do transplante a partir do volume residual (VR) e da capacidade pulmonar total (CPT), respectivamente. Os pacientes foram classificados conforme a ocorrência de valores absolutos de pressão respiratória menores ou iguais a 50 cm H2O. As variáveis estudadas foram o tempo de ventilação mecânica pós-operatório, a necessidade de re-intubação orotraqueal ou de ventilação mecânica não-invasiva, o tempo de internação e a sobrevida dos pacientes. RESULTADO: Os resultados mostraram que os valores observados de PImáx e PEmáx estavam abaixo de 50 cm H2O em 19,7% (45/228) e 14,5% (33/228) dos pacientes, respectivamente. A freqüência de óbito até 6 meses após o transplante foi de 26/183 (14,2%) nos pacientes com PImáx > 50 cm H2O e de 15/45 (33,3%) nos pacientes com PImáx mais baixa (p=0,003). A sobrevida de 1, 3 e 5 anos foi 84%, 77% e 71% no grupo com PImáx > 50 cm H2O e 57%, 50% e 50% no grupo com PImáx mais baixa (p=0,0024). Em relação à PEmáx, essas probabilidades foram 80%, 74% e 69% no grupo com valores maiores que 50 cm H2O e 66%, 59% e 51% nos pacientes com força expiratória menor (p=0,1039). Não houve diferença estatisticamente significante em relação às demais variáveis analisadas. CONCLUSÃO: Pacientes com PImáx baixa apresentam maior mortalidade após o transplante de fígado. Entretanto, não foram encontrados efeitos estatisticamente significantes da medida pré-operatória da força da musculatura respiratória nas variáveis de resposta mais diretamente relacionadas com alterações respiratórias. / INTRODUCTION: Maximal inspiratory pressure (PImax) and maximal expiratory pressure (PEmax) were reduced in most patients with end-stage liver disease. In recipients of orthotopic liver transplantation (OLT), respiratory muscle weakness is worsened in the immediate postoperative period. In patients undergoing coronary artery bypass grafting, respiratory muscle weakness is associated with prolonged postsurgical mechanical ventilation and higher incidence of pulmonary complications. However, to our knowledge, no study has evaluated the effect of preoperative respiratory muscle strength on the postoperative course of OLT. AIM: To evaluate the effect of preoperative respiratory muscle strength on OLT outcome. METHODS: We reviewed 228 deceased donors elective OLT performed between 28th December, 1994 and 30th July, 2001. PImax e PEmax were assessed at residual volume and total lung capacity, respectively, immediately before OLT. Patients were classified according to the occurrence of muscle strength absolute values equal or lower than 50 cm H2O. The following response variables were analyzed: duration of postoperative mechanical ventilation, incidence of tracheal reintubation and noninvasive positive pressure ventilation, length of hospital stay and patient survival. RESULTS: PImax e PEmax were equal or lower than 50 cm H2O in 19.7% (45/228) and 14.5% (33/228) of patients, respectively. Patient mortality up to 6 months after OLT was 14.2% (26/183) in the group with PImax > 50 cm H2O and 33.3% (15/45) in the group with lower values (p=0.003). The 1-, 3-, and 5-year patient survival was 84%, 77% and 71% for the group with PImax > 50 cm H2O and 57%, 50% and 50% for the group with lower values (p=0.0024). In relation to PEmax, these probabilities were 80%, 74% e 69% for the group with higher values and 66%, 59% e 51% for patients with respiratory muscle weakness (p=0.1039). There is no significant difference regarding the others variables analyzed. CONCLUSION: Patients with low PImax present higher mortality after OLT. However, there are no statistically significant effects of the preoperative respiratory muscle strength on the response variables more directly related to the pulmonary outcome.

