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@#RUNX1::RUNX1T1 is a core-binding factor driving fusion gene which arises from t(8;21)(q22;q22). It is one of the most common chromosomal rearrangements in both pediatric and adult Acute Myeloid Leukemia (AML) with a reported incidence o 15% in children and young adults. There are few case reports documenting RUNX1::RUNX1T1 translocation in pediatric AML. Although this is generally associated with a favorable prognosis, we report two (2) cases of de novo pediatric AML in the Philippines harboring a RUNX1::RUNX1T1 translocation, one eventually relapsed while the other attained remission but succumbed to sepsis.
Subject(s)
High-Throughput Nucleotide SequencingABSTRACT
There are many medications available to treat spasticity, but the tolerability of medications is the main issue for choosing the best treatment. The objectives of this study were to compare the efficacy and adverse effects of tolperisone compared to baclofen among patients with spasticity associated with spinal cord injury. Patients received baclofen plus physical therapy (BAF+PT, n=135) or tolperisone plus physical therapy (TOL+PT, n=116), or physical therapy alone (PT, n=180). The modified Ashworth scale score, the modified Medical Research Council score, the Barthel Index score, and the Disability Assessment scale score were improved (P<0.05 for all) in all the patients at the end of 6 weeks compared to before interventions. After 6 weeks, the overall coefficient of efficacy of the intervention(s) in the BAF+PT, TOL+PT, and PT groups were 1.15, 0.45, and 0.05, respectively. The patients of the BAF+PT group reported asthenia, drowsiness, and sleepiness and those of the TOL+PT group reported dyspepsia and epigastric pain as adverse effects. When comparing drug interventions to physical therapy alone, both baclofen plus physical therapy and tolperisone plus physical therapy played a significant role in the improvement of daily activities of patients. Nonetheless, baclofen plus physical therapy was tentatively effective. Tolperisone plus physical therapy was slightly effective. In addition, baclofen caused adverse effects related to the sedative manifestation (Level of Evidence: III; Technical Efficacy Stage: 4).
Subject(s)
Humans , Spinal Cord Injuries/complications , Tolperisone , Muscle Relaxants, Central/adverse effects , Baclofen/adverse effects , China , Retrospective StudiesABSTRACT
Introdução: As pessoas estão envelhecendo cada vez mais no Brasil e no mundo. Estudos apontam que o processo de envelhecimento populacional é acompanhado com o aumento de ocorrência de morbidade e incapacidade. Diferentes condições podem ser apresentadas pelo envelhecimento, como: diminuição da capacidade funcional, diminuição da força e da massa muscular, entre outros. Objetivo: Investigar a redução da capacidade funcional, secundária a redução de força muscular de membros inferiores em idosos institucionalizados em duas instituições de longa permanência (ILPI) no município de Nova Iguaçu (RJ/Brasil). Métodos: Trata-se de um estudo transversal, exploratório de caráter quantitativo em duas IAPI em Nova Iguaçu/RJ/ no período do mês de julho e agosto de 2017, em que foram selecionados 60 idosos de ambos os sexos (34 homens e 26 mulheres), com faixa etária ≥ 60 anos. Os voluntários foram avaliados quanto a capacidade funcional e a força muscular. Para a capacidade funcional, foi utilizado a escala de Barthel. Para o teste de força, o presente estudo usou para forma de avaliação a escala de Medical Research Council (MRC). Resultados: Os resultados mostraram que 61,7% dos idosos são classificados como independentes pelo índice de Barthel. Na escala de MRC, 95% dos idosos apresentaram fraqueza muscular. Conclusão: Concluímos que a maior proporção de idosos institucionalizados foram considerados independentes, porém, apresentaram quadro de fraqueza muscular. (AU)
