Pesquisa | SciELO

ABSTRACT Objective: To update the recommendations to support decisions regarding the pharmacological treatment of patients hospitalized with COVID-19 in Brazil. Methods: Experts, including representatives of the Ministry of Health and methodologists, created this guideline. The method used for the rapid development of guidelines was based on the adoption and/or adaptation of existing international guidelines (GRADE ADOLOPMENT) and supported by the e-COVID-19 RecMap platform. The quality of the evidence and the preparation of the recommendations followed the GRADE method. Results: Twenty-one recommendations were generated, including strong recommendations for the use of corticosteroids in patients using supplemental oxygen and conditional recommendations for the use of tocilizumab and baricitinib for patients on supplemental oxygen or on noninvasive ventilation and anticoagulants to prevent thromboembolism. Due to suspension of use authorization, it was not possible to make recommendations regarding the use of casirivimab + imdevimab. Strong recommendations against the use of azithromycin in patients without suspected bacterial infection, hydroxychloroquine, convalescent plasma, colchicine, and lopinavir + ritonavir and conditional recommendations against the use of ivermectin and remdesivir were made. Conclusion: New recommendations for the treatment of hospitalized patients with COVID-19 were generated, such as those for tocilizumab and baricitinib. Corticosteroids and prophylaxis for thromboembolism are still recommended, the latter with conditional recommendation. Several drugs were considered ineffective and should not be used to provide the best treatment according to the principles of evidence-based medicine and to promote resource economy.

RESUMO Objetivo: Atualizar as recomendações para embasar as decisões para o tratamento farmacológico de pacientes hospitalizados com COVID-19 no Brasil. Métodos: A elaboração desta diretriz foi feita por especialistas, incluindo representantes do Ministério da Saúde e metodologistas. O método utilizado para o desenvolvimento rápido de diretrizes baseou-se na adoção e/ou adaptação de diretrizes internacionais existentes (GRADE ADOLOPMENT) e contou com o apoio da plataforma e-COVID-19 RecMap. A qualidade das evidências e a elaboração das recomendações seguiram o método GRADE. Resultados: Chegaram-se a 21 recomendações, incluindo recomendações fortes quanto ao uso de corticosteroides em pacientes em uso de oxigênio suplementar e recomendações condicionais para o uso de tocilizumabe e baricitinibe, em pacientes com oxigênio suplementar ou ventilação não invasiva, e de anticoagulantes, para prevenção de tromboembolismo. Devido à suspensão da autorização de uso, não foi possível fazer recomendações para o tratamento com casirivimabe + imdevimabe. Foram feitas recomendações fortes contra o uso de azitromicina em pacientes sem suspeita de infecção bacteriana, hidroxicloroquina, plasma convalescente, colchicina e lopinavir + ritonavir, além de recomendações condicionais contra o uso de ivermectina e rendesivir. Conclusão: Foram criadas novas recomendações para o tratamento de pacientes hospitalizados com COVID-19, como as recomendações de tocilizumabe e baricitinibe. Ainda são recomendados corticosteroides e profilaxia contra tromboembolismo, esta em caráter condicional. Vários medicamentos foram considerados ineficazes e não devem ser usados, no intuito de proporcionar o melhor tratamento segundo os princípios da medicina baseada em evidências e promover a economia de recursos.

https://doi.org/10.5935/2965-2774.20230136-en

Patient suspected susceptibility to malignant hyperthermia: impact of the disease
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Rodrigues, Gislene

; Andrade, Pamela Vieira

; Santos, Joilson Moura dos

; Castro, Isac de

; Amaral, José Luiz Gomes do

; Silva, Helga Cristina Almeida da

Brazilian Journal of Anesthesiology

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Abr 2023, Volume 73 Nº 2 Páginas 138 - 144

Resumo: En | Texto: En | PDF: En

Abstract Introduction Malignant Hyperthermia (MH) is an inherited hypermetabolic syndrome triggered by exposure to halogenated anesthetics/succinylcholine. The lack of knowledge regarding this condition might be associated with the rare occurrence of MH reaction and symptoms. Methods This observational study evaluated 68 patients from 48 families with confirmed or suspected MH susceptibility due to medical history of MH reaction or idiopathic increase of creatine kinase or MH-related myopathies. Participants were assessed by a standardized questionnaire and submitted to physical/neurological examination to assess the characteristics of patients with MH, their knowledge about the disease, and the impact suspected MH had on their daily lives. Results Suspected MH impacted the daily life of 50% of patients, creating difficulties in performing surgical/clinical/dental treatment and problems related to their family life/working/practicing sports. The questionnaire on MH revealed a correct answer score of 62.1 ± 20.8 (mean ± standard deviation) on a scale 0 to 100. Abnormal physical/neurological examination findings were detected in 92.6% of susceptible patients. Conclusions Suspected MH had impacted the daily lives of most patients, with patients reporting problems even before MH investigation with IVCT. Patients showed a moderate level of knowledge about MH, suggesting the need to implement continuing education programs. MH susceptible patients require regular follow-up by a health team to detect abnormalities during physical and neurological examination.