Introdução. O imobilismo durante a internação hospitalar pode levar a diversos efeitos deletérios, sendo a fraqueza muscular uma das principais alterações encontradas(1). Estudos mostram que pacientes críticos podem ter uma diminuição na força e na massa muscular com variações de 1 a 1,5% por dia e até 50% do total da massa muscular em duas semanas(2). Embora exista o conhecimento da pré-disposição a disfunções musculoesqueléticas durante a internação na Unidade de Terapia Intensiva (UTI), poucas são as evidências claras e consistentes que analisem o quanto que a força e a ativação muscular estão relacionadas com a perda funcional. Objetivo. Analisar a associação entre alterações musculares, perda funcional e variáveis clínicas de pacientes críticos após internação na Unidade de Terapia Intensiva através da avaliação da força, função e atividade muscular no momento da alta da UTI. Desenho do estudo. Estudo observacional, longitudinal. Métodos. O estudo incluiu pacientes de Unidades de terapia intensiva do Hospital das Clínicas de São Paulo e foi aprovado pelo Comitê de Ética e Pesquisa CAAE: 45060215.3.3001.0068. Foram incluídos no estudo pacientes com idade igual ou maior de 18 anos, internados diretamente na UTI desse hospital, não vindo de outros serviços, sem déficits neurológicos, sem contraindicações para a execução dos testes propostos e sem contraindicação para mobilização durante a estadia na UTI. Foram excluídos pacientes com menos de 4 dias de internação, que apresentarem edema no momento da avaliação de eletromiografia ou que durante a internação evoluíssem com alterações neurológicas. No momento da admissão foram colocados aceleromêtros nos indivíduos para o controle do nível de atividade física. No momento da alta da UTI foi realizada uma avaliação para a análise de força a ativação muscular através da Força de preensão palmar (FPP), medida pela dinamometria, e a Eletromiografia (EMG). Também foram realizados testes de campo, entre eles estão o teste de sentar e levantar de 30 segundos, teste de marcha estacionária de 2 minutos e Timed up and Go (TUG). Resultados. Foram analisados 107 pacientes com 53±15 anos, 51% do sexo feminino, 26% estiveram sob VMI, 20% apresentaram algum quadro séptico, com um tempo de internação na UTI de 10±8. Na análise de FPP obtivemos um valor médio de 20 kgf ±9, já para ativação muscular verificamos uma porcentagem de ativação de 30%±19 para o músculo vasto lateral. Na análise de associação verificamos valores significativos para ambos testes em relação a perda funcional na UTI, sendo que a diminuição da FPP aumentou 8% a chance de perda funcional (OD=1,084, IC 95% 1,025-1,147) e a menor ativação do vasto lateral 13% (OD=1,131, IC 95% 1,027 - 1,246). Os testes de campo mostraram associação com a FPP: o teste de sentar e levantar apresentou um R ajustado de 0,325 e um p < 0,001, já o teste de marcha estacionária teve um R ajustado 0,208 e p = 0,03. A FPP também teve associação com as variáveis clínicas: idade (R ajustado 0,22 e p = 0,031) e sepse (R ajustado 0,242 e p = 0,016). Já o músculo vasto lateral teve associação com a porcentagem de tempo em inatividade (R ajustado 0,235 e p = 0,01) e uso de VMI (R ajustado 0,2 e p = 0,03). Conclusão. Nossos resultados mostraram que a FPP e a ativação do músculo vasto lateral tem associação com o declínio funcional. Além disso a força muscular apresentou associação com o teste de sentar e levantar e de marcha estacionária. Percebemos que esses achados apresentaram também associação de variáveis como VMI, idade, sepse e porcentagem de tempo em inatividade. Portanto a avaliação dessas variáveis provou ser importante para auxiliar na proteção da perda funcional em pacientes de UTI / Introduction. Immobilism during hospitalization can lead to several deleterious effects, with muscle weakness being one of the main alterations found (1). Studies have shown that critical patients may have a decrease in strength and muscle mass ranging from 1 to 1.5% per day and up to 50% of total muscle mass in two weeks (2). Although there is knowledge of the pre-disposition to musculoskeletal disorders during hospitalization in the Intensive Care Unit (ICU), there are few clear and consistent evidences that analyze how much muscle strength and activation are related to functional loss. Goal. To analyze the association between muscle changes, functional loss and clinical variables of critically ill patients after admission to the Intensive Care Unit through the evaluation of muscle strength, function and activity at discharge. Study design. Observational, longitudinal study. Methods. The study included patients from Intensive Care Units of the Hospital das Clínicas of São Paulo and was approved by the Ethics and Research Committee CAAE: 45060215.3.3001.0068. The study included patients aged 18 years and over, admitted directly to the ICU of this hospital, not coming from other services, without neurological deficits, without contraindications to the proposed tests and without contraindication to mobilization during the ICU stay. Patients with less than 4 days of hospitalization were excluded, who presented edema at the time of electromyography evaluation or who, during hospitalization, progressed with neurological changes. At the time of admission, accelerometers were placed in the individuals to control the level of physical activity. At the time of ICU discharge, an evaluation was performed for the analysis of muscle activation through FPP, measured by dynamometry, and EMG. Field tests were also performed, among which are the sit-and-stand test of 30 seconds, 2-minute stationary gait test and 3-meter lift and walk test. Results. A total of 107 patients, 53 ± 15 years old, 51% female, 26% were under IMV, 20% presented septic, with an ICU length of 10 ± 8. In the FPP analysis we obtained a mean value of 20 kgf ± 9, whereas for muscle activation we verified a percentage of activation of 30% ± 19 for the vastus lateralis