Base de dados : MEDLINE
Pesquisa : C10.668 [Categoria DeCS]
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[PMID]:28448298
[Au] Autor:Katz JA; Murphy GS
[Ad] Endereço:Northshore University Health System, University of Chicago, Pritzker School of Medicine, Illinois, USA.
[Ti] Título:Anesthetic consideration for neuromuscular diseases.
[So] Source:Curr Opin Anaesthesiol;30(3):435-440, 2017 Jun.
[Is] ISSN:1473-6500
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE OF REVIEW: The aim of this review is to examine data relating to perioperative management of the patient with neuromuscular disorders RECENT FINDINGS: Patients with pre-existing neuromuscular disorders are at risk for a number of postoperative complications that are related to anesthetic drugs that are administered intraoperatively. Careful preoperative assessment is necessary to reduce morbidity and mortality. In particular, the risk of postoperative respiratory failure and need for long-term ventilation should be reviewed with patients. The use of succinylcholine should be avoided in muscular dystrophies, motor neuron diseases, and intrinsic muscle disease due to a risk of malignant hyperthermia, hyperkalemia, rhabdomyolysis, and cardiac arrest. The use of quantitative neuromuscular monitoring should be strongly considered whenever nondepolarizing neuromuscular blocking agents are administered. A number of case series and reports have been recently published demonstrating that sugammadex can be safely used in patients with neuromuscular disease; the risk of residual neuromuscular is nearly eliminated when this agent is administered intraoperatively. SUMMARY: Careful assessment and management of patients with underlying neuromuscular diseases is required to reduce postoperative complications. This article reviews the anesthetic implications of patients undergoing surgery with neuromuscular disorder.
[Mh] Termos MeSH primário: Anestesia/efeitos adversos
Anestésicos/efeitos adversos
Bloqueadores Neuromusculares/efeitos adversos
Doenças Neuromusculares/complicações
Assistência Perioperatória/métodos
Complicações Pós-Operatórias/etiologia
Procedimentos Cirúrgicos Operatórios/efeitos adversos
[Mh] Termos MeSH secundário: Anestesia/métodos
Anestésicos/administração & dosagem
Parada Cardíaca/induzido quimicamente
Parada Cardíaca/prevenção & controle
Seres Humanos
Hiperpotassemia/induzido quimicamente
Complicações Intraoperatórias/induzido quimicamente
Complicações Intraoperatórias/prevenção & controle
Hipertermia Maligna/etiologia
Bloqueadores Neuromusculares/administração & dosagem
Doenças Neuromusculares/epidemiologia
Monitoração Neuromuscular
Complicações Pós-Operatórias/prevenção & controle
Prevalência
Insuficiência Respiratória/prevenção & controle
Rabdomiólise/induzido quimicamente
Rabdomiólise/prevenção & controle
Medição de Risco
Succinilcolina/administração & dosagem
Succinilcolina/efeitos adversos
gama-Ciclodextrinas/administração & dosagem
gama-Ciclodextrinas/efeitos adversos
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Anesthetics); 0 (Neuromuscular Blocking Agents); 0 (gamma-Cyclodextrins); 361LPM2T56 (Sugammadex); J2R869A8YF (Succinylcholine)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180309
[Lr] Data última revisão:
180309
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170428
[St] Status:MEDLINE
[do] DOI:10.1097/ACO.0000000000000466


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[PMID]:29181926
[Au] Autor:Haug MK; Willumsen J; Fossmo HL
[Ti] Título:Kjenner du de hemmelige tjenestene?.
[So] Source:Tidsskr Nor Laegeforen;137(22), 2017 11 28.
[Is] ISSN:0807-7096
[Cp] País de publicação:Norway
[La] Idioma:nor
[Mh] Termos MeSH primário: Competência Clínica
Doenças Neuromusculares
Atenção Secundária à Saúde
[Mh] Termos MeSH secundário: Pessoal de Saúde/normas
Seres Humanos
Doenças Neuromusculares/diagnóstico
Doenças Neuromusculares/terapia
Encaminhamento e Consulta
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180301
[Lr] Data última revisão:
180301
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171129
[St] Status:MEDLINE
[do] DOI:10.4045/tidsskr.17.0882