Introduction: People are aging more and more in Brazil and worldwide. Studies show that the process of population aging is accompanied by increased occurrence of morbidity and disability. Different conditions can be presented by aging, such as: decreased functional capacity, decreased strength and muscle mass, among others. Objective: To investigate the reduction of functional capacity, secondary to the reduction of muscular strength of lower limbs in elderly institutionalized in two institutions in the municipality of Nova Iguaçu/RJ Brazil. Methods: This is a quantitative exploratory study in two elderly institutions at Nova Iguaçu during the period of July and August 2017, with 60 elderly men and women (34 males and 26 females), age range ≥ 60 years. The volunteers were assessed for functional capacity and muscle strength. For functional capacity, the Barthel scale was used. For strength testing, the present study used the Medical Research Council (MRC) scale for evaluation. Results: The results of the present study showed that 61.7% of the elderly are classified as independent by the Barthel index. In the MRC scale, 95% of the elderly presented muscular weakness. Conclusion: We concluded that the most proportion of institutionalized elderly was considered independent but presented a picture of muscular weakness. (AU)
Subject(s)
Humans , Male , Female , Personal Autonomy , Muscle Strength , Aging , Repertory, Barthel , Lower ExtremityABSTRACT
Objetivo: Correlacionar a sensação de dispneia obtida pela escala Medical Research Council modificada (mMRC) com as variáveis respiratórias e o tempo de internação em portadores de doença pulmonar obstrutiva crônica (DPOC) hospitalizados. Material e Métodos: Estudo transversal de caráter observacional e descritivo; no qual participaram do estudo pacientes interna-dos na Santa Casa de Misericórdia (SCM) de Goiânia-GO e Hospital Geral de Goiânia Dr. Alberto Rassi (HGG), com diag-nóstico de DPOC. Foram coletados sinais vitais, dados antro-pométricos e aplicada a escala de mMRC. Resultados: Foram avaliados 28 participantes, com média de idade de 74,10±12,46 anos; a média de mMRC foi de 3,10±1,19, comprometimento moderado, não ocorrendo diferença de mMRC entre homens e mulheres (p=0,503), além de não ter sido encontrada cor-relação entre o mMRC com a FR (r= -0,035 p=0,864), SpO2 (r=-0,228 p=0,222) e o tempo de internação (r=0,140 p=0,486). No entanto, em relação a necessidade de internação em uni-dade de terapia intensiva e o tempo de internação na unidade houve correlação significativa (r-0,457 p<0,01 e r 0,388 p<0,04, respectivamente). Conclusão: Não se encontrou relação da sensação de dispneia com as variáveis respiratórias e o tempo de internação total, porém foi possível verificar uma correlação entre o mMRC e a necessidade de internação e o tempo de internação em unidade de terapia intensiva. (AU)
Objetive:To correlate the dyspnea syndrome with the modified Medical Research Council scale (mMRC) with the respiratory and temporal variables of hospitalization in patients with hospitalized chronic obstructive pulmonary disease (COPD). Method: Cross-sectional observational and descriptive studyin which participated patients from the Santa Casa de Misericórdia (SCM) of Goiânia-GO and the General Hospital of Goiânia Dr. Alberto Rassi (HGG), with the diagnosis of COPD. Vital signs and anthropometric data were collected and the mMRC scale was applied. Results: Twenty-eight participants were evaluated, with a mean age of 74.10 ± 12.46 years; the mean mMRC was 3.10 ± 1.19, there was moderate impairment, no difference of mMRC between men and women (p = 0.503), nor was it found among mMRC with FR (r = -0.035 p = 0.864), SpO2 (r = -0.228 p = 0.222) and length of stay (r = 0.140 p = 0.486), which means that intensive care unit stay and length of stay in the domestic unit are important (r-0,457 p<0,01 e r 0,388 p<0,04 respectively). Conclusion: No differences between dyspnea syndrome and respiratory variables and total hospitalization time were found, but it was possible that they occurred between the MRC and the need for hospitalization and length of stay in intensive care therapy. There is a moderate influence of the dyspnea syndrome to direct the attention to the individuals under hospitalization, in order to minimize the progression of the disorder and greater impairment in the general state of health. (AU)