muscle. In the analysis of association, we found significant values for both tests in relation to the functional loss in the ICU. The FPP increased the chance of functional loss (OD = 1,084, 95% CI 1,025-1,147) and the vastus lateral activation 13% (OD = 1.131, 95% CI 1.027-1.246). Field tests showed association with FPP: the sit-up test had an adjusted R of 0.325 and a p < 0.001, while the stationary gait test had an adjusted R of 0.208 and p = 0.03. The FPP was also associated with clinical variables: age (adjusted R 0.22 and p = 0.031) and sepsis (R adjusted 0.242 and p = 0.016). The vastus lateralis muscle was associated with the percentage of time in inactivity (R adjusted 0.235 and p = 0.01) and VMI use (adjusted R 0.2 and p = 0.03). Conclusion. Our results showed that FPP and the activation of the vastus lateralis muscle are associated with functional decline. In addition, muscle strength was associated with sit-up and standing gait testing. We found that these findings also showed an association of variables such as VMI, age, sepsis and percentage of time in inactivity. Therefore, the evaluation of these variables proved to be important to assist in the protection of functional loss in ICU patients

Contextualização - A distrofia muscular de cinturas tipo 2A ou calpainopatia é uma desordem causada pelas mutações no gene CAPN3 (15q15.1) que codifica a calpaína. Entender como ocorre a perda de força muscular, da mobilidade articular e suas relações com a função nestes pacientes pode auxiliar na melhor compreensão da evolução da doença e indicar os biomarcadores pertinentes para o acompanhamento desta doença. Objetivo - Descrever e correlacionar a força muscular, a amplitude de movimentos articulares (ADM) e a função de um grupo de pacientes brasileiros com distrofia muscular de cinturas tipo 2A (calpainopatia). Método - Trata-se de um estudo transversal. A população estudada foi composta por 50 pacientes acompanhados no Centro de Pesquisa sobre o Genoma Humano e Células Tronco (CEGH-CEL) do Instituto de Biociências da Universidade de São Paulo, São Paulo, Brasil. Foram coletados os dados de força muscular (Medical Research Council - MRC), amplitude de movimento, escala de Vignos, escala de Medida de Independência Funcional (MIF), teste de caminhada de 10 metros (TC10m) e Escala Egen Klassifikation (EK). Para análise de correlação entre a força muscular e função foram utilizados os testes de correlação de Pearson e de Spearman. Resultados - Houve correlação positiva forte entre o índice de força muscular de cotovelo e o escore da Escala de Medida de independência funcional total (MIFT) (rho=0.70) e correlação negativa forte entre a Escala de Vignos e escore da MIFT (rho= -0.90). No teste de caminhada de 10 metros, a média do tempo utilizado pelos participantes foi de 17.82 segundos. Os resultados mostraram correlação negativa moderada entre o escore da escala EK e o índice de força muscular (MRC) dos segmentos corporais do cotovelo (rho= -0.51) e punho (r= -0.40) para o subgrupo cadeirantes. A amplitude de movimento não é um biomarcador pertinente para o acompanhamento da doença, pois não apresentou correlação com as demais variáveis deste estudo. A maioria dos pacientes apresentou limitações articulares na região de joelho e tornozelo, condizentes com outras pesquisas. A comparação da força muscular entre os músculos extensores dos joelhos direito e esquerdo mostrou diferença significativa (p < 0.02). Conclusão - Amostra brasileira apresentou incidência similar com os países do continente europeu (32%). Os escores de força muscular mostraram correlação com a função motora. Nossos achados permitem determinar, com melhor embasamento, os biomarcadores funcionais força muscular e função como os mais indicados na prática clínica e de pesquisa / Contextualization - Limb-girdle muscular dystrophy type 2A or calpainopathy is a disorder caused by mutation in the CAPN3 gene (15q15.1) that codes for calpain. Understanding how loss of muscular strength, joint mobility and their relationship with the function in these patients can help in better understanding the evolution of the disease and indicate the biomarkers pertinent to the follow-up of this disease. Objective - To describe and correlate muscle strength, range of joint movements (ROM) and function of a group of Brazilian patients with limb-girdle muscular dystrophy type 2A (calpainopathy). Method - This is a cross-sectional study. The studied population was composed for 50 patients at the Center for the Study of the Human Genome (CSHG) of the Institute of Biosciences of the University of São Paulo, São Paulo, Brazil. The data recorded were muscle strength (Medical Research Council - MRC), range of motion, Vignos scale, Functional Independence Measure (FIM), 10-meter walk test and Egen Klassifikation scale (EK). Para análise de correlação entre a força muscular e função foram utilizados os testes de correlação de Pearson e de Spearman. Results - There was a strong positive correlation between MRC and FIM (rho=0.70) and negative correlation between the Vignos Scale and FIM (rho= -0.90). The 10-meter walk test, the mean time used by participants was 17.82 seconds. The results showed a moderate negative correlation between the EK scale score and the MRC of the elbow (rho = -0.51) and wrist (r = -0.40) for the wheelchair subgroup. The range of motion is not a biomarker pertinent to the disease follow-up, since it did not present a correlation with the other variables of this study. The patients had contractures around the knee and ankle, is in keeping with what other studies. The comparison of muscle strength between the extensor muscles of the right and left knees showed a significant difference (p < 0.02). Conclusion - Brazilian sample had an incidence similar with the countries of the European continent (32%). The scores of muscle strength and motor function showed correlation. Our findings allow us to determine, with better foundation, functional biomarkers muscle strength and function as the most indicated in clinical practice and research