  3 / 9320 MEDLINE  
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[PMID]:28451990
[Au] Autor:Solav D; Meric H; Rubin MB; Pradon D; Lofaso F; Wolf A
[Ad] Endereço:Faculty of Mechanical Engineering, Technion Israel Institute of Technology, 32000, Haifa, Israel. danask@mit.edu.
[Ti] Título:Chest Wall Kinematics Using Triangular Cosserat Point Elements in Healthy and Neuromuscular Subjects.
[So] Source:Ann Biomed Eng;45(8):1963-1973, 2017 08.
[Is] ISSN:1573-9686
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Optoelectronic plethysmography (OEP) is a noninvasive method for assessing lung volume variations and the contributions of different anatomical compartments of the chest wall (CW) through measurements of the motion of markers attached to the CW surface. The present study proposes a new method for analyzing the local CW kinematics from OEP measurements based on the kinematics of triangular Cosserat point elements (TCPEs). 52 reflective markers were placed on the anterior CW to create a mesh of 78 triangles according to an anatomical model. Each triangle was characterized by a TCPE and its kinematics was described using four time-variant scalar TCPE parameters. The total CW volume ([Formula: see text]) and the contributions of its six compartments were also estimated, using the same markers. The method was evaluated using measurements of ten healthy subjects, nine patients with Pompe disease, and ten patients with Duchenne muscular dystrophy (DMD), during spontaneous breathing (SB) and vital capacity maneuvers (VC) in the supine position. TCPE parameters and compartmental volumes were compared with [Formula: see text] by computing the phase angles [Formula: see text] (for SB) and the correlation r (for VC) between them. Analysis of [Formula: see text] and r of the outward translation parameter [Formula: see text] of each TCPE revealed that for healthy subjects it provided similar results to those obtained by compartmental volumes, whereas for the neuromuscular patients the TCPE method was capable of detecting local asynchronous and paradoxical movements also in cases where they were undistinguished by volumes. Therefore, the TCPE approach provides additional information to OEP that may enhance its clinical evaluation capabilities.
[Mh] Termos MeSH primário: Medidas de Volume Pulmonar/métodos
Modelos Biológicos
Doenças Neuromusculares/fisiopatologia
Fotopletismografia/métodos
Mecânica Respiratória
Parede Torácica/fisiopatologia
Volume de Ventilação Pulmonar
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Simulação por Computador
Feminino
Análise de Elementos Finitos
Doença de Depósito de Glicogênio Tipo II
Seres Humanos
Masculino
Meia-Idade
Doenças Neuromusculares/diagnóstico
Valores de Referência
Reprodutibilidade dos Testes
Sensibilidade e Especificidade
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180215
[Lr] Data última revisão:
180215
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170429
[St] Status:MEDLINE
[do] DOI:10.1007/s10439-017-1840-6


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[PMID]:29326244
[Au] Autor:Dunbar CE; High KA; Joung JK; Kohn DB; Ozawa K; Sadelain M
[Ad] Endereço:Hematology Branch, National Heart, Lung and Blood Institute, Bethesda, MD, USA. dunbarc@nhlbi.nih.gov m-sadelain@ski.mskcc.org.
[Ti] Título:Gene therapy comes of age.
[So] Source:Science;359(6372), 2018 01 12.
[Is] ISSN:1095-9203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:After almost 30 years of promise tempered by setbacks, gene therapies are rapidly becoming a critical component of the therapeutic armamentarium for a variety of inherited and acquired human diseases. Gene therapies for inherited immune disorders, hemophilia, eye and neurodegenerative disorders, and lymphoid cancers recently progressed to approved drug status in the United States and Europe, or are anticipated to receive approval in the near future. In this Review, we discuss milestones in the development of gene therapies, focusing on direct in vivo administration of viral vectors and adoptive transfer of genetically engineered T cells or hematopoietic stem cells. We also discuss emerging genome editing technologies that should further advance the scope and efficacy of gene therapy approaches.
[Mh] Termos MeSH primário: Terapia Genética
[Mh] Termos MeSH secundário: Animais
Edição de Genes
Técnicas de Transferência de Genes
Doenças Genéticas Inatas/terapia
Engenharia Genética
Terapia Genética/efeitos adversos
Vetores Genéticos
Doenças Hematológicas/terapia
Seres Humanos
Neoplasias/terapia
Doenças Neuromusculares/terapia
Pesquisa Médica Translacional
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180207
[Lr] Data última revisão:
180207
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180113
[St] Status:MEDLINE