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Severity of Illness Index , Pulmonary Disease, Chronic Obstructive/physiopathology , Dyspnea/diagnosis , Symptom Assessment/methods , Length of Stay , Cross-Sectional Studies , Dyspnea/physiopathology , Intensive Care UnitsABSTRACT
Objective@#To investigate the impact of removing restraint on intensive care unit acquired weakness(ICU-AW), muscle strength and the incidence of extubation in patients with pancreatitis but normal cognitive function.@*Methods@#Eighty patients with pancreatitis but normal cognitive function hospitalized from February 2017 to November 2018 were divided into experimental group (40 cases) and control group (40 cases) by random digits table method. The control group received routine care and restraint, while the experimental group was removed from restraint compared with the control group. The Medical Research Council (MRC) score, incidence rate of ICU-AW, and extubation rate were compared between the two groups.@*Results@#The MRC scores of the experimental group before intervention, on 3rd, 7th and 10th day were (55.1±4.2), (54.2±4.6), (53.4±4.3), and (52.3±4.8) respectively. The MRC scores before the intervention, on the 3rd, 7th, and 10th day in the control group were (53.8±5.6), (52.0±6.2), (49.7±6.2), and (48.7±5.7) respectively. The MRC scores of the experimental group were significantly higher than those of the control group. The difference of MRC scores between the two groups was statistically significant on the 7th and 10th day(t=3.012, 3.060, P<0.05). The incidence of ICU-AW was 25.0%(10/40) in the experimental group and 37.5%(15/40) in the control group. The experimental group was lower than the control group, but the difference was not statistically significant (χ2=1.455, P>0.05). There was no unplanned extubation in both groups.@*Conclusions@#The removal of restraint in patients with pancreatitis but normal cognitive function can slow down the decline in muscle strength but has no positive impact on the incidence rate of ICU-AW.
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Background: Therapeutic plasma exchange (TPE) is the separation and removal of plasma from whole blood with replacement by a crystalloid/colloid solution (typically albumin or plasma). The DGHS has established guidelines and recommendations for application of therapeutic apheresis in clinical practice. Guillain-Barré syndrome (GBS) is considered category I indications for TPE. This study was undertaken to establish the effectiveness and safety of therapeutic plasma exchange in GBS which is one of the common indication for TPE at our tertiary care teaching hospital.Methods: A retrospective study of 30 patients admitted to a tertiary care teaching hospital, from January 2014 to December 2016 with clinical signs of Guillain-Barre syndrome (GBS) and/or GBS variants were evaluated for performing TPE. A total of 104 procedures were performed for 30 patients. Replacement of crystalloids and plasma was used. Medical Research Council scale was used to assess the clinical improvement by measuring the grade of muscle power. Information was collected in a structured proforma and statistical analysis was performed using SPSS software (version 20). P value less than 0.05 was considered statistically significant.Results: During the study period, 104 procedures were performed on 30 patients on an average of three procedures per patient. The average age of the patients was 41.4±10.4 years. The mean period of illness at admission was 14.5±5.4 (range 4-32) days. In 23 out of 30 patients, more than three TPE procedures were done, out of which 21 patients clinically improved. The common complications during the procedure were chills (16%), hypotension (10%) and non-hemolytic febrile transfusion reaction (10%) and they were managed accordingly. Two (6.7%) patients who were not ambulatory at discharge had significantly (p <0.05) lower grade of power in lower limbs at admission and all patients recovered fully on follow up.Conclusions: GBS is one of the most commonly occurring clinical paralytic disorders. 76.7% of patients underwent three or more cycles of TPE with 70% had excellent clinical improvement which was comparable with various other studies. Based on results published by various other studies, therapeutic plasma exchange is a comparatively safe and effective procedure