Qu’est ce qui explique la faiblesse d’un Etat qui s’est engagé depuis plus de vingt ans à rompre avec les pratiques de l’autoritarisme et du néo-patrimonialisme ? Pourquoi les promesses d’une démocratisation réussie, malgré le soutien massif des acteurs de la « société internationale » ont paradoxalement débouché sur la déstabilisation de l’Etat dans un contexte de crises récurrentes ? Ce sont les interrogations principales qui orientent cette recherche qui se donne pour ambition d’étudier les processus par lesquels les élites et les masses haïtiennes s’approprient la démocratie. De ce point de vue, la démocratisation s’analyse comme une ressource politique et institutionnelle revendiquée par ces dernières comme élément probant de leur adhésion à la grammaire démocratique. Dès lors, il s’agit de scruter les routes sinueuses et chaotiques empruntées par la démocratisation en Haïti, en interrogeant les diverses séquences marquantes de l’histoire politique haïtienne. L’accent est mis sur l’importation des technologies institutionnelles d’origine occidentale, notamment durant la période ouverte en 1994 avec la réinstallation de Jean-Bertrand Aristide dans ses fonctions de président et celle qui commence en 2004 avec son départ forcé du pouvoir. Ces deux séquences historiques, sous-tendues par des plans de reconstruction de l’Etat, sont marquées par la mobilisation de ressources diverses, en vue de jeter les bases d’une nouvelle gouvernance politique et économique. Elles circonscrivent également un champ de représentations conflictuelles, caractérisé par la compétition entre les Etats occidentaux et la lutte pour la répartition des enjeux de puissance entre les organisations internationales et les élites locales. L’analyse du processus de démocratisation conduit à des observations paradoxales : l’exacerbation de la violence, l’actualisation de l’autoritarisme, la multiplication des situations déviantes découlant des stratégies des acteurs locaux. Ces derniers tentent d’échapper aux contraintes imposées par des acteurs externes qui interviennent afin de sanctionner, d’arbitrer et d’imposer des décisions aux factions en luttes pour le pouvoir. Ainsi, la démocratisation passe par des crises d’adaptation, des phases contradictoires de recomposition, d’hybridation des institutions importées et parfois de contournement des normes et procédures. Entre les acteurs internes et externes s’établit et s’organise une dynamique interactive qui influence l’Etat dont la configuration finale est loin de correspondre aux objectifs affichés à travers les réformes engagées. Afin de mieux mettre en évidence les traits distinctifs de cet Etat forgé dans l’incertitude et l’imprécision, la thèse propose de restituer les dynamiques et l’historicité propres à la société haïtienne, en privilégiant l’hypothèse de la réinvention de l’Etat. Paradoxalement, l’État peut être le produit de processus de violence et la part chaotique que comporte la situation en Haïti peut recéler les prémices de sa construction. / What does explain the weakness of a State which has committed more than 20 years to break with the practices of authoritarianism and neo-patrimonialism? Why the promises of a successful democratization, despite the massive support of the actors of the “international society” paradoxically led to the destabilization of the State in the context of recurrent crises? These are the key questions guiding this research, whose ambition is to study the processes by which elites and the Haitian masses appropriate democracy. From this point of view, democratization is analyzed as a political and institutional resource claimed by them as convincing evidence of their adherence to democratic grammar. Therefore, it is scrutinizing the winding roads and chaotic way borrowed by democratization in Haiti, by querying various striking sequences of Haitian political history. Emphasis is placed on the importation of institutional technologies of Western origin, particularly during the period opened in 1994 with the resettlement of Jean-Bertrand Aristide in his duties as president and on the period which starts in 2004 with his forced departure from power. These two historical sequences, underpinned by plans for the reconstruction of the State, are marked by the mobilization of various resources, with a view to laying the foundations for new political and economic governance. They also define a field of conflicting representations, characterized by competition between Western States and the struggle for the distribution of power between the international organizations and local elites issues.The analysis of the process of democratization leads to paradoxical representations: the exacerbation of violence, the updating of authoritarianism, the multiplication of deviant situations arising from the strategies of local actors. The latter are trying to escape from the constraints imposed by external actors involved in order to punish, to arbitrate and to impose decisions on the factions struggling for power. Thus, democratization goes hand in hand with crises of adaptation, contradictory phases of re-composition, hybridization of the imported institutions and, sometimes with circumvention of the standards and procedures. Between internal and external actors are settled and organized interactive dynamics that influence the State of which the final configuration is far short of the objectives set out through the aimed reforms. In order to better highlight the distinguishing features of this State forged in uncertainty and vagueness, the thesis proposes to analyze the dynamics and historicity of Haitian society, privileging, the hypothesis of the reinvention of the State. Paradoxically, the State may be the product of process of violence and the chaotic part that includes the situation in Haiti can conceal the beginning of its construction.