https://doi.org/10.1016/j.bjane.2021.10.021

Fatigue, depression, and physical activity in patients with malignant hyperthermia: a cross-sectional observational study
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Andrade, Pâmela Vieira de

; Valim, Lívia Maria

; Santos, Joilson Moura dos

; Castro, Isac de

; Amaral, José Luiz Gomes do

; Silva, Helga Cristina Almeida da

Brazilian Journal of Anesthesiology

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Abr 2023, Volume 73 Nº 2 Páginas 132 - 137

Resumo: En | Texto: En | PDF: En

Abstract Background Malignant Hyperthermia (MH) is a pharmacogenetic disorder triggered by halogenated anesthesia agents/succinylcholine and characterized by hypermetabolism crisis during anesthesia, but also by day-to-day symptoms, such as exercise intolerance, that may alert the health professional. Objective The study aimed to analyze the incidence of fatigue in MH susceptible patients and the variables that can impact perception of fatigue, such as the level of routine physical activity and depression. Methods A cross-sectional observational study was carried out with three groups - 22 patients susceptible to MH (positive in vitro muscle contracture test), 13 non-susceptible to MH (negative in vitro muscle contracture test) and 22 controls (no history of MH). Groups were assessed by a demographic/clinical questionnaire, a fatigue severity scale (intensity, specific situations, psychological consequences, rest/sleep response), and the Beck depression scale. Subgroups were re-assessed with the Baecke habitual physical exercise questionnaire (occupational physical activity, leisure physical exercise, leisure/locomotion physical activity). Results There were no significant differences among the three groups regarding fatigue intensity, fatigue related to specific situations, psychological consequences of fatigue, fatigue response to resting/sleeping, depression, number of active/sedentary participants, and the mean time and characteristics of habitual physical activity. Nevertheless, unlike the control sub-group, the physically active MH-susceptible subgroup had a higher fatigue response to resting/sleeping than the sedentary MH susceptible subgroup (respectively, 5.9 ± 1.9 vs. 3.9 ± 2, t-test unpaired, p< 0.05). Conclusion We did not detect subjective fatigue in MH susceptible patients, although we reported protracted recovery after physical activity, which may alert us to further investigation requirements.

https://doi.org/10.1016/j.bjane.2021.07.038

Predictive factors of the contracture test for diagnosing malignant hyperthermia in a Brazilian population sample: a retrospective observational study
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de Mello, Jean Marcel

; Andrade, Pamela Vieira

; Santos, Joilson Moura

; Oliveira, Acary Souza Bulle

; Vainzof, Mariz

; do Amaral, José Luiz Gomes

; Almeida da Silva, Helga Cristina

Brazilian Journal of Anesthesiology

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Abr 2023, Volume 73 Nº 2 Páginas 145 - 152

Resumo: En | Texto: En | PDF: En

Abstract Introduction Malignant Hyperthermia (MH) is a pharmacogenetic, hereditary and autosomal dominant syndrome triggered by halogenates/succinylcholine. The In Vitro Contracture Test (IVCT) is the gold standard diagnostic test for MH, and it evaluates abnormal skeletal muscle reactions of susceptible individuals (earlier/greater contracture) when exposed to caffeine/halothane. MH susceptibility episodes and IVCT seem to be related to individual features. Objective To assess variables that correlate with IVCT in Brazilian patients referred for MH investigation due to a history of personal/family MH. Methods We examined IVCTs of 80 patients investigated for MH between 2004‒2019. We recorded clinical data (age, sex, presence of muscle weakness or myopathy with muscle biopsy showing cores, genetic evaluation, IVCT result) and IVCT features (initial and final maximum contraction, caffeine/halothane concentration triggering contracture of 0.2g, contracture at caffeine concentration of 2 and 32 mmoL and at 2% halothane, and contraction after 100 Hz stimulation). Results Mean age of the sample was 35±13.3 years, and most of the subjects were female (n=43 or 54%) and MH susceptible (60%). Of the 20 subjects undergoing genetic investigation, 65% showed variants in RYR1/CACNA1S genes. We found no difference between the positive and negative IVCT groups regarding age, sex, number of probands, presence of muscle weakness or myopathy with muscle biopsy showing cores. Regression analysis revealed that the best predictors of positive IVCT were male sex (+12%), absence of muscle weakness (+20%), and personal MH background (+17%). Conclusions Positive IVCT results have been correlated to male probands, in accordance with early publications. Furthermore, normal muscle strength has been confirmed as a significant predictor of positive IVCT while investigating suspected MH cases.