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Objective To evaluate the health status ,dyspnea and exercise endurance in the patients with chronic obstructive pul-monary disease(COPD) by the CAT scale ,modified medical research council(mMRC) dyspnea scale and 6-min walk test(6MWT) and to analyze the correlation between their evaluation results with the percentage (FEV1% pred) of the forced expiratory volume at 1 second(FEV1) in the predict value and whether complicating pulmonary hypertension (PH) .Methods 70 patients with COPD were performed the examinations of the lung function ,CAT scale ,mMRC score and 6MWT .The correlation among the various e-valuation systems and between each evaluation system with the severity of the pulmonary function airway limitation were compre -hensively evaluated .The CAT scores ,mMRC scores and 6 min walking distance(6MWD) were compared between the COPD com-plicating PH group and the simple COPD group .Results The CAT scale was negatively correlated with 6MWD(r= -0 .623 ,P0 .05) .The CAT scores had statistical difference between the COPD complicating PH group and the simple COPD group (P<0 .01) .6MWD had the highest accuracy for screening COPD whether complicating PH ,followed by CAT .The diagnostic cut-off point by adopting CAT for judging the risk of COPD complicating PH was 21 .Conclusion The CAT scale has good correlation with 6MWD and no correlation with FEV1% pred .The CAT scale is more suitable for the overall assessment of the total severity of COPD .Patients with the high CAT scores have higher risk of suffering from PH .
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OBJECTIVE: Evaluate muscle force and motor function in patients with Duchenne muscular dystrophy (DMD) in a period of six months. METHOD: Twenty children and adolescents with diagnosis of DMD were evaluated trough: measurement of the strength of the flexors and extensors of the shoulder, elbow, wrist, knee and ankle through the Medical Research Council (MRC), and application of the Motor Function Measure (MFM). The patients were evaluated twice within a six-month interval. RESULTS: Loss of muscle strength was identified in the MRC score for upper proximal members (t=-2.17, p=0.04). In the MFM, it was noted significant loss in the dimension 1 (t=-3.06, p=0.006). Moderate and strong correlations were found between the scores for muscular strength and the MFM dimensions. CONCLUSION: The MFM scale was a useful instrument in the follow up of patients with DMD. Moreover, it is a more comprehensive scale to assess patients and very good for conducting trials to evaluate treatment.
OBJETIVO: Avaliar a força muscular e função motora em pacientes com distrofia muscular de Duchenne (DMD) em intervalo de seis meses. MÉTODO: Vinte crianças e adolescentes com diagnóstico de DMD foram avaliados quanto às seguintes medidas: força de flexores e extensores de ombro, cotovelo, punho, quadril, joelho e tornozelo, por meio do Medical Research Council (MRC) e aplicação da Medida da Função Motora (MFM). Os pacientes foram analisados em dois momentos, com intervalo de seis meses. RESULTADOS: Observou-se perda de força muscular identificada por meio da MRC para membros superiores proximais (t=-2,17, p=0,04). Na MFM, observou-se perda significativa na dimensão 1 (t=-3,06, p=0,006). Foram registradas correlações médias a fortes entre os escores de força muscular e as dimensões da MFM. CONCLUSÃO: A escala MFM mostrou ser um instrumento útil no acompanhamento dos pacientes com DMD. É uma escala funcional, o que a torna boa candidata para o acompanhamento de pacientes com DMD em uso de novas drogas.