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[PMID]:29236822
[Au] Autor:Cotta A; Paim JF; Carvalho E; da-Cunha-Júnior AL; Navarro MM; Valicek J; Menezes MM; Nunes SV; Xavier-Neto R; Baptista S; Lima LR; Takata RI; Vargas AP
[Ad] Endereço:Rede SARAH de Hospitais de Reabilitação, Departamento de Patologia, Belo Horizonte MG, Brasil.
[Ti] Título:The relative frequency of common neuromuscular diagnoses in a reference center.
[So] Source:Arq Neuropsiquiatr;75(11):789-795, 2017 Nov.
[Is] ISSN:1678-4227
[Cp] País de publicação:Brazil
[La] Idioma:eng
[Ab] Resumo:The diagnostic procedure in neuromuscular patients is complex. Knowledge of the relative frequency of neuromuscular diseases within the investigated population is important to allow the neurologist to perform the most appropriate diagnostic tests. OBJECTIVE: To report the relative frequency of common neuromuscular diagnoses in a reference center. METHODS: A 17-year chart review of patients with suspicion of myopathy. RESULTS: Among 3,412 examinations, 1,603 (46.98%) yielded confirmatory results: 782 (48.78%) underwent molecular studies, and 821 (51.21%) had muscle biopsies. The most frequent diagnoses were: dystrophinopathy 460 (28.70%), mitochondriopathy 330 (20.59%), spinal muscular atrophy 158 (9.86%), limb girdle muscular dystrophy 157 (9.79%), Steinert myotonic dystrophy 138 (8.61%), facioscapulohumeral muscular dystrophy 99 (6.17%), and other diagnoses 261 (16.28%). CONCLUSION: Using the presently-available diagnostic techniques in this service, a specific limb girdle muscular dystrophy subtype diagnosis was reached in 61% of the patients. A neuromuscular-appropriate diagnosis is important for genetic counseling, rehabilitation orientation, and early treatment of respiratory and cardiac complications.
[Mh] Termos MeSH primário: Doenças Neuromusculares/diagnóstico
[Mh] Termos MeSH secundário: Biópsia
Feminino
Seres Humanos
Masculino
Doenças Neuromusculares/genética
Doenças Neuromusculares/patologia
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180129
[Lr] Data última revisão:
180129
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171214
[St] Status:MEDLINE


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[PMID]:29199768
[Au] Autor:Luo F; Annane D; Orlikowski D; He L; Yang M; Zhou M; Liu GJ
[Ad] Endereço:Department of Neurology, West China Hospital, Sichuan University, Chengdu, China.
[Ti] Título:Invasive versus non-invasive ventilation for acute respiratory failure in neuromuscular disease and chest wall disorders.
[So] Source:Cochrane Database Syst Rev;12:CD008380, 2017 Dec 04.
[Is] ISSN:1469-493X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Acute respiratory failure is a common life-threatening complication of acute onset neuromuscular diseases, and may exacerbate chronic hypoventilation in patients with neuromuscular disease or chest wall disorders. Standard management includes oxygen supplementation, physiotherapy, cough assistance, and, whenever needed, antibiotics and intermittent positive pressure ventilation. Non-invasive mechanical ventilation (NIV) via nasal, buccal or full-face devices has become routine practice in many centres. OBJECTIVES: The primary objective of this review was to compare the efficacy of non-invasive ventilation with invasive ventilation in improving short-term survival in acute respiratory failure in people with neuromuscular disease and chest wall disorders. The secondary objectives were to compare the effects of NIV with those of invasive mechanical ventilation on improvement in arterial blood gas after 24 hours and lung function measurements after one month, incidence of barotrauma and ventilator-associated pneumonia, duration of mechanical ventilation, length of stay in the intensive care unit and length of hospital stay. SEARCH METHODS: We searched the following databases on 11 September 2017: the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE and Embase. We also searched conference proceedings and clinical trials registries. SELECTION CRITERIA: We planned to include randomised or quasi-randomised trials with or without blinding. We planned to include trials performed in children or adults with acute onset neuromuscular diseases or chronic neuromuscular disease or chest wall disorders presenting with acute respiratory failure that compared the benefits and risks of invasive ventilation versus NIV. DATA COLLECTION AND ANALYSIS: Two review authors reviewed searches and independently selected studies for assessment. We planned to follow standard Cochrane methodology for data collection and analysis. MAIN RESULTS: We did not identify any trials eligible for inclusion in the review. AUTHORS' CONCLUSIONS: Acute respiratory failure is a life-threatening complication of acute onset neuromuscular disease and of chronic neuromuscular disease and chest wall disorders. We found no randomised trials on which to elaborate evidence-based practice for the use of non-invasive versus invasive mechanical ventilation. For researchers, there is a need to design and conduct new randomised trials to compare NIV with invasive ventilation in acute neuromuscular respiratory failure. These trials should anticipate variations in treatment responses according to disease condition (acute onset versus acute exacerbation on chronic neuromuscular diseases) and according to the presence or absence of bulbar dysfunction.
[Mh] Termos MeSH primário: Doenças Neuromusculares/complicações
Ventilação não Invasiva
Respiração Artificial
Insuficiência Respiratória/terapia
Parede Torácica/anormalidades
[Mh] Termos MeSH secundário: Doença Aguda
Seres Humanos
Insuficiência Respiratória/etiologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180123
[Lr] Data última revisão:
180123
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171205
[St] Status:MEDLINE
[do] DOI:10.1002/14651858.CD008380.pub2