https://doi.org/10.1016/j.bjane.2022.06.010

Reactions of physicians in the state of São Paulo to the use of telemedicine during the SARS-CoV-2 pandemic: cross-sectional study
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Amaral, José Luiz Gomes

; Endrigo, Antonio Carlos

; Malik, Ana Maria

; Ogata, Alberto José Niituma

; Assumpção Neto, Jorge Corrêa

Sao Paulo Medical Journal

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Jun 2022, Volume 140 Nº 3 Páginas 499 - 504

Resumo: En | Texto: En | PDF: En

ABSTRACT BACKGROUND: Telemedicine can be a component of integrated healthcare practices and its use is not a recent phenomenon around the world. In Brazil, its more widespread use began during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, through extraordinary authorization from the Brazilian Ministry of Health. OBJECTIVES: To describe some aspects of use of teleconsultation among a sample of physicians in the state of São Paulo during the SARS-CoV-2 pandemic. DESIGN AND SETTING: Cross-sectional study based on a survey conducted by the São Paulo Medical Association (Associação Paulista de Medicina, APM) on medical practice during the SARS-CoV-2 pandemic between December 18, 2020, and January 18, 2021. RESULTS: This survey generated responses from 2,052 physicians. Of these, 981 (47.8%) reported not practicing any form of telemedicine. Among those who reported practicing telemedicine, 274 (28.4%) reported not receiving remuneration directly for the attendance provided and 225 (23.3%) reported receiving remuneration equal to what they would have received from face-to-face consultations. Regarding the professional linkage of the physicians who undertook telemedicine attendance, the majority (499; 51.8%) only attended private patients. Regarding the resources used to provide telemedicine attendance, most of the respondents used specialized digital platforms (594; 61.6%), electronic health records (592; 61.4%) and electronic prescriptions (700; 72.6%). CONCLUSION: This study demonstrates that important issues such as professional remuneration, use of electronic platforms and medical records, ensuring data protection and relationships between physicians and other stakeholders still need to be better defined, in order to achieve the desired scale and reach the outcomes defined.

https://doi.org/10.1590/1516-3180.2021.0475.r3.03112021

Evaluation of dexmedetomidine anesthesia-related temperature changes: preliminary retrospective observational study
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Cruz, Felipe Aparecido Ferreira da

; Falcão, Luiz Fernando dos Reis

; Amaral, José Luiz Gomes do

; Silva, Helga Cristina Almeida da

Brazilian Journal of Anesthesiology

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Abr 2022, Volume 72 Nº 2 Páginas 232 - 240

Resumo: En | Texto: En | PDF: En

Abstract Introduction and objective: Dexmedetomidine is a potent adrenergic alpha-2 agonist, and analgesic, sedative, anxiolytic and sympatholytic. Given there have been reports of dexmedetomidine associated temperature changes, in which these events have been associated with complications, our objective was to describe both temperature increase and decrease, during the intra and postoperative period (initial 24 hours), and factors associated, in patients who received dexmedetomidine for anesthesia/sedation in the surgical suite. Method: Retrospective observational study, analyzing charts of patients ≥ 18 years submitted to anesthesia/sedation with dexmedetomidine, between 1/1/2017 and 31/12/2017. Upper temperature threshold was considered ≥ 37.8°C, and lower, < 35°C. The association with dexmedetomidine was assessed by the OMS/UMC causality system and by the Naranjo algorithm. Results: The sample included 42 patients who received dexmedetomidine and whose temperature data were available, with predominance of men 26 (62%), 49.4/16.5 years old (mean/standard deviation), and weight 65/35.8 kg. None of the patients presented intraoperative temperature equal to or above 37.8°C or below 35°C. During the postoperative period, one patient presented an increase ≥ 37.8°C (2.4%) and three, temperature decrease < 35°C (7%). Surgery/anesthesia time and exposure time to dexmedetomidine were not appropriate linear predictors of maximum temperature. Older age (p < 0.01), longer exposure to dexmedetomidine (p < 0.05) and shorter surgery time (p < 0.01) were significant linear predictors for lower minimum temperature. Abbreviations: MH, malignant hyperthermia; UMC, Uppsala Monitoring Centre; ICU, Intensive Care Unit; WHO, World Health Organization. Conclusions: Increase ≥ 37.8°C/decrease < 35°C of temperature possibly associated with dexmedetomidine did not occur in the intraoperative period and had a low frequency during the postoperative period.

https://doi.org/10.1016/j.bjane.2021.02.062

Diretrizes Brasileiras para o tratamento farmacológico de pacientes hospitalizados com COVID-19: Diretriz conjunta da Associação Brasileira de Medicina de Emergência, Associação de Medicina Intensiva Brasileira, Associação Médica Brasileira, Sociedade Brasileira de Angiologia e Cirurgia Vascular, Sociedade Brasileira de Infectologia, Sociedade Brasileira de Pneumologia e Tisiologia e Sociedade Brasileira de Reumatologia
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Falavigna, Maicon