Introdução. O imobilismo durante a internação hospitalar pode levar a diversos efeitos deletérios, sendo a fraqueza muscular uma das principais alterações encontradas(1). Estudos mostram que pacientes críticos podem ter uma diminuição na força e na massa muscular com variações de 1 a 1,5% por dia e até 50% do total da massa muscular em duas semanas(2). Embora exista o conhecimento da pré-disposição a disfunções musculoesqueléticas durante a internação na Unidade de Terapia Intensiva (UTI), poucas são as evidências claras e consistentes que analisem o quanto que a força e a ativação muscular estão relacionadas com a perda funcional. Objetivo. Analisar a associação entre alterações musculares, perda funcional e variáveis clínicas de pacientes críticos após internação na Unidade de Terapia Intensiva através da avaliação da força, função e atividade muscular no momento da alta da UTI. Desenho do estudo. Estudo observacional, longitudinal. Métodos. O estudo incluiu pacientes de Unidades de terapia intensiva do Hospital das Clínicas de São Paulo e foi aprovado pelo Comitê de Ética e Pesquisa CAAE: 45060215.3.3001.0068. Foram incluídos no estudo pacientes com idade igual ou maior de 18 anos, internados diretamente na UTI desse hospital, não vindo de outros serviços, sem déficits neurológicos, sem contraindicações para a execução dos testes propostos e sem contraindicação para mobilização durante a estadia na UTI. Foram excluídos pacientes com menos de 4 dias de internação, que apresentarem edema no momento da avaliação de eletromiografia ou que durante a internação evoluíssem com alterações neurológicas. No momento da admissão foram colocados aceleromêtros nos indivíduos para o controle do nível de atividade física. No momento da alta da UTI foi realizada uma avaliação para a análise de força a ativação muscular através da Força de preensão palmar (FPP), medida pela dinamometria, e a Eletromiografia (EMG). Também foram realizados testes de campo, entre eles estão o teste de sentar e levantar de 30 segundos, teste de marcha estacionária de 2 minutos e Timed up and Go (TUG). Resultados. Foram analisados 107 pacientes com 53±15 anos, 51% do sexo feminino, 26% estiveram sob VMI, 20% apresentaram algum quadro séptico, com um tempo de internação na UTI de 10±8. Na análise de FPP obtivemos um valor médio de 20 kgf ±9, já para ativação muscular verificamos uma porcentagem de ativação de 30%±19 para o músculo vasto lateral. Na análise de associação verificamos valores significativos para ambos testes em relação a perda funcional na UTI, sendo que a diminuição da FPP aumentou 8% a chance de perda funcional (OD=1,084, IC 95% 1,025-1,147) e a menor ativação do vasto lateral 13% (OD=1,131, IC 95% 1,027 - 1,246). Os testes de campo mostraram associação com a FPP: o teste de sentar e levantar apresentou um R ajustado de 0,325 e um p < 0,001, já o teste de marcha estacionária teve um R ajustado 0,208 e p = 0,03. A FPP também teve associação com as variáveis clínicas: idade (R ajustado 0,22 e p = 0,031) e sepse (R ajustado 0,242 e p = 0,016). Já o músculo vasto lateral teve associação com a porcentagem de tempo em inatividade (R ajustado 0,235 e p = 0,01) e uso de VMI (R ajustado 0,2 e p = 0,03). Conclusão. Nossos resultados mostraram que a FPP e a ativação do músculo vasto lateral tem associação com o declínio funcional. Além disso a força muscular apresentou associação com o teste de sentar e levantar e de marcha estacionária. Percebemos que esses achados apresentaram também associação de variáveis como VMI, idade, sepse e porcentagem de tempo em inatividade. Portanto a avaliação dessas variáveis provou ser importante para auxiliar na proteção da perda funcional em pacientes de UTI / Introduction. Immobilism during hospitalization can lead to several deleterious effects, with muscle weakness being one of the main alterations found (1). Studies have shown that critical patients may have a decrease in strength and muscle mass ranging from 1 to 1.5% per day and up to 50% of total muscle mass in two weeks (2). Although there is knowledge of the pre-disposition to musculoskeletal disorders during hospitalization in the Intensive Care Unit (ICU), there are few clear and consistent evidences that analyze how much muscle strength and activation are related to functional loss. Goal. To analyze the association between muscle changes, functional loss and clinical variables of critically ill patients after admission to the Intensive Care Unit through the evaluation of muscle strength, function and activity at discharge. Study design. Observational, longitudinal study. Methods. The study included patients from Intensive Care Units of the Hospital das Clínicas of São Paulo and was approved by the Ethics and Research Committee CAAE: 45060215.3.3001.0068. The study included patients aged 18 years and over, admitted directly to the ICU of this hospital, not coming from other services, without neurological deficits, without contraindications to the proposed tests and without contraindication to mobilization during the ICU stay. Patients with less than 4 days of hospitalization were excluded, who presented edema at the time of electromyography evaluation or who, during hospitalization, progressed with neurological changes. At the time of admission, accelerometers were placed in the individuals to control the level of physical activity. At the time of ICU discharge, an evaluation was performed for the analysis of muscle activation through FPP, measured by dynamometry, and EMG. Field tests were also performed, among which are the sit-and-stand test of 30 seconds, 2-minute stationary gait test and 3-meter lift and walk test. Results. A total of 107 patients, 53 ± 15 years old, 51% female, 26% were under IMV, 20% presented septic, with an ICU length of 10 ± 8. In the FPP analysis we obtained a mean value of 