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[PMID]:29243458
[Au] Autor:Siirala W; Vainionpää A; Kainu A; Korpela J; Olkkola K; Aantaa R
[Ti] Título:Prevalence of life-supporting prolonged invasive ventilation support in Finland.
[So] Source:Duodecim;133(7):675-82, 2017.
[Is] ISSN:0012-7183
[Cp] País de publicação:Finland
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: There is no comprehensive data in our country on the prevalence of life-supporting prolonged invasive ventilation support. The objective of the survey was to clarify in all hospital districts of continental Finland the prevalence of patients who were dependent on invasive ventilation support, and the disease leading to the treatment. PATIENTS AND METHODS: The KOTIVEHNO 2015 survey was carried out as population-based cross-sectional study by sending a questionnaire to all doctors in charge of prolonged invasive ventilation support. The questionnaires were used to collect data on the patients within care on 1st January, 2017. RESULTS: The prevalence of life-supporting prolonged invasive ventilation support in Finland among the population aged over 16 years or more was 2/4 patients/100,000 habitants. Altogether 107 patients were within the care. There was variation in the prevalence among the hospital districts. Of the patients, 24% were affected with a motoneuron disease, in 18% the cause was spinal cord injury, 15% suffered from Duchenne's muscular dystrophy, and the rest had some other rare neurological disease. CONCLUSIONS: Life-supporting prolonged invasive ventilation support is rare in Finland. The treatment is associated with neuromuscular diseases causing respiratory insufficiency, and with spinal cord injuries.
[Mh] Termos MeSH primário: Cuidados para Prolongar a Vida
Doenças Neuromusculares/fisiopatologia
Doenças Neuromusculares/terapia
Respiração Artificial/métodos
Insuficiência Respiratória/fisiopatologia
Insuficiência Respiratória/terapia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Estudos Transversais
Feminino
Finlândia
Seres Humanos
Masculino
Meia-Idade
Prevalência
Inquéritos e Questionários
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180115
[Lr] Data última revisão:
180115
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171216
[St] Status:MEDLINE