; Stein, Cinara

; Amaral, José Luiz Gomes do

; Azevedo, Luciano Cesar Pontes de

; Belli, Karlyse Claudino

; Colpani, Verônica

; Cunha, Clóvis Arns da

; Dal-Pizzol, Felipe

; Dias, Maria Beatriz Souza

; Ferreira, Juliana Carvalho

; Freitas, Ana Paula da Rocha

; Gräf, Débora Dalmas

; Guimarães, Hélio Penna

; Lobo, Suzana Margareth Ajeje

; Monteiro, José Tadeu

; Nunes, Michelle Silva

; Oliveira, Maura Salaroli de

; Prado, Clementina Corah Lucas

; Santos, Vania Cristina Canuto

; Silva, Rosemeri Maurici da

; Sobreira, Marcone Lima

; Veiga, Viviane Cordeiro

; Vidal, Ávila Teixeira

; Xavier, Ricardo Machado

; Zavascki, Alexandre Prehn

; Machado, Flávia Ribeiro

; Carvalho, Carlos Roberto Ribeiro de

Revista Brasileira de Terapia Intensiva

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Mar 2022, Volume 34 Nº 1 Páginas 1 - 12

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

RESUMO Objetivos: Há diversas terapias sendo utilizadas ou propostas para a COVID-19, muitas carecendo de apropriada avaliação de efetividade e segurança. O propósito deste documento é elaborar recomendações para subsidiar decisões sobre o tratamento farmacológico de pacientes hospitalizados com COVID-19 no Brasil. Métodos: Um grupo de 27 membros, formado por especialistas, representantes do Ministério da Saúde e metodologistas, integra essa diretriz. Foi utilizado o método de elaboração de diretrizes rápidas, tomando por base a adoção e/ou a adaptação de recomendações a partir de diretrizes internacionais existentes (GRADE ADOLOPMENT), apoiadas pela plataforma e-COVID-19 RecMap. A qualidade das evidências e a elaboração das recomendações seguiram o método GRADE. Resultados: Foram geradas 16 recomendações. Entre elas, estão recomendações fortes para o uso de corticosteroides em pacientes em uso de oxigênio suplementar, para o uso de anticoagulantes em doses de profilaxia para tromboembolismo e para não uso de antibacterianos nos pacientes sem suspeita de infecção bacteriana. Não foi possível fazer uma recomendação quanto à utilização do tocilizumabe em pacientes hospitalizados com COVID-19 em uso de oxigênio, pelas incertezas na disponibilidade e de acesso ao medicamento. Foi feita recomendação para não usar azitromicina, casirivimabe + imdevimabe, cloroquina, colchicina, hidroxicloroquina, ivermectina, lopinavir/ ritonavir, plasma convalescente e rendesivir. Conclusão: Até o momento, poucas terapias se provaram efetivas no tratamento do paciente hospitalizado com COVID-19, sendo recomendados apenas corticosteroides e profilaxia para tromboembolismo. Diversos medicamentos foram considerados ineficazes, devendo ser descartados, de forma a oferecer o melhor tratamento pelos princípios da medicina baseada em evidências e promover economia de recursos não eficazes.

ABSTRACT Objective: Several therapies are being used or proposed for COVID-19, and many lack appropriate evaluations of their effectiveness and safety. The purpose of this document is to develop recommendations to support decisions regarding the pharmacological treatment of patients hospitalized with COVID-19 in Brazil. Methods: A group of 27 experts, including representatives of the Ministry of Health and methodologists, created this guideline. The method used for the rapid development of guidelines was based on the adoption and/or adaptation of existing international guidelines (GRADE ADOLOPMENT) and supported by the e-COVID-19 RecMap platform. The quality of the evidence and the preparation of the recommendations followed the GRADE method. Results: Sixteen recommendations were generated. They include strong recommendations for the use of corticosteroids in patients using supplemental oxygen, the use of anticoagulants at prophylactic doses to prevent thromboembolism and the nonuse of antibiotics in patients without suspected bacterial infection. It was not possible to make a recommendation regarding the use of tocilizumab in patients hospitalized with COVID-19 using oxygen due to uncertainties regarding the availability of and access to the drug. Strong recommendations against the use of hydroxychloroquine, convalescent plasma, colchicine, lopinavir + ritonavir and antibiotics in patients without suspected bacterial infection and also conditional recommendations against the use of casirivimab + imdevimab, ivermectin and rendesivir were made. Conclusion: To date, few therapies have proven effective in the treatment of hospitalized patients with COVID-19, and only corticosteroids and prophylaxis for thromboembolism are recommended. Several drugs were considered ineffective and should not be used to provide the best treatment according to the principles of evidence-based medicine and promote economical resource use.