20 kgf ± 9, whereas for muscle activation we verified a percentage of activation of 30% ± 19 for the vastus lateralis muscle. In the analysis of association, we found significant values for both tests in relation to the functional loss in the ICU. The FPP increased the chance of functional loss (OD = 1,084, 95% CI 1,025-1,147) and the vastus lateral activation 13% (OD = 1.131, 95% CI 1.027-1.246). Field tests showed association with FPP: the sit-up test had an adjusted R of 0.325 and a p < 0.001, while the stationary gait test had an adjusted R of 0.208 and p = 0.03. The FPP was also associated with clinical variables: age (adjusted R 0.22 and p = 0.031) and sepsis (R adjusted 0.242 and p = 0.016). The vastus lateralis muscle was associated with the percentage of time in inactivity (R adjusted 0.235 and p = 0.01) and VMI use (adjusted R 0.2 and p = 0.03). Conclusion. Our results showed that FPP and the activation of the vastus lateralis muscle are associated with functional decline. In addition, muscle strength was associated with sit-up and standing gait testing. We found that these findings also showed an association of variables such as VMI, age, sepsis and percentage of time in inactivity. Therefore, the evaluation of these variables proved to be important to assist in the protection of functional loss in ICU patients

INTRODUÇÃO: Em pacientes com doença hepática avançada, pode ocorrer diminuição das pressões inspiratória máxima (PImáx) e expiratória máxima (PEmáx), respectivamente. Nos pacientes submetidos a transplante de fígado (Tx) essas alterações são agravadas no pós-operatório imediato. Para nosso conhecimento, a importância da medida pré-operatória da força da musculatura respiratória na evolução pós-operatória ainda não foi investigada no Tx. OBJETIVO: Avaliar o efeito das medidas pré-operatórias de força da musculatura respiratória no resultado do Tx. MÉTODO: Foram estudados retrospectivamente 228 pacientes de ambos os sexos, sem limite de idade, submetidos a primeiro Tx eletivo, com enxerto proveniente de doador cadáver. As medidas de PImáx e PEmáx foram obtidas imediatamente antes do transplante a partir do volume residual (VR) e da capacidade pulmonar total (CPT), respectivamente. Os pacientes foram classificados conforme a ocorrência de valores absolutos de pressão respiratória menores ou iguais a 50 cm H2O. As variáveis estudadas foram o tempo de ventilação mecânica pós-operatório, a necessidade de re-intubação orotraqueal ou de ventilação mecânica não-invasiva, o tempo de internação e a sobrevida dos pacientes. RESULTADO: Os resultados mostraram que os valores observados de PImáx e PEmáx estavam abaixo de 50 cm H2O em 19,7% (45/228) e 14,5% (33/228) dos pacientes, respectivamente. A freqüência de óbito até 6 meses após o transplante foi de 26/183 (14,2%) nos pacientes com PImáx > 50 cm H2O e de 15/45 (33,3%) nos pacientes com PImáx mais baixa (p=0,003). A sobrevida de 1, 3 e 5 anos foi 84%, 77% e 71% no grupo com PImáx > 50 cm H2O e 57%, 50% e 50% no grupo com PImáx mais baixa (p=0,0024). Em relação à PEmáx, essas probabilidades foram 80%, 74% e 69% no grupo com valores maiores que 50 cm H2O e 66%, 59% e 51% nos pacientes com força expiratória menor (p=0,1039). Não houve diferença estatisticamente significante em relação às demais variáveis analisadas. CONCLUSÃO: Pacientes com PImáx baixa apresentam maior mortalidade após o transplante de fígado. Entretanto, não foram encontrados efeitos estatisticamente significantes da medida pré-operatória da força da musculatura respiratória nas variáveis de resposta mais diretamente relacionadas com alterações respiratórias. / INTRODUCTION: Maximal inspiratory pressure (PImax) and maximal expiratory pressure (PEmax) were reduced in most patients with end-stage liver disease. In recipients of orthotopic liver transplantation (OLT), respiratory muscle weakness is worsened in the immediate postoperative period. In patients undergoing coronary artery bypass grafting, respiratory muscle weakness is associated with prolonged postsurgical mechanical ventilation and higher incidence of pulmonary complications. However, to our knowledge, no study has evaluated the effect of preoperative respiratory muscle strength on the postoperative course of OLT. AIM: To evaluate the effect of preoperative respiratory muscle strength on OLT outcome. METHODS: We reviewed 228 deceased donors elective OLT performed between 28th December, 1994 and 30th July, 2001. PImax e PEmax were assessed at residual volume and total lung capacity, respectively, immediately before OLT. Patients were classified according to the occurrence of muscle strength absolute values equal or lower than 50 cm H2O. The following response variables were analyzed: duration of postoperative mechanical ventilation, incidence of tracheal reintubation and noninvasive positive pressure ventilation, length of hospital stay and patient survival. RESULTS: PImax e PEmax were equal or lower than 50 cm H2O in 19.7% (45/228) and 14.5% (33/228) of patients, respectively. Patient mortality up to 6 months after OLT was 14.2% (26/183) in the group with PImax > 50 cm H2O and 33.3% (15/45) in the group with lower values (p=0.003). The 1-, 3-, and 5-year patient survival was 84%, 77% and 71% for the group with PImax > 50 cm H2O and 57%, 50% and 50% for the group with lower values (p=0.0024). In relation to PEmax, these probabilities were 80%, 74% e 69% for the group with higher values and 66%, 59% e 51% for patients with respiratory muscle weakness (p=0.1039). There is no significant difference regarding the others variables analyzed. CONCLUSION: Patients with low PImax present higher mortality after OLT. However, there are no statistically significant effects of the preoperative respiratory muscle strength on the response variables more directly related to the pulmonary outcome.

