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1.
Rev. Hosp. El Cruce ; (25): 1-9, Dic 2019.
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1047115

RESUMO

Las escoliosis graves plantean dificultades y complicaciones en su resolución, en particular cuando se presentan con bajo peso, comorbilidades, edad temprana y/o por causas neurológicas o congénitas. Las técnicas mini-invasivas con anclaje bipolar se ha propuesto en los últimos años como una alternativa de tratamiento. OBJETIVO: Presentar las características y resultados en estos pacientes operados en el Hospital El Cruce con técnica mini-invasiva de estabilización con anclaje bipolar. METODOLOGÍA: Se evaluaron pacientes operados por escoliosis con técnica bipolar modificada en el Hospital El Cruce. La misma consistió en abordaje cefálico y caudal de 2 a 3 vértebras, con instrumentación de las mismas y fijación a barras previamente deslizadas en forma intramuscular paravertebral uniendo los dos abordajes. RESULTADOS: Se evaluaron cuatro pacientes intervenidos con técnica bipolar. Edad 11,5 años, dos mujeres. Tres silla-dependientes (neuromusculares) y uno congénito. Peso 27,5 kg (19-40). Tres curvas sinistroconvexa. El Cobb pasó de 105º a 45º.Tiempo de cirugía 187 minutos. La fijación fue en todas tóracolumbar y en un caso incluyó la pelvis. La media de vértebras incluidas fue de 14.Una paciente presentó complicaciones mayores resueltas durante la internación. CONCLUSIONES:La técnica bipolar impresiona una alternativa válida en pacientes con escoliosis graves de bajo peso, temprana edad, comorbilidades y etiologías no idiopáticas.


Severe scoliosis raises difficulties and complications in its resolution, particularly when they present with low weight, comorbidities, early age and / or due to neurological or congenital causes. Minimally invasive techniques with bipolar anchoring have been proposed in recent years as an alternative treatment. OBJECTIVE: is to present the characteristics and results in patients that had surgery at El Cruce Hospital with a minimally invasive technique of stabilization with bipolar anchoring. METHODS:We evaluated patients operated on scoliosis with a modified bipolar technique at El Cruce Hospital.It consisted of a cephalic and a caudal approach of 2 to 3 vertebrae,with instrumentation and anchoring ofthose vertebrae to rods previously sliced in a paravertebral intramuscular form by joining the two approaches. RESULTS:Four patients were evaluated using bipolar technique. Average age 11.5, 2 women. Three chair-dependents (neuromuscular) and one congenital. Weight 27.5 kg (19-40). Three curves were left sided. The Cobb went from 105º to 45º. Surgery time 187 minutes), fixation was in all thoracolumbar and in one case included the pelvis. The mean included vertebrae were 14. A patient with major complications resolved during hospitalization. CONCLUSIONS: The bipolar technique seems to be a valid alternative in patients with severe low-weight scoliosis, early age, comorbidities and nonidiopathic etiologies.


Assuntos
Ortopedia , Escoliose , Cirurgia Geral , Procedimentos Cirúrgicos Operatórios , Doenças Neuromusculares
2.
Cochrane Database Syst Rev ; 9: CD011711, 2019 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-31487757