https://doi.org/10.5935/0103-507x.20220001-pt

[SciELO Preprints] - Intervention with ivermectin for of COVID-19: synopsis of evidence
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Person, Osmar Clayton Puga, Maria Eduarda dos Santos Atallah, Alvaro Nagib Amaral, José Luiz Gomes do

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Data de publicação: 2021-03-01

Resumo: En Pt

Context: COVID-19 is a viral disease that has recently emerged and is associated with severe respiratory distress syndrome (SARS). Its potential for severity and impact on the health of the population and the global economy is a prime object in the search for effective therapy and ivermectin has been recommended for prevention and treatment. Objective: To evaluate the evidence in the literature regarding the use of ivermectin for the prevention and treatment of cases of COVID-19. Study design: This is a synopsis of evidence. Methods: We searched the electronic databases PubMed (1966-2021), EMBASE (1974-2021) and Clinical Trials (2021) and two evidence megabusers: Turning Research Into Practice (TRIP) database (2021) and Epstemonikos (2021). There was no geographic or language restriction, using DeCS descriptors and terms (Health Sciences Descriptors). The synthesis method involved the combination of similar studies in a narrative review. Results: 527 citations were identified and 5 studies were included. There are few completed clinical trials, all of which have a small sample size. Discussion: Most of the studies available in the literature are based on in vitro therapeutic responses and the recommendation for use in humans has been based on the findings of these studies. The question cannot be answered with current studies, and quality clinical trials are recommended. Conclusions: There is currently no support in the literature for the use of ivermectin in the prevention or treatment of COVID-19.

Contexto: A COVID-19 é uma doença viral que surgiu recentemente e associada à síndrome da angústia respiratória severa (SARS). Seu potencial de gravidade e impacto na saúde da população e economia global é objeto primordial na busca por uma terapêutica eficaz ea ivermectina tem sido recomendada para prevenção e tratamento da COVID-19. Objetivo: avaliar como evidências na literatura relativa ao uso de ivermectina para prevenção e tratamento de casos de COVID-19. Desenho de estudo: Trata-se de sinopse de evidências. Métodos:Procedeu-se à busca de bases eletrônicas de dados PubMed (1966-2021), EMBASE (1974-2021) e Clinical Trials (2021) e em dois megabuscadores de evidências: Turning Research Into Practice (TRIP) database (2021) e Epstemonikos ( 2021). Não houve restrição geográfica e de idioma, sendo utilizados descritores e termos do DeCS (Descritores em Ciências da Saúde). O método de síntese envolveu uma combinação de estudos semelhantes em uma revisão narrativa. Resultados: Foram identificados 527 citações e 5 estudos foram incluídos. Há poucos ensaios clínicos concluídos todos apresentam pequena. Discussão: A maioria dos estudos disponíveis na literatura respalda-se em respostas terapêuticas in vitroea recomendação para uso em humanos tem-se baseado nos achados desses estudos. A questão não pode ser respondida com os estudos atuais, sendo recomendada a realização de ensaios de qualidade. Conclusões: Não há suporte atualmente na literatura para uso da ivermectina na prevenção ou tratamento COVID-19.

https://doi.org/10.1590/SciELOPreprints.1871

Web of Science Journal Citation Report 2020: the Brazilian contribution to the “Medicine, General & Internal” category of the journal impact factor (JIF) ranking (SCI 2019)
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Atallah, Álvaro Nagib

; Puga, Maria Eduarda dos Santos

; Amaral, José Luiz Gomes do

Sao Paulo Medical Journal

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Ago 2020, Volume 138 Nº 4 Páginas 271 - 274

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1590/1516-3180.2020.138419092020 1085 downloads

10.

Anesthesia for muscle biopsy to test susceptibility to malignant hyperthermia
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Silva, Helga Cristina Almeida da

; Onari, Elton Shinji

; Castro, Isac de

; Perez, Marcelo Vaz

; Hortensi, Alexandre

; Amaral, José Luiz Gomes do

Revista Brasileira de Anestesiologia

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Ago 2019, Volume 69 Nº 4 Páginas 335 - 341