Subject(s)
Adolescent , Child , Humans , Young Adult , Disability Evaluation , Motor Activity/physiology , Muscle Strength/physiology , Muscular Dystrophy, Duchenne/physiopathology , Disease ProgressionABSTRACT
As doenças neuromusculares acometem a unidade motora, podendo comprometer os neurônios do corno anterior da medula, as raízes nervosas e os nervos periféricos, a junção neuromuscular ou o músculo. Podem ser de origem genética ou adquirida. Na infância predominam as de origem genética, sendo as mais frequentes a distrofia muscular tipo Duchenne e a amiotrofia espinhal progressiva. Vários métodos e escalas foram propostos para a avaliação e acompanhamento aos pacientes com doenças neuromusculares, tais como a escala de força manual, conhecida como medical research counsil (MRC), a goniometria e escalas funcionais. Entre as escalas funcionais, destaca-se a escala medida da função motora (MFM), uma vez que ela pode ser utilizada em qualquer doença neuromuscular,é de fácil aplicação e de baixo custo. O presente estudo é uma revisão bibliográfica não sistemática sobre as doenças neuromusculares mais comuns na infância e os instrumentos de medida úteis na avaliação dos pacientes portadores dessas doenças...
The neuromuscular diseases affect the motor unit, and may compromise the neurons of the anterior horn of the spinal cord, the nerves roots and the peripheral nerves, the neuro muscular junction or the muscle. They can have genetic or acquired origin. The genetic origin diseases predominate in childhood, and the most frequent are the Duchenne muscular dystrophy and the spinal muscular atrophy. Several methods and scales were proposed for the assessment and monitoring of patients with neuromuscular diseases, such as the manual strength know as medical research council (MRC), the goniometry and the functional scales. Among the functional scales the motor function measure (MFM) is noteworthy, asit can be used in any neuromuscular disease, is easily applicable and has low cost. This study is a bibliographic review on the most common neuromuscular diseases in childhood and the useful measuring instruments for the assessment of patients with these diseases...
Subject(s)
Humans , Male , Female , Child , Adolescent , Motor Activity , Neuromuscular Diseases , Neuromuscular Diseases/classification , Muscular Dystrophy, Duchenne , Weights and Measures , Muscular Disorders, AtrophicABSTRACT
OBJECTIVE: To compare muscle strength (MS) and motor function in patients with Duchenne muscular dystrophy (DMD) receiving steroids for different times against the natural evolution of DMD described by Scott et al. METHOD: 90 patients with DMD (aged 5- 12 years), receiving steroids for one to seven years, were evaluated by Medical Research Council Scale (MRC) and Hammersmith motor ability score. The relation between MS and motor abilities measurement from our data and Scott's ones were ascertained statistically. RESULTS: The relation between patient's age and Hammersmith scores revealed decrease of 0.76 point per year for age against decrease of 2.23 points on Scott's study. The relation between MRC scale and patient's age showed decrease of 0.80 point per year of age against decrease of 3.65 points on Scott's study. CONCLUSION: In patients with DMD aged five to 12 years the progression of the disease is delayed by steroids and the motor function is less reduced than muscular strength.
OBJETIVO: Comparar força muscular e função motora de pacientes com distrofia muscular de Duchenne (DMD) em corticoterapia com a evolução natural da doença descrita por Scott et al. MÉTODO: Noventa pacientes, entre 5 e 12 anos de idade, em corticoterapia por um até sete anos, foram avaliados quanto à força muscular (FM) (escala MRC) e função motora (Hammersmith motor ability score). A relação entre idade, FM e função motora e a comparação com o estudo de Scott et al foram determinadas estatisticamente. RESULTADOS: a relação idade/escore Hammersmith diminuiu 0,76 pontos a cada ano de aumento da idade (2,23 pontos na história natural). A relação idade/MRC decresceu 0,80 pontos a cada ano de aumento da idade (3,65 pontos na história natural). CONCLUSÃO: Nos pacientes em corticoterapia, a progressão da doença é mais lenta que na evolução natural em todas as faixas etárias avaliadas, sendo a FM mais comprometida que a função motora.