RESUMO

BACKGROUND: Neuromuscular diseases (NMDs) are a heterogeneous group of diseases affecting the anterior horn cell of spinal cord, neuromuscular junction, peripheral nerves and muscles. NMDs cause physical disability usually due to progressive loss of strength in limb muscles, and some NMDs also cause respiratory muscle weakness. Respiratory muscle training (RMT) might be expected to improve respiratory muscle weakness; however, the effects of RMT are still uncertain. This systematic review will synthesize the available trial evidence on the effectiveness and safety of RMT in people with NMD, to inform clinical practice. OBJECTIVES: To assess the effects of respiratory muscle training (RMT) for neuromuscular disease (NMD) in adults and children, in comparison to sham training, no training, standard treatment, breathing exercises, or other intensities or types of RMT. SEARCH METHODS: On 19 November 2018, we searched the Cochrane Neuromuscular Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, and Embase. On 23 December 2018, we searched the US National Institutes for Health Clinical Trials Registry (ClinicalTrials.gov), the World Health Organization International Clinical Trials Registry Platform, and reference lists of the included studies. SELECTION CRITERIA: We included randomized controlled trials (RCTs) and quasi-RCTs, including cross-over trials, of RMT in adults and children with a diagnosis of NMD of any degree of severity, who were living in the community, and who did not need mechanical ventilation. We compared trials of RMT (inspiratory muscle training (IMT) or expiratory muscle training (EMT), or both), with sham training, no training, standard treatment, different intensities of RMT, different types of RMT, or breathing exercises. DATA COLLECTION AND ANALYSIS: We followed standard Cochrane methodological procedures. MAIN RESULTS: We included 11 studies involving 250 randomized participants with NMDs: three trials (N = 88) in people with amyotrophic lateral sclerosis (ALS; motor neuron disease), six trials (N = 112) in Duchenne muscular dystrophy (DMD), one trial (N = 23) in people with Becker muscular dystrophy (BMD) or limb-girdle muscular dystrophy, and one trial (N = 27) in people with myasthenia gravis.Nine of the trials were at high risk of bias in at least one domain and many reported insufficient information for accurate assessment of the risk of bias. Populations, interventions, control interventions, and outcome measures were often different, which largely ruled out meta-analysis. All included studies assessed lung capacity, our primary outcome, but four did not provide data for analysis (1 in people with ALS and three cross-over studies in DMD). None provided long-term data (over a year) and only one trial, in ALS, provided information on adverse events. Unscheduled hospitalisations for chest infection or acute exacerbation of chronic respiratory failure were not reported and physical function and quality of life were reported in one (ALS) trial.Amyotrophic lateral sclerosis (ALS)Three trials compared RMT versus sham training in ALS. Short-term (8 weeks) effects of RMT on lung capacity in ALS showed no clear difference in the change of the per cent predicted forced vital capacity (FVC%) between EMT and sham EMT groups (mean difference (MD) 0.70, 95% confidence interval (CI) -8.48 to 9.88; N = 46; low-certainty evidence). The mean difference (MD) in FVC% after four months' treatment was 10.86% in favour of IMT (95% CI -4.25 to 25.97; 1 trial, N = 24; low-certainty evidence), which is larger than the minimal clinically important difference (MCID, as estimated in people with idiopathic pulmonary fibrosis). There was no clear difference between IMT and sham IMT groups, measured on the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALFRS; range of possible scores 0 = best to 40 = worst) (MD 0.85, 95% CI -2.16 to 3.85; 1 trial, N = 24; low-certainty evidence) or quality of life, measured on the EuroQol-5D (0 = worst to 100 = best) (MD 0.77, 95% CI -17.09 to 18.62; 1 trial, N = 24; low-certainty evidence) over the medium term (4 months). One trial report stated that the IMT protocol had no adverse effect (very low-certainty evidence).Duchenne muscular dystrophy (DMD)Two DMD trials compared RMT versus sham training in young males with DMD. In one study, the mean post-intervention (6-week) total lung capacity (TLC) favoured RMT (MD 0.45 L, 95% CI -0.24 to 1.14; 1 trial, N = 16; low-certainty evidence). In the other trial there was no clear difference in post-intervention (18 days) FVC between RMT and sham RMT (MD 0.16 L, 95% CI -0.31 to 0.63; 1 trial, N = 20; low-certainty evidence). One RCT and three cross-over trials compared a form of RMT with no training in males with DMD; the cross-over trials did not provide suitable data. Post-intervention (6-month) values showed no clear difference between the RMT and no training groups in per cent predicted vital capacity (VC%) (MD 3.50, 95% CI -14.35 to 21.35; 1 trial, N = 30; low-certainty evidence).Becker or limb-girdle muscular dystrophyOne RCT (N = 21) compared 12 weeks of IMT with breathing exercises in people with Becker or limb-girdle muscular dystrophy. The evidence was of very low certainty and conclusions could not be drawn.Myasthenia gravisIn myasthenia gravis, there may be no clear difference between RMT and breathing exercises on measures of lung capacity, in the short term (TLC MD -0.20 L, 95% CI -1.07 to 0.67; 1 trial, N = 27; low-certainty evidence). Effects of RMT on quality of life are uncertain (1 trial; N = 27).Some trials reported effects of RMT on inspiratory and/or expiratory muscle strength; this evidence was also of low or very low certainty. AUTHORS' CONCLUSIONS: RMT may improve lung capacity and respiratory muscle strength in some NMDs. In ALS there may not be any clinically meaningful effect of RMT on physical functioning or quality of life and it is uncertain whether it causes adverse effects. Due to clinical heterogeneity between the trials and the small number of participants included in the analysis, together with the risk of bias, these results must be interpreted very cautiously.


Assuntos
Exercícios Respiratórios/métodos , Doenças Neuromusculares/reabilitação , Adulto , Criança , Expiração/fisiologia , Humanos , Debilidade Muscular , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto , Capacidade Vital
3.
Rev. neurol. (Ed. impr.) ; 69(3): 113-122, 1 ago., 2019. tab
Artigo em Espanhol | IBECS | ID: ibc-184024

RESUMO

Introducción. El dengue es una infección arboviral causada por el virus del dengue. Se revisan las complicaciones neurológicas asociadas a dicha infección. Desarrollo. El carácter neurotropo del virus del dengue se ha confirmado en estudios epidemiológicos, series de casos y estudios histopatológicos. El rango de complicaciones neurológicas es del 5,6-14,6%, y son más frecuentes en los serotipos 1 y 3. La encefalopatía es el síndrome neurológico más común (0,5-6%); su prevalencia es mayor en los niños y los adolescentes. La detección del antígeno viral en el tejido cerebral y la presencia de pleocitosis o ARN en el líquido cefalorraquídeo son evidencia del carácter neurotropo del virus del dengue, que se manifiesta en forma de encefalitis. Los síndromes neurológicos durante la fase de convalecencia (encefalomielitis aguda diseminada, cerebelitis, opsoclonía-mioclonía, mononeuritis, polirradiculoneuritis y plexitis) parecen ser inmunomediados. La mielitis puede suceder durante la infección aguda por el virus del dengue y por un mecanismo inmunomediado en la fase de convalecencia. Mialgias, miositis, rabdomiólisis y parálisis hipopotasémica son ejemplos de disfunción muscular asociada al virus del dengue. La incidencia de ictus es del 0,26%, y puede ser isquémico o hemorrágico. Las complicaciones oftalmológicas incluyen maculopatía, hemorragia retiniana, neuropatía óptica y vitritis. Conclusiones. El espectro de complicaciones neurológicas por el virus del dengue es amplio. No existen datos fiables sobre su incidencia real porque la mayor parte de los estudios publicados son series o casos aislados