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Resumo Introdução Hipertermia maligna é uma doença farmacogenética autossômica dominante, caracterizada por crise hipermetabólica desencadeada por anestésicos halogenados e/ou succinilcolina. O padrão para diagnóstico da suscetibilidade à hipertermia maligna é o teste de contratura muscular in vitro em resposta ao halotano-cafeína, para o qual é necessária biopsia muscular sob anestesia. Descrevemos uma série de anestesias sem agentes desencadeantes na hipertermia maligna e comparamos bloqueios de nervo periférico e anestesias subaracnóideas. Método Foram analisados os prontuários/fichas anestésicas de 69 pacientes suspeitos de susceptibilidade à hipertermia maligna, submetidos à biópsia muscular para teste de contratura muscular in vitro durante sete anos. Analisamos dados demográficos, indicação para investigação de hipertermia maligna, resultado do teste de contratura muscular in vitro e dados da cirurgia/anestesia/recuperação. Resultados Amostra com 34 ± 13,7 anos, 60,9% mulheres, 65,2% de teste de contratura muscular in vitro positivos. Técnicas empregadas: 47,8% bloqueios de nervo periférico (femoral e cutâneo femoral lateral, latência 65 ± 41 minutos), 49,3% anestesias subaracnóideas e 1,4% anestesia venosa total. Falha em 39,4% dos bloqueios de nervo periférico e 11,8% das anestesias subaracnóideas. Eventos adversos (8,7%) como bradicardia, náuseas e síndrome neurológica transitória só ocorreram com bloqueio subaracnóideo. Todos os pacientes permaneceram na sala de recuperação pós-anestésica até liberação. Idade e peso foram significativamente maiores nos pacientes com falha no bloqueio (ponto de corte da curva ROC de 23,5 anos e 59,5 Kg) e esta foi mais frequente na presença de aumento idiopático de creatinoquinase. Conclusão Anestesia com agentes não desencadeantes mostrou-se segura em pacientes suscetíveis à hipertermia maligna. Variáveis como idade, peso e antecedente de aumento idiopático de creatinoquinase podem ser úteis para selecionar a técnica anestésica nesse grupo.

Abstract Introduction Malignant hyperthermia is an autosomal dominant pharmacogenetic disorder, characterized by hypermetabolic crisis triggered by halogenated anesthetics and/or succinylcholine. The standard method for diagnosing malignant hyperthermia susceptibility is the in vitro muscle contracture test in response to halothane-caffeine, which requires muscle biopsy under anesthesia. We describe a series of anesthetic procedures without triggering agents in malignant hyperthermia, comparing peripheral nerve block and subarachnoid anesthesia. Method We assessed the anesthetic record charts of 69 patients suspected of malignant hyperthermia susceptibility who underwent muscle biopsy for in vitro muscle contracture in the period of 7 years. Demographic data, indication for malignant hyperthermia investigation, in vitro muscle contracture test results, and surgery/anesthesia/recovery data were analyzed. Results Sample with 34 ± 13.7 years, 60.9% women, 65.2% of in vitro muscle contracture test positive. Techniques used: peripheral nerve blocks — lateral femoral and femoral cutaneous, latency 65 ± 41 min — (47.8%); subarachnoid anesthesia (49.3%), and total venous anesthesia (1.4%). There was 39.4% failure of peripheral nerve block and 11.8% of subarachnoid anesthesia. Adverse events (8.7%) occurred only with subarachnoid blockade (bradycardia, nausea, and transient neurological syndrome). All patients remained in the post-anesthesia care unit until discharge. Age and weight were significantly higher in patients with blockade failure (ROC cut-off point of 23.5 years and 59.5 kg) and blockade failure was more frequent in the presence of increased idiopathic creatine kinase. Conclusion Anesthesia with non-triggering agents has been shown to be safe in patients with malignant hyperthermia susceptibility. Variables such as age, weight, and history of increased idiopathic creatine kinase may be useful in selecting the anesthetic technique for this group of patients.

https://doi.org/10.1016/j.bjane.2019.02.003 1221 downloads

11.

Profile of malignant hyperthermia susceptibility reports confirmed with muscular contracture test in Brazil
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Silva, Helga Cristina Almeida da

; Ferreira, Gisele

; Rodrigues, Gislene

; Santos, Joilson Moura dos

; Andrade, Pamela Vieira

; Hortense, Alexandre

; Perez, Marcelo Vaz

; Amaral, José Luiz Gomes do

Revista Brasileira de Anestesiologia

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Abr 2019, Volume 69 Nº 2 Páginas 152 - 159