Introdução: As disferlinopatias são doenças genéticas causadas por alterações no gene da disferlina (DYSF), também denominadas distrofia muscular de cinturas (DMC) do tipo 2B, sendo a segunda em frequência em diversos países. A determinação de biomarcadores de função muscular desta doença se faz necessária. Objetivo: Estudo de caracterização da força e da função muscular nas disferlinopatias para estabelecer biomarcadores de habilidades motoras. Método: Amostra de 40 pacientes com dados de prontuário de força muscular (Medical Research Council - MRC), índice MRC, tempo de deambulação de 10 metros e, escalas de Vignos, Egen Klassifikation, Avaliação Funcional para distrofia muscular de Duchenne (FES-DMD) e North Star Ambulatory Assessment adaptada (a-NSAA). Resultados: Prevalência da disferlinopatia de 25,5% no Centro de Pesquisa sobre o Genoma Humano e Células Tronco, idade média de 36,5 anos, 52,5% do sexo masculino e 75% deambuladores. Músculos mais acometidos: abdominal, glúteo, íliopsoas, isquiotibial, quadríceps femoral, tibial anterior e deltoide médio. Correlação forte entre MRC e tempo de deambulação de 10 metros (média r=0,77) e, muito forte da MRC distal dos MMII com a-NSAA (r=0,90). Interação da MRC dos membros superiores (MMSS) e membros inferiores (MMII) entre os segmentos proximal e distal (p < 0,001), sendo mais evidente em MMSS do que em MMII. Taxa variável de progressão da doença, com 60% dos pacientes moderadamente ou gravemente afetados com menos de 12 anos de doença. Conclusão: Os achados caracterizam o padrão de fraqueza muscular dos brasileiros com disferlinopatia como proximal e distal dos MMII, com comprometimento associado da região proximal dos MMSS, além de elucidar as habilidades motoras em relação ao processo de locomoção. A forte correlação encontrada entre a força muscular, o tempo de deambulação de 10 metros e a escala a-NSAA, associada ao acompanhamento da evolução do desempenho de alguns grupos músculos podem fornecer um biomarcador adequado para o estudo da doençaCharacterization of muscle strength and function in Brazilian subjects with dysferlinopathy / Introduction: Dysferlinopathies are genetic diseases, caused to changes in the disferlina gene (DYSF), also named limb-girdle dystrophy type 2B, that is the second one in frequency in several countries. The small number of biomarkers of functional performance researches brings the need for studies in this area. Objective: This study characterizes muscle strength and function in subjects with dysferlinopathy to establish biomarkers of motor skills. Method: Data were available from 40 patients and included muscle strength assessment using the Medical Research Council (MRC) power scale, MRC index, timed motor performances for walking and data from the Vignos, Egen Klassifikation, Functional Assessment for Duchenne muscular dystrophy (FES-DMD) and the adapted North Star Ambulatory Assessment (a-NSAA) scales. Results: The prevalence of dysferlinopathy was 25.5% in the Centre for the Study of the Human Genome and Stem Cells (CEGH-CEL), the mean cohort age was 36.5 years, 52.5% were males and 75% were walkers. The weaker muscle found were the abdominal, gluteus, iliopsoas, hamstrings, quadriceps femoris, tibialis anterior and medial deltoid. Strong correlations were observed between the MRC power score and walking time (r = 0.77) and very strong between the MRC distal lower limb power score and a-NSAA (r = 0.90). Interactions of MRC scores were observed between the upper and lower limbs and the proximal and distal regions (p < 0.001) but were more evident in the upper limbs. The disease progression rates were variable with 60% of patients moderately or severely affected after more than 12 years since diagnosis. Conclusion: These findings suggest the pattern of muscular weakness in Brazilians with dysferlinopathy is predominantly in the lower limbs (proximal and distal) with associated involvement of the proximal upper limbs and elucidates the motor abilities in relation to locomotion. Due to the strong correlation with muscle strength, the walking time and the a-NSAA scale, in association with monitoring the evolution of the performance of some specific muscles can provide a suitable biomarker for the study of the disease

La myopathie centronucléaire autosomique dominante (MCN-AD) est une maladie congénitale rare liée à des mutations principalement retrouvées dans le gène dynamine-2. La majorité des patients atteints de MCN-AD présente une évolution lentement progressive, avec une perte de masse et de force musculaire. A ce jour, aucune thérapie n’est disponible pour la MCN-AD. Des interventions thérapeutiques visant à limiter la progression et la sévérité de l’atteinte musculaire ainsi qu’à améliorer la qualité de vie des patients, sont donc nécessaires. Nous faisons l’hypothèse qu’une hypertrophie induite par l’invalidation de la myostatine (mstn), régulateur négatif majeur de la masse musculaire, pourrait être bénéfique pour la souris modèle de cette pathologie (KI-Dnm2R465W/+), permettant notamment le maintien de la masse et de la force musculaire. Nous avons généré un modèle doublement muté résultant du croisement de souris KI-Dnm2R465W/+ myopathe avec des souris KO-mstn hypermusclées. Notre étude démontre que l'inactivation du gène mstn permet une amélioration de la masse et du volume musculaire, limite la perte de force et de motricité. Nos données suggèrent également que cette amélioration est majoritairement due à une diminution du niveau d’expression de certains acteurs impliqués dans le système catabolique ubiquitine-protéasome. De plus, nous montrons une accélèration de la diminution de la fréquence des anomalies histologiques caractéristiques de la myopathie chez les souris KI-Dnm2R465W/+. Nous proposons que ces anomalies pourraient être dues à une altération de la structure et/ou de la fonction mitochondriale. / Autosomal