Introduction. Dengue is an arboviral infection caused by the dengue virus. The neurological complications associated with this infection are reviewed. Development. The neurotropic nature of dengue virus has been confirmed in epidemiological studies, case series and histopathological studies. The range of neurological complications is 5.6-14.6%, and they are more frequent in serotypes 1 and 3. Encephalopathy is the most common neurological syndrome (0.5-6%) and its prevalence is higher in children and adolescents. The detection of the viral antigen in brain tissue and the presence of pleocytosis or RNA in cerebrospinal fluid are evidence of the neurotropic nature of dengue virus, which manifests itself in the form of encephalitis. Neurological syndromes during convalescence (disseminated acute cerebellitis, opsoclonus-myoclonus syndrome, mononeuritis, polyradiculoneuritis and plexitis) appear to be immunomediated. Myelitis can occur during acute dengue virus infection and through an immunomediated mechanism in the convalescence phase. Myalgias, myositis, rhabdomyolysis and hypokalemic paralysis are examples of muscular dysfunction associated with the dengue virus. The incidence of stroke is 0.26%, and may be ischaemic or haemorrhagic. Ophthalmological complications include maculopathy, retinal haemorrhage, optic neuropathy and vitritis. Conclusions. The spectrum of neurological complications from dengue virus is broad. There are no reliable data on its real incidence because most of the studies published to date are isolated series or cases


Assuntos
Humanos , Dengue/complicações , Doenças do Sistema Nervoso Central/virologia , Doenças Neuromusculares/virologia , Doenças Vasculares/virologia
4.
Life Sci ; 233: 116684, 2019 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-31351083

RESUMO

Traumatic brain injury (TBI) is a devastating condition that often triggers a sequel of neurological disorders that can last throughout lifespan. From a metabolic viewpoint, the compromising of the energy metabolism of the brain has produced evidence linking the severity of brain injury to the extent of disturbances in the cerebral metabolism. The cerebral metabolic crisis, however, displays that regional heterogeneity varies temporally post-injury. It is important to note that energy generation and mitochondrial function are closely related and interconnected with delayed secondary manifestations of brain injury, including early neuromotor dysfunction, cognitive impairment, and post-traumatic epilepsy (PTE). Given the extent of post-traumatic changes in neuronal function and the possibility of amplifying secondary cascades, different therapies designed to minimize damage and retain/restore cellular function after TBI are currently being studied. One of the possible strategies may be the inclusion of ergogenic compounds, which is a class of supplements that typically includes ingredients used by athletes to enhance their performance. The combination of these compounds offers specific physiological advantages, which include enhanced energy availability/metabolism and improved buffering capacity. However, the literature on their effects in certain biological systems and neurological diseases, such as TBI, has yet to be determined. Thus, the present review aims to discuss the role of ergogenic compounds popularly used in secondary damage induced by this neurological injury. In this narrative review, we also discuss how the results from animal studies can be applied to TBI clinical settings.


Assuntos
Lesões Encefálicas Traumáticas/complicações , Disfunção Cognitiva/tratamento farmacológico , Epilepsia Pós-Traumática/tratamento farmacológico , Mitocôndrias/efeitos dos fármacos , Doenças Neuromusculares/tratamento farmacológico , Animais , Arginina/farmacologia , Cafeína/farmacologia , Carnitina/farmacologia , Estimulantes do Sistema Nervoso Central/farmacologia , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/fisiopatologia , Creatina/farmacologia , Metabolismo Energético , Epilepsia Pós-Traumática/etiologia , Epilepsia Pós-Traumática/fisiopatologia , Glutamina/farmacologia , Humanos , Mitocôndrias/metabolismo , Mitocôndrias/patologia , Doenças Neuromusculares/etiologia , Doenças Neuromusculares/fisiopatologia , Taurina/farmacologia
5.
Nervenarzt ; 90(8): 809-816, 2019 Aug.
Artigo em Alemão | MEDLINE | ID: mdl-31286145

RESUMO

BACKGROUND: For a long time the treatment of neuromuscular diseases was considered to be purely symptomatic. Due to new technologies in recent years novel causal forms of treatment could be developed. Gene therapies for spinal muscular atrophy, Duchenne muscular dystrophy, limb-girdle muscular dystrophy, myotubular myopathy and hereditary motor and sensory neuropathy type 1A are currently being evaluated in clinical trials. Initial preliminary results are promising and the first preparation onasemnogene abeparvovec-xioi (Zolgensma®) for the treatment of spinal muscular atrophy has recently been approved by the U.S. Food and Drug Administration (FDA). OBJECTIVE: This review describes the principles of gene therapy, summarizes the interim results published so far and provides an overview of currently active or soon to be initiated gene therapy trials. CONCLUSION: Gene therapies have the potential to significantly influence the course of neuromuscular diseases. First positive intermediate results have been published and the first treatment has recently been approved in the USA. Long-term data on sustained effects and toxicity of gene therapies are not yet available. These novel treatment options will present new challenges for the healthcare systems concerning diagnosis, treatment and reimbursement.