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Resumo Justificativa e objetivo: Hipertermia maligna é uma síndrome farmacogenética hipermetabólica, autossômica dominante, com mortalidade entre 10%-20%, desencadeada por uso de anestésico inalatório halogenado ou relaxante muscular succinilcolina. O padrão-ouro para pesquisa de suscetibilidade à hipertermia maligna é o teste de contratura muscular in vitro em resposta ao halotano e à cafeína. A determinação da suscetibilidade nas famílias suspeitas permite planejar anestesias seguras sem agentes desencadeantes para os pacientes confirmados como suscetíveis à hipertermia maligna pelo teste de contratura muscular in vitro positivo. Além disso, o paciente no qual a suspeita de hipertermia maligna foi excluída pelo teste de contratura muscular in vitro negativo pode ser anestesiado de forma convencional. Suscetibilidade à hipertermia maligna tem manifestação variável, desde indivíduo assintomático que apresenta crise de hipertermia maligna durante anestesia com agentes desencadeantes, até paciente com atrofia e fraqueza muscular por miopatia central core disease. O objetivo deste trabalho é analisar o perfil dos relatos de suscetibilidade à hipertermia maligna confirmados com teste de contratura muscular in vitro. Método: Análise das fichas de notificação dos pacientes com suspeita pessoal/familiar de hipertermia maligna investigados com teste de contratura muscular in vitro, após assinatura do termo de consentimento, entre 1997-2010. Resultados: Dos 50 eventos que motivaram a suspeita de hipertermia maligna e a investigação familiar (amostra com 27 ± 18 anos, 52% homens, 76% brancos), 64% foram investigados por crise de hipertermia maligna anestésica, com mortalidade de 25%. Sinais mais comuns da crise de hipertermia maligna foram hipertermia, taquicardia e rigidez muscular. Suscetibilidade à hipertermia maligna foi confirmada em 79,4% dos 92 parentes investigados com teste de contratura muscular in vitro. Conclusão: Crises de hipertermia maligna assemelharam-se às descritas em outros países, porém com frequência inferior à estimada no país.

Abstract Background and objectives: Malignant hyperthermia is an autosomal dominant hypermetabolic pharmacogenetic syndrome, with a mortality rate of 10%–20%, which is triggered by the use of halogenated inhaled anesthetics or muscle relaxant succinylcholine. The gold standard for suspected susceptibility to malignant hyperthermia is the in vitro muscle contracture test in response to halothane and caffeine. The determination of susceptibility in suspected families allows the planning of safe anesthesia without triggering agents for patients with known susceptibility to malignant hyperthermia by positive in vitro muscle contracture test. Moreover, the patient whose suspicion of malignant hyperthermia was excluded by the in vitro negative muscle contracture test may undergo standard anesthesia. Susceptibility to malignant hyperthermia has a variable manifestation ranging from an asymptomatic subject presenting a crisis of malignant hyperthermia during anesthesia with triggering agents to a patient with atrophy and muscle weakness due to central core myopathy. The aim of this study is to analyze the profile of reports of susceptibility to malignant hyperthermia confirmed with in vitro muscle contracture test. Method: Analysis of the medical records of patients with personal/family suspicion of malignant hyperthermia investigated with in vitro muscle contracture test, after given written informed consent, between 1997 and 2010. Results: Of the 50 events that motivated the suspicion of malignant hyperthermia and family investigation (sample aged 27 ± 18 years, 52% men, 76% white), 64% were investigated for an anesthetic malignant hyperthermia crisis, with mortality rate of 25%. The most common signs of a malignant hyperthermia crisis were hyperthermia, tachycardia, and muscle stiffness. Susceptibility to malignant hyperthermia was confirmed in 79.4% of the 92 relatives investigated with the in vitro muscle contracture test. Conclusion: The crises of malignant hyperthermia resembled those described in other countries, but with frequency lower than that estimated in the country.

https://doi.org/10.1016/j.bjane.2018.09.009 1887 downloads

12.

Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy
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Silva, Helga Cristina Almeida da

; Hiray, Marcia

; Vainzof, Mariz

; Schmidt, Beny

; Oliveira, Acary Souza Bulle

; Amaral, José Luiz Gomes do

Revista Brasileira de Anestesiologia

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Ago 2018, Volume 68 Nº 4 Páginas 404 - 407

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Resumo Justificativa/objetivos Distrofia muscular de Duchenne/Becker afeta a musculatura esquelética e leva a fraqueza muscular progressiva e risco de reações atípicas anestésicas após exposição à succinilcolina ou halogenados. O objetivo do presente relato é descrever investigação e diagnóstico de paciente com distrofia muscular de Becker e revisar os cuidados necessários na anestesia. Relato de caso Paciente masculino, 14 anos, encaminhado por hiperCKemia (aumento crônico dos níveis séricos de creatinoquinase - CK), com valores de CK de 7.779-29.040 UI.L-1 (normal 174 UI.L-1). Apresentou discreto atraso da aquisição de marcos motores (sentou aos nove meses, andou aos 18). Antecedente de transplante hepático. No exame neurológico apresentava dificuldade para andar nos calcanhares, levantar miopático (apoiava mãos nas coxas para ficar de pé), palato arqueado alto, hipertrofia de panturrilhas, escápulas aladas, hipotonia muscular global e arreflexia. Havia insuficiência respiratória restritiva leve na espirometria (capacidade vital forçada: 77% do previsto). O teste de contratura muscular in vitro em resposta ao halotano e à cafeína foi normal. Estudo da distrofina muscular por técnica de Western blot mostrou redução da distrofina (20% do normal) para ambos os anticorpos (C e N-terminal), e permitiu o diagnóstico de distrofia muscular de Becker. Conclusão Na avaliação pré-anestésica, história de atraso do desenvolvimento motor, bem como sinais clínicos e/ou laboratoriais de miopatia, deve motivar avaliação neurológica, com o objetivo de diagnosticar miopatias subclínicas e planejar cuidados necessários para prevenir complicações anestésicas. Distrofia muscular de Duchenne/Becker, apesar de não conferir suscetibilidade aumentada à HM, pode levar a reações atípicas fatais na anestesia.