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[PMID]:29292335
[Au] Autor:Konan S; Duncan CP
[Ad] Endereço:University College London Hospitals NHS Trust, 250 Euston Road, London NW1 2BU, UK.
[Ti] Título:Total hip arthroplasty in patients with neuromuscular imbalance.
[So] Source:Bone Joint J;100-B(1 Supple A):17-21, 2018 Jan.
[Is] ISSN:2049-4408
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Patients with neuromuscular imbalance who require total hip arthroplasty (THA) present particular technical problems due to altered anatomy, abnormal bone stock, muscular imbalance and problems of rehabilitation. In this systematic review, we studied articles dealing with THA in patients with neuromuscular imbalance, published before April 2017. We recorded the demographics of the patients and the type of neuromuscular pathology, the indication for surgery, surgical approach, concomitant soft-tissue releases, the type of implant and bearing, pain and functional outcome as well as complications and survival. Recent advances in THA technology allow for successful outcomes in these patients. Our review suggests excellent benefits for pain relief and good functional outcome might be expected with a modest risk of complication. Cite this article: 2018;100-B(1 Supple A):17-21.
[Mh] Termos MeSH primário: Artroplastia de Quadril
Doenças Neuromusculares/complicações
Osteoartrite do Quadril/cirurgia
Equilíbrio Postural
[Mh] Termos MeSH secundário: Artroplastia de Quadril/instrumentação
Artroplastia de Quadril/métodos
Seres Humanos
Doenças Neuromusculares/fisiopatologia
Osteoartrite do Quadril/complicações
Complicações Pós-Operatórias/etiologia
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180104
[Lr] Data última revisão:
180104
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180103
[St] Status:MEDLINE
[do] DOI:10.1302/0301-620X.100B1.BJJ-2017-0571.R1


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[PMID]:29173414
[Au] Autor:Limipitikul W; Ong CS; Tomaselli GF
[Ad] Endereço:Department of Biomedical Engineering, Johns Hopkins University, Baltimore, MD, USA.
[Ti] Título:Neuromuscular Disease: Cardiac Manifestations and Sudden Death Risk.
[So] Source:Card Electrophysiol Clin;9(4):731-747, 2017 Dec.
[Is] ISSN:1877-9190
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Cardiovascular complications of neuromuscular diseases disproportionately affect the cardiac conduction system. Cardiomyopathy and cardiac arrhythmias produce significant morbidity and mortality. Patients with neuromuscular diseases should be carefully and frequently evaluated for the presence of bradycardia, heart block, and tachyarrhythmias. Preemptive treatment with permanent pacemakers or implanted defibrillators is appropriate in patients with conduction system disease or who are at risk for ventricular arrhythmias.
[Mh] Termos MeSH primário: Morte Súbita Cardíaca
Doenças Neuromusculares
[Mh] Termos MeSH secundário: Arritmias Cardíacas
Desfibriladores Implantáveis
Bloqueio Cardíaco
Seres Humanos
Fatores de Risco
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171227
[Lr] Data última revisão:
171227
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE


  10 / 9320 MEDLINE  
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[PMID]:28969372
[Au] Autor:Adriaenssens E; Geuens T; Baets J; Echaniz-Laguna A; Timmerman V
[Ad] Endereço:Peripheral Neuropathy Research Group, Institute Born Bunge, University of Antwerp, Antwerpen, Belgium.
[Ti] Título:Novel insights in the disease biology of mutant small heat shock proteins in neuromuscular diseases.
[So] Source:Brain;140(10):2541-2549, 2017 Oct 01.
[Is] ISSN:1460-2156
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Small heat shock proteins are molecular chaperones that exert diverse cellular functions. To date, mutations in the coding regions of HSPB1 (Hsp27) and HSPB8 (Hsp22) were reported to cause distal hereditary motor neuropathy and Charcot-Marie-Tooth disease. Recently, the clinical spectrum of HSPB1 and HSPB8 mutations was expanded to also include myopathies. Here we provide an update on the molecular genetics and biology of small heat shock protein mutations in neuromuscular diseases.
[Mh] Termos MeSH primário: Proteínas de Choque Térmico HSP27/genética
Proteínas de Choque Térmico/genética
Mutação/genética
Doenças Neuromusculares/genética
Proteínas Serina-Treonina Quinases/genética
[Mh] Termos MeSH secundário: Idoso
Idoso de 80 Anos ou mais
Seres Humanos
Masculino
Modelos Moleculares
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (HSP27 Heat-Shock Proteins); 0 (HSPB1 protein, human); 0 (Heat-Shock Proteins); EC 2.7.1.- (HSPB8 protein, human); EC 2.7.11.1 (Protein-Serine-Threonine Kinases)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171006
[Lr] Data última revisão:
171006
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171004
[St] Status:MEDLINE
[do] DOI:10.1093/brain/awx187



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