Assuntos
Terapia Genética , Doenças Neuromusculares , Terapia Genética/tendências , Humanos , Doenças Neuromusculares/terapia
6.
MMWR Morb Mortal Wkly Rep ; 68(27): 608-614, 2019 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-31295232

RESUMO

BACKGROUND: Acute flaccid myelitis (AFM), a serious paralytic illness, was first recognized as a distinct condition in 2014, when cases were reported concurrent with a large U.S. outbreak of severe respiratory illness caused by enterovirus D-68 (EV-D68). Since 2014, nationwide outbreaks of AFM have occurred every 2 years in the United States; the cause for the recent change in the epidemiology of AFM in the United States, including the occurrence of outbreaks and a biennial periodicity since 2014, is under investigation. This report updates clinical, laboratory, and outcome data for cases reported to CDC during 2018. METHODS: Clinical data and specimens from persons in the United States who met the clinical criterion for AFM (acute onset of flaccid limb weakness) with onset in 2018 were submitted to CDC for classification of the illnesses as confirmed, probable, or non-AFM cases. Enterovirus/rhinovirus (EV/RV) testing was performed on available specimens from persons meeting the clinical criterion. Descriptive analyses, laboratory results, and indicators of early recognition and reporting are summarized. RESULTS: From January through December 2018, among 374 reported cases of AFM, 233 (62%) (from 41 states) were classified as confirmed, 26 (7%) as probable, and 115 (31%) as non-AFM cases. Median ages of patients with confirmed, probable, and non-AFM cases were 5.3, 2.9, and 8.8 years, respectively. Laboratory testing identified multiple EV/RV types, primarily in respiratory and stool specimens, in 44% of confirmed cases. Among confirmed cases, the interval from onset of limb weakness until specimen collection ranged from 2 to 7 days, depending on specimen type. Interval from onset of limb weakness until reporting to CDC during 2018 ranged from 18 to 36 days, with confirmed and probable cases reported earlier than non-AFM cases. CONCLUSION: Identification of risk factors leading to outbreaks of AFM remains a public health priority. Prompt recognition of signs and symptoms, early specimen collection, and complete and rapid reporting will expedite public health investigations and research studies to elucidate the recent epidemiology of AFM and subsequently inform treatment and prevention recommendations.


Assuntos
Viroses do Sistema Nervoso Central/epidemiologia , Surtos de Doenças/estatística & dados numéricos , Mielite/epidemiologia , Doenças Neuromusculares/epidemiologia , Vigilância da População , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Estações do Ano , Estados Unidos/epidemiologia , Adulto Jovem
7.
Expert Rev Clin Pharmacol ; 12(8): 757-770, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31220956

RESUMO

Introduction: Genetic neuromuscular diseases (NMDs) constitute a heterogeneous group of rare conditions, including some of the most disabling conditions in childhood. Recently, advanced technologies have greatly expanded preclinical and clinical research, and specific therapies have been developed. Area covered: We provide an overview of novel pharmacological approaches to the main NMDs, including Duchenne muscular dystrophy (DMD), spina muscular atrophy (SMA), X-linked myotubular myopathy, Pompe disease (PD), and myotonic dystrophy type 1, with attention to both achievements and unresolved therapeutic challenges. We conducted a selected review of relevant publications in the last five years identified through PubMed and Scopus. Additional information was derived from the website of clinicaltrials.gov and from the authors' direct knowledge of research activities. Expert Opinion: For the first time, targeted therapies have received conditional regulatory approval and have been introduced into clinical care: enzyme replacement therapy for PD, gene expression modulation for DMD and SMA, and gene therapy for SMA. Though not curative, these treatments can improve functioning and increase survival. Issues still to be addressed include: early recognition, definition of new emerging phenotypes, development of more sensitive outcome measures, long-term risk-benefit estimates, high costs sustainability, and criteria for therapy initiation and discontinuation.


Assuntos
Terapia Genética/métodos , Terapia de Alvo Molecular , Doenças Neuromusculares/terapia , Animais , Criança , Desenho de Drogas , Humanos , Doenças Neuromusculares/genética , Doenças Neuromusculares/fisiopatologia
8.
Medicine (Baltimore) ; 98(25): e15983, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31232929

RESUMO

Knowledge of the patient's own perception of functioning and dependence, and of environmental factors, is of significant value. The main goals of this study are (1) to obtain a general profile of the occupational performance of persons with neuromuscular disorders (NMD) and their needs related to that performance and (2) to determine the support resources (assistive technologies [AT], adjustment, and/or caregiver) required to improve or maintain their level of independence.This cross-sectional study involved 24 persons with NMD. The functional independence measure (FIM), the checklist of a home's accessibility level, and a specific questionnaire were administered.The sample included 14 women (58.3%) and 10 men (41.7%). A mean of 61.7 (standard deviation = 17.2) was obtained for FIM motor, over 91, indicating a moderate level of dependence. The AT most frequently used was a wheelchair (70.8%). Architectural barriers were detected in the majority of users' homes (87.5%).Concise assessment of the independence needs of people with NMD, according to their occupational performance, and prescription of resources to meet those needs are required. This procedure should be implemented in healthcare programs, including care to a caregiver.


Assuntos
Atividades Cotidianas , Pessoas com Deficiência/reabilitação , Doenças Neuromusculares/reabilitação , Equipamentos de Autoajuda , Adolescente , Adulto , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Terapia Ocupacional , Inquéritos e Questionários , Adulto Jovem
9.
J Rehabil Med ; 51(7): 518-524, 2019 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-31243469

RESUMO

OBJECTIVE: To evaluate the current application of aerobic exercise in adult neuromuscular rehabilitation. DESIGN: Cross-sectional survey. PARTICIPANTS: Dutch rehabilitation specialists and physical therapists in specialized centres for slowly progressive neuromuscular diseases and in primary care. METHODS: Participants received a self-designed, web-based, questionnaire, including 27 close-ended questions covering 4 categories: respondent profile, application of aerobic exercise, barri-ers to prescribing aerobic exercise, and need for support to improve the application of aerobic exercise. RESULTS: All respondents (n = 52) prescribed aerobic exercise and in a wide variety of neuromuscular diseases, mostly applying sessions of more than 20 min, 2 days per week, over a period of 9-16 weeks, using different exercise modes and methods to target intensity. The majo-rity (81%) agreed that aerobic exercise should be incorporated into neuromuscular rehabilitation. However, all respon-dents perceived barriers to the application of aerobic exercise in one or more domains, and 77% of the respondents indicated needing support to improve application of this type of training, mostly with respect to screening procedures (54%) and dosing of exercise programmes (48%). CONCLUSION: Aerobic exercise is widely applied, yet our results raise awareness of the necessity of more evidence based knowledge, in order to develop and implement guidelines in adult neuromuscular rehabilitation.


Assuntos
Terapia por Exercício/métodos , Exercício/fisiologia , Pessoal de Saúde/tendências , Doenças Neuromusculares/reabilitação , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Inquéritos e Questionários
10.
Neurol Neurochir Pol ; 53(3): 173-180, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31180130

RESUMO

There is an ever-growing need for molecular biomarkers in assessing clinical course and diagnosing neuromuscular disorders, as well as in monitoring drug therapy. With the development of high throughput techniques, there has been an acceleration in the discovery of potential biomarkers. It is quite easy to find potential candidates, but difficult to validate them and translate into a clinical setting. Neuromuscular diseases (NMD) are a major challenge in terms of finding potential molecular biomarkers, mainly because of their heterogeneous aetiology and variability in phenotype, their as yet incompletely understood pathophysiology, and their slow clinical progression. Furthermore, it is challenging to assemble a large cohort of patients, as many NMDs are rare diseases. In this literature review, we provide an update on the latest discoveries in DNA, RNA, miRNA, epigenetic, protein, metabolic and cellular biomarkers for NMD. The advantages and potential difficulties of clinical application and the role of identification of biomarker panels are discussed. We have especially sought to highlight translational biomarkers which can be easily transferred to the clinic, where they may eventually present possible future therapies related to molecular biomarker discoveries.


Assuntos
Doenças Neuromusculares , Biomarcadores , Humanos
11.
Cien Saude Colet ; 24(5): 1609-1616, 2019 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-31166496

RESUMO

This study targets the development of a nursing clinical data model for neuromuscular processes. To achieve this purpose, content analysis based on Bardin's perspective was performed on the Portuguese nursing local customizations regarding neuromuscular processes, with the International Classification for Nursing Practice concepts and the ISO 18104:2014 used as encoding rules. From analysis of the data, a total of 1766 diagnoses were related to neuromuscular processes. After application of exclusion criteria, a corpus with a total of 900 diagnoses was subjected to content analysis. After application of the encoding rules, a total of 81 context units were obtained, and through an inductive approach, were defined into three categories: clinical findings (e.g. aphasia); negative judgment diagnoses (e.g. impaired communication); transition properties (e.g. preparation and knowledge). These interpretations were validated by experts in the field. This study not only demonstrates the need to standardize data, but also the importance of neuromuscular processes in nursing practice. We hope this study will guide the definition of a nursing clinical data model that will help in increasing complexity in the level of care provided with high impact in the patient's quality of life.


Assuntos
Modelos de Enfermagem , Doenças Neuromusculares/diagnóstico , Diagnóstico de Enfermagem , Qualidade de Vida , Humanos , Portugal , Terminologia Padronizada em Enfermagem
12.
Recurso na Internet em Inglês | LIS - Localizador de Informação em Saúde, LIS-bvsms | ID: lis-LISBR1.1-46532

RESUMO

Website dedicated to transform the lives of people living with muscular dystrophy, ALS and related neuromuscular diseases.


Assuntos
Esclerose Amiotrófica Lateral , Distrofias Musculares , Doenças Neuromusculares , Doenças Raras
14.
Neurologist ; 24(3): 75-83, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045716

RESUMO

OBJECTIVES: The objective of this study was to assess risk of neurological toxicities following the use of different immune checkpoint inhibitor (ICI) regimens in solid tumors. METHODS: Pubmed, Embase, and ClinicalTrials.gov databases were searched for publications, and data were analyzed using Review Manager 5.3 software to compare the risk of immune-related and nonspecific neurological complications potentially triggered by ICIs to controls. RESULTS: In total 23 randomized clinical trials comprising 11,687 patients were included in this meta-analysis. Patients with PD-L1 (OR, 0.29; 95% confidence interval [CI], 0.18-0.48; P<0.01) or programmed cell-death protein 1 (PD-1) inhibitor (OR, 0.21; 95% CI, 0.14-0.31; P<0.01) were less likely to develop any-grade peripheral neuropathy than chemotherapy, while the risk of grade 3-5 was also smaller for PD-1 inhibitor (OR, 0.16; 95% CI, 0.05-0.54; P=0.003). Combination therapy with CTLA4 and PD-1 inhibitor did not significantly increase the risk of any-grade (OR, 0.83; 95% CI, 0.21-3.32; P>0.05) or grade 3-5 (OR, 1.4; 95% CI, 0.2-9.61; P>0.05) peripheral neuropathy compared to monotherapy with CTLA4 or PD-1 inhibitor. However, difference in risk of immune-related adverse events (irAEs) involving central nervous system did not reach statistical significance in patients with different ICI regimens compared those under chemotherapy. Additionally, risk of experiencing paresthesia was in line with that of peripheral neuropathy (OR, 0.42; 95% CI, 0.28-0.62; P<0.01). CONCLUSIONS: This meta-analysis shows that PD-L1/PD-1 and CTLA4 inhibitor have decreased risk of peripheral neuropathy compared to chemotherapy, while combination therapy with CTLA4 and PD-1 inhibitor have no difference in neurological toxicities compared to monotherapy with CTLA4 or PD-1 inhibitor.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Neoplasias/tratamento farmacológico , Síndromes Neurotóxicas/epidemiologia , Antígeno B7-H1/antagonistas & inibidores , Antígeno CTLA-4/antagonistas & inibidores , Síndrome de Guillain-Barré/induzido quimicamente , Humanos , Miastenia Gravis/induzido quimicamente , Miosite/induzido quimicamente , Doenças Neuromusculares/induzido quimicamente , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Ensaios Clínicos Controlados Aleatórios como Assunto , Fatores de Risco
15.
Medicine (Baltimore) ; 98(18): e15321, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045770

RESUMO

Patients with myotonic muscular dystrophy type 1 (DM1) tend to exhibit earlier respiratory insufficiency than patients with other neuromuscular diseases at similar or higher forced vital capacity (FVC). This study aimed to analyze several pulmonary function parameters to determine which factor contributes the most to early hypercapnia in patients with DM1.We analyzed ventilation status monitoring, pulmonary function tests (including FVC, maximal voluntary ventilation [MVV], and maximal inspiratory and expiratory pressure), and polysomnography in subjects with DM1 who were admitted to a single university hospital. The correlation of each parameter with hypercapnia was determined. Subgroup analysis was also performed by dividing the subjects into 2 subgroups according to usage of mechanical ventilation.Final analysis included 50 patients with a mean age of 42.9 years (standard deviation = 11.1), 46.0% of whom were male. The hypercapnia was negatively correlated with MVV, FVC, forced expiratory volume in 1 second (FEV1), and their ratios to predicted values in subjects with myotonic muscular dystrophy type 1. At the same partial pressure of carbon dioxide, the ratio to the predicted value was lowest for MVV, then FEV1, followed by FVC. Moreover, the P values for differences in MVV and its ratio to the predicted value between ventilator users and nonusers were the lowest.When screening ventilation failure in patients with DM1, MVV should be considered alongside other routinely measured parameters.


Assuntos
Hipercapnia/fisiopatologia , Pulmão/fisiopatologia , Ventilação Voluntária Máxima/fisiologia , Distrofia Miotônica/complicações , Adulto , Dióxido de Carbono/análise , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Masculino , Pressões Respiratórias Máximas/métodos , Pessoa de Meia-Idade , Distrofia Miotônica/classificação , Distrofia Miotônica/fisiopatologia , Doenças Neuromusculares/epidemiologia , Doenças Neuromusculares/fisiopatologia , Polissonografia/métodos , Valor Preditivo dos Testes , Pressão , Estudos Prospectivos , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Capacidade Vital/fisiologia
16.
Crit Care Resusc ; 21(2): 110-118, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31142241

RESUMO

OBJECTIVES: Persistent critical illness (PerCI) is associated with high mortality and discharge to institutional care. Little is known about factors involved in its progression, complications and cause of death. We aimed to identify such factors and the time when the original illness was no longer the reason for intensive care unit (ICU) stay. DESIGN: Retrospective matched case-control study using an accepted PerCI definition (> 10 days in ICU). SETTING: Single-centre tertiary metropolitan ICU. PARTICIPANTS: All adult patients admitted during a 2-year period were eligible, matched on diagnostic code, gender, age and risk of death. MAIN RESULTS: Seventy-two patients staying > 10 days (PerCI cases) were matched to 72 control patients. The original illness was no longer a cause for continued ICU stay after a median of 10 days (interquartile range [IQR], 7-16) versus 2 days (IQR, 0-3); P < 0.001. Patients with PerCI were more likely to develop new sepsis (52.8% v 23.6%; P < 0.001), delirium (37.5% v 9.7%; P < 0.001), ICU-acquired weakness (15.3% v 0%, P = 0.001), and to be discharged to chronic care or rehabilitation (37.5% v 16.7%; P < 0.005). Death resulting from sepsis with multi-organ failure occurred in 16.7% v 8.3% of control patients (P = 0.13), and one-third of patients with PerCI were not mechanically ventilated on Day 10. CONCLUSION: PerCI likely results from complications acquired after ICU admission and mostly unrelated to the original illness; by Day 10, the original illness does not appear to be its cause, and new sepsis appears an important association.


Assuntos
Cuidados Críticos , Estado Terminal/mortalidade , Sepse/complicações , Adulto , Estudos de Casos e Controles , Causas de Morte , Estado Terminal/terapia , Infecção Hospitalar/mortalidade , Delírio/complicações , Delírio/mortalidade , Feminino , Humanos , Unidades de Terapia Intensiva , Tempo de Internação , Masculino , Doenças Neuromusculares/complicações , Doenças Neuromusculares/mortalidade , Estudos Retrospectivos , Sepse/mortalidade
17.
Arch. bronconeumol. (Ed. impr.) ; 55(5): 246-251, mayo 2019. graf, tab
Artigo em Inglês | IBECS | ID: ibc-181745

RESUMO

Introduction: The aim of this study was to assess several air-pressure settings for MI–E to determine their effect on peak cough flow (PCF), and to compare the best pressures with those are more common used in the literature (±40cmH2O) in patients with neuromuscular disorders (NMD). Methods: Adults with NMD in whom MI–E was indicated were recruited. Assisted PCF was measured by an external pneumotachograph. The protocol included 9 PCF measures per patient: 1 baseline (non-assisted), 4 with increasing inspiratory pressures without negative pressure (10, 20, 30 and 40cmH2O or maximum tolerated), and then 4 adding expiratory pressures (-10, -20, -30 and -40cm H2O or maximum tolerated) with maximum inspiratory pressure previously achieved. Results: Twenty one patients were included, 61% with amyotrophic lateral sclerosis (ALS). Mean PCFs with recommended pressures (± 40 cmH2O) were lower than the scored in the individualized steps of the titration protocol (197.7 ± 67l/min vs 214.2 ± 60 l/min, p < 0.05). Regarding subgroups, mean PCFmax values in ALS patients with bulbar symptoms were significantly higher than those achieved with recommended pressures (163.6 ± 80 vs 189 ± 66l/min, p < 0.05). Conclusion: The PCFmax obtained with the protocol did not always match the recommended settings. It may be advisable to perform MI–E titration assessed by non-invasive PCF monitoring in patients with NMD, especially in ALS with bulbar involvement to improve the therapy detecting airway collapse induced by high pressures


Introducción: El objetivo del estudio fue evaluar varios ajustes de presión para insuflación-exuflación mecánica (IEM) para determinar su efecto sobre el flujo pico de tos (FPT), y comparar las mejores presiones con aquellas que son habitualmente utilizadas en la literatura (± 40 cmH2O) en pacientes con enfermedades neuromusculares (ENM). Métodos: Se reclutaron adultos con ENM en los que se indicó IEM. Se midió el FPT asistido mediante un neumotacógrafo externo, y se registraron las curvas de presión y flujo/tiempo. El protocolo incluía 9 medidas de FPT por paciente: una basal (no asistida), 4 con presiones inspiratorias crecientes sin presión negativa (10, 20, 30 y 40 cmH2O o máximo tolerado), y luego 4 presiones espiratorias (-10, -20, -30, -40 cmH2O o máxima tolerada) con la presión inspiratoria máxima conseguida. Resultados: Se incluyeron 21 pacientes, 61% con esclerosis lateral amiotrófica (ELA). Las altas presiones (>±35 cmH2O) lograron el mejor FPT solo en el 50% de los sujetos. Los FPT medios con presiones recomendadas de ± 40 cmH2O (197,7 ± 67 l/min) fueron más bajos que los conseguidos con presiones de titulación individualizadas con el protocolo (214,2 ± 60 l/min, p < 0,05). Con respecto a los subgrupos, los valores medios de FPT máximos en los pacientes con ELA con síntomas bulbares fueron significativamente mayores que los logrados con las presiones recomendadas (163,6±80 frente a 189 ± 66 l/min p < 0,05). Conclusión: El FPT máximo obtenido con el protocolo no siempre coincide con las presiones más altas. Puede ser aconsejable realizar una valoración de IEM evaluada mediante monitorización no invasiva de FPT en pacientes con ENM, especialmente en ELA con afectación bulbar, para mejorar la terapia que detecta el colapso de la vía aérea inducida por altas presiones


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Pressões Respiratórias Máximas/métodos , Tosse/diagnóstico , Insuflação/métodos , Esclerose Amiotrófica Lateral/reabilitação , Respiração Artificial/métodos , Estudos Prospectivos , Estudos de Coortes , Pressões Respiratórias Máximas/instrumentação , Tosse/fisiopatologia , Doenças Neuromusculares/fisiopatologia , Mecânica Respiratória/fisiologia , Testes de Função Respiratória/métodos , Monitorização Fisiológica
19.
MBio ; 10(2)2019 04 02.
Artigo em Inglês | MEDLINE | ID: mdl-30940708

RESUMO

Since 2014, acute flaccid myelitis (AFM), a long-recognized condition associated with polioviruses, nonpolio enteroviruses, and various other viral and nonviral causes, has been reemerging globally in epidemic form. This unanticipated reemergence is ironic, given that polioviruses, once the major causes of AFM, are now at the very threshold of global eradication and cannot therefore explain any aspect of AFM reemergence. Instead, the new AFM epidemic has been temporally associated with reemergences of nonpolio enteroviruses such as EV-D68, until recently thought to be an obscure virus of extremely low endemicity. This perspective reviews the enigmatic epidemiologic, virologic, and diagnostic aspects of epidemic AFM reemergence; examines current options for clinical management; discusses future research needs; and suggests that the AFM epidemic offers important clues to mechanisms of viral disease emergence.


Assuntos
Viroses do Sistema Nervoso Central/epidemiologia , Doenças Transmissíveis Emergentes/epidemiologia , Gerenciamento Clínico , Mielite/epidemiologia , Doenças Neuromusculares/epidemiologia , Viroses do Sistema Nervoso Central/diagnóstico , Viroses do Sistema Nervoso Central/etiologia , Viroses do Sistema Nervoso Central/terapia , Doenças Transmissíveis Emergentes/diagnóstico , Doenças Transmissíveis Emergentes/etiologia , Doenças Transmissíveis Emergentes/terapia , Saúde Global , Humanos , Mielite/diagnóstico , Mielite/etiologia , Mielite/terapia , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/etiologia , Doenças Neuromusculares/terapia
20.
Medicine (Baltimore) ; 98(15): e15124, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30985673

RESUMO

The current study was performed to assess serial changes in neuromuscular control until 1 year postoperatively in nonathletic patients undergoing anterior cruciate ligament reconstruction (ACLR).Ninety-six patients were included. Serial neuromuscular control tests were performed preoperatively, at 6 months, and 1 year postoperatively. Neuromuscular control was evaluated using acceleration time (AT) and dynamic postural stability (overall stability index, OSI). Functional activity levels were assessed using the Tegner activity-level scale.Preoperative AT of quadriceps and hamstrings in operated knees was 78.9 ±â€Š6.4 and 86.5 ±â€Š6.2 ms, respectively, which significantly reduced to 56.9 ±â€Š2.0 and 62.5 ±â€Š2.8 ms at 1 year (P = 0.006 and 0.002, respectively). In nonoperated knees, preoperative AT of quadriceps and hamstrings was 47.6 ±â€Š1.7 and 56.5 ±â€Š1.7 ms, respectively, which was significantly prolonged to 54.3 ±â€Š2.0 and 67.9 ±â€Š2.7 ms at 1 year (P = 0.02 and 0.001, respectively). Preoperative OSI of nonoperated knees was 1.2 ±â€Š0.0°. It significantly increased to 1.5 ±â€Š0.1° at 1 year (P < 0.001). In operated knees, preoperative OSI was 1.8 ±â€Š0.1°. It significantly decreased to 1.4 ±â€Š0.1° at 1 year (P = 0.001). Tegner scale at 6 months and 1 year were significantly lower than pre-operative scale (P < 0.001). AT and OSI on both knees showed significant negative correlation with Tegner scale at 6 months and 1 year.Neuromuscular control in both knees was not restored to preoperative levels of the nonoperated knees until 1 year after ACLR. Therefore, clinicians and physical therapists should attempt to enhance neuromuscular control in both nonoperated and operated knees.


Assuntos
Reconstrução do Ligamento Cruzado Anterior , Doenças Neuromusculares/etiologia , Complicações Pós-Operatórias , Adulto , Feminino , Músculos Isquiotibiais , Humanos , Masculino , Destreza Motora , Doenças Neuromusculares/reabilitação , Complicações Pós-Operatórias/reabilitação , Equilíbrio Postural , Músculo Quadríceps , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
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