Subject(s)
Child , Child, Preschool , Female , Humans , Male , Glucocorticoids/therapeutic use , Motor Activity/physiology , Muscle Strength/physiology , Muscle, Skeletal/physiopathology , Muscular Dystrophy, Duchenne/physiopathology , Age Factors , Anti-Inflammatory Agents/therapeutic use , Disease Progression , Muscular Dystrophy, Duchenne/drug therapy , Prednisolone/therapeutic use , Pregnenediones/therapeutic useABSTRACT
Abstract: Public health is the societal approach to protecting and promoting health. Public health ethics can be defined as the identification, analysis, and resolution of ethical problems arising in public health practice and research. The emerging interest in ethical issues in public health research and practice reflects both the important societal role of public health and the growing public interest in the scientific integrity of health information and the equitable distribution of health care resources. This article provides an overview of ethical issues in public health research for young researchers and readers who do not necessarily have an in-depth knowledge of public health ethics. A framework of ethics analysis geared specifically for public health is needed to provide practical guidance for public health professionals and researchers in Bangladesh. Bangladesh Medical Research Council is playing a role in setting a standard in the field of biomedical research including public health concerning its strategy and ethical issues and by helping different health institutes to build up a research environment. Though public policy is based on many factors in addition to public health goals and ethical reasoning, it should not lead to the politically preferable option for a given time.
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Os objetivos deste trabalho foram: comparar a força muscular (FM) e as habilidades motoras de pacientes com Distrofia Muscular de Duchenne (DMD) em corticoterapia com a evolução natural da doença (Scott, 1982) e identificar a idade ideal de início da corticoterapia. Noventa pacientes com DMD em seguimento ambulatorial no Hospital das Clínicas da Faculdade de Medicina da USP, submetidos à corticoterapia (deflazacort ou prednisolona) por um período variável de um a sete anos, foram avaliados quanto à FM através da escala MRC e quanto às habilidades motoras através da escala Hammersmith motor ability score. Foram incluídos no estudo todos os pacientes com idade entre cinco e 12 anos, que compreendiam comandos verbais e que não haviam sido submetidos a cirurgias ortopédicas corretivas ou interrompido em algum momento a corticoterapia. A relação entre FM e habilidades motoras, a comparação dos dados com aqueles do estudo de Scott et al. e a análise da influência do tempo de tratamento, da idade de início e da idade na avaliação, sobre os valores obtidos nos testes foram submetidas a tratamento estatístico. Concluiu-se que: a progressão da perda da FM e das habilidades motoras em relação à idade foi mais lenta do que a da evolução natural em todas as faixas etárias avaliadas; quanto maior a idade maior a diferença entre os dois estudos; a perda da FM foi mais intensa do que a perda da funcionalidade; a metodologia utilizada não permitiu estabelecer com clareza a influência da idade de início do tratamento sobre os parâmetros avaliados, porém demonstrou a influência positiva do tempo de tratamento sobre a FM e habilidades motoras.
The aims of this study were: to compare muscle strength (MS) and motor abilities of Duchenne Muscular Dystrophy (DMD) patients in use of steroids, with the natural evolution of the disease (Scott, 1982) and to identify the ideal age for starting on steroid therapy. Ninety patients with DMD followed as outpatients at the Clinics Hospital of the University of São Paulo School of Medicine and submitted to steroid therapy (deflazacort or prednisolone) for a period of one to seven years were assessed for MS using the MRC scale, and for motor abilities with the motor ability score from the Hammersmith scale. All patients aged between five and 12 years who understood verbal commands and who had not been submitted to corrective orthopedic surgery and had no interruption in steroid therapy, were included in the study. Statistical analysis was carried out to assess the relationship between MS and motor abilities and to compare our data against results of Scott's study. The influence of length of treatment, age at disease onset and first assessment, on values obtained in the tests was investigated. We concluded that: the progression in loss of MS and of motor abilities with age was slower than the natural evolution across all age groups studied; the higher the age the greater the difference between the two studies; loss of MS was more intense than loss of functionality; the methodology used was unable to clearly ascertain the influence of age at treatment on the parameters assessed, but a positive influence of length of treatment on both MS and motor abilities was identified.