Abstract Background and objectives Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. Case report Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels - CK), with CK values of 7,779-29,040 IU.L-1 (normal 174 IU.L-1). He presented with a discrete delay in motor milestones acquisition (sitting at 9 months, walking at 18 months). He had a history of liver transplantation. In the neurological examination, the patient showed difficulty in walking on one's heels, myopathic sign (hands supported on the thighs to stand), high arched palate, calf hypertrophy, winged scapulae, global muscle hypotonia and arreflexia. Spirometry showed mild restrictive respiratory insufficiency (forced vital capacity: 77% of predicted). The in vitro muscle contracture test in response to halothane and caffeine was normal. Muscular dystrophy analysis by Western blot showed reduced dystrophin (20% of normal) for both antibodies (C and N-terminal), allowing the diagnosis of Becker muscular dystrophy. Conclusion On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia.

https://doi.org/10.1016/j.bjane.2017.06.001 2432 downloads

13.

Pre-clinical interaction of ayahuasca, a brew used in spiritual movements, with morphine and propofol
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Pires, Júlia Movilla

; Mendes, Fúlvio Rieli

; Pires, Ana Paula Salum

; Yonamine, Maurício

; Amaral, José Luiz Gomes do

; Carlini, Elisaldo Araújo

Brazilian Journal of Pharmaceutical Sciences

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2018, Volume 54 Nº 1 elocation e17174

Resumo: En | Texto: En | PDF: En

ABSTRACT Ayahuasca is a beverage with psychoactive properties used in religious and ceremonial rituals by some religious groups. The main active components of ayahuasca are dimethyltryptamine and the harmala alkaloids with β-carboline structure acting as monoamine oxidase A inhibitors. This combination produces a pronounced activation of serotonergic pathways and presents potential interaction with other psychotropics. The objective of this study was to investigate the possible interactions between ayahuasca and agents employed in general anesthesia. The pharmacological interactions between ayahuasca and morphine or propofol were evaluated in mice using doses of 12, 120 and 1200 mg/kg (0.1 to 10 times the average dose consumed by humans in religious rituals). Ayahuasca alone showed an antinociceptive effect in the writhing and formalin tests, and intensified the analgesic effect of morphine in the hot plate test. Concerning the pharmacological interactions between ayahuasca and propofol, the results were opposite; ayahuasca intensified the depressant effect of propofol in the rotarod test, but decreased the sleeping time induced by propofol. These set of results showed the occurrence of some interactions between ayahuasca and the drugs morphine and propofol, possibly by both pharmacokinetics and pharmacodynamics mechanisms.

https://doi.org/10.1590/s2175-97902018000117174 2263 downloads

14.

Contração versus contratura e miopatia do núcleo central versus miopatia da parte central em hipertermia maligna
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Silva, Helga Cristina Almeida da

; Andrade, Pamela Vieira de

; Amaral, José Luiz Gomes do

Revista Brasileira de Anestesiologia

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Abr 2014, Volume 64 Nº 2 Páginas 142 - 144

Resumo: Pt En Es | Texto: Pt En Es | PDF: Pt | PDF: En | PDF: Es

https://doi.org/10.1016/j.bjan.2013.06.020 5557 downloads

15.

Hipertermia maligna no Brasil: análise da atividade do hotline em 2009
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Silva, Helga Cristina Almeida da

; Almeida, Clea dos Santos

; Brandão, Julio Cézar Mendes

; Silva, Cleyton Amaral Nogueira e

; Lorenzo, Mariana Elisa Pinto de

; Ferreira, Carolina Baeta Neves Duarte

; Resende, André Hosoi

; Barreira, Sara Rocha

; Almeida, Priscilla Antunes de

; Ferraro, Leonardo Henrique Cunha

; Takeda, Alexandre

; Oliveira, Kátia Ferreira de

; Lelis, Talitha Gonçalez

; Hortense, Alexandre

; Perez, Marcelo Vaz

; Schmidt, Beny

; Oliveira, Acary Souza Bulle

; Amaral, José Luiz Gomes do

Revista Brasileira