dominant centronuclear myopathy (AD-CNM) is a rare congenital muscle disease caused by mutations predominantly found in the dynamin 2 gene (DNM2). The clinical features generally reported are progressive muscle atrophy and weakness. To date, no treatment is available. The mouse model for AD-CNM harboring a mutation of the dynamin-2 gene (KI-Dnm2R465W/+) reproduces some of the human clinical features, notably muscle atrophy and weakness. Mstn, is a master negative regulator of skeletal muscle mass. We hypothesized that inactivation of mstn could limit muscle atrophy and weakness reported in the AD-CNM mouse model (KI-dnm2R465W/+). To test this hypothesis, we intercrossed KI-Dnm2R465W/+ mice with mice inactivated for mstn (KO-mstn) to generate a double mutated lineage (KIKO). The present study demonstrates that mstn gene inactivation allows for an improvement of muscle weight and volume, prevents muscle weakness and motor skill alterations. Our data also reveal that inactivation of mstn essentially downregulates some actors implicated in the catabolic ubiquitin-proteasome system. Furthermore, we show that inactivation of mstn decreases the frequency of of histological abnormalities characteristical in KI mice. We hypothesize that these abnormalities could be due to an alteration of mitochondrial function and network. The perspective to this work is to verify this hypothesis in the mouse model, which will contribute to a better understanding of the physiopathological mechanisms and can open new insight in the therapeutical approach to AD-CNM.

Deconditioning occurs in critically ill patients as early as 4 days after entering the intensive care unit (ICU) resulting in a loss of up to 25% peripheral muscle tone and 18% body weight by the time the patient is discharged. Early mobility (EM) has been shown to reduce complications such as neuromuscular weakness, muscle wasting, pneumonia, and the effects of prolonged periods of time on the ventilator. No formal education on EM had been provided to nurses at the clinical site. The purpose of this project was to develop an educational program on EM to promote early ambulation of critically ill ICU patients. The theory of knowledge to action was used to guide the development of the educational program. The practice-focused question addressed whether an educational program would improve nurses' perceptions of their knowledge of EM and if they would promote the use of EM among ICU patients. After a literature review to identify evidence-based practices and a protocol on EM, an educational program was developed that included a 25-item Likert-style pretest and posttest to measure percent agreement with perceptions of knowledge gained and likelihood of behavior change related to the practice of EM. Participants included 60 ICU nurses. Results demonstrated improvement in perceptions of knowledge of EM (from 74% before education to 88% after) and in likelihood of behavior change related to EM (from 69% before education to 91% after). Findings may be used to integrate EM into the ICU setting to reduce complications such as neuromuscular weakness, muscle wasting, and pneumonia. Results may also include improved patient outcomes, reduced length of stay, and increased quality of life for patients and their families, and thereby promote positive social change.

The Sarbanes-Oxley Act mandates public companies to establish internal control systems and assess their effectiveness. Quarterly reports by all companies and annual reports by companies with less than $75 million public float (non-accelerated filers) do not require auditor’s attestation while annual reports by companies with $75 million or more public float (accelerated filers) do require such auditor attestations. Quarterly reports should provide early warning of any impending material weakness (MW) to be disclosed in subsequent annual filings. This dissertation explores three types of “surprise” MW disclosures—positive, negative and no surprise—and consequences of such surprise disclosures. In part one, I document the frequency of surprise MW disclosures and internal control factors that are associated with each surprise type by filer status. Results show that 78 (77) percent of accelerated (non-accelerated) MW disclosures are negative surprise MW disclosures during 2004-2016. Entity level MWs are more associated with no-surprise rather than negative or positive surprise MW disclosures. In part two, I examine some consequences of surprise MW disclosures. The results show that companies with MW disclosures are more likely to dismiss their auditors and CFOs, and experience more shareholder voting against auditor ratification, compared to companies that issue clean reports. Auditor dismissal and CFO turnover are equally likely at negative and no-surprise MW disclosure companies. However, negative surprise accelerated filer companies’ shareholders are more likely to vote against auditor ratification than no-surprise accelerated filer companies. The third essay investigates the association between MW disclosures and audit fees. The results indicate that there is a significant positive association between audit fees and MW disclosures. Further, the results show that audit fees are higher at no-surprise companies than at negative surprise companies. The fourth essay focuses on audit report lag. The results indicate that MWs are associated with increased audit report lags, for both accelerated and non-accelerated filers. Further, surprise MW firms are more likely to experience increased audit report lag than no-surprise MW firms. Overall the results suggest that adverse internal control reports have consequences, and that the consequences vary between surprise and no-surprise MW firms. The results provide relevant empirical evidence to the ongoing debate on the necessity and efficacy of SOX Section 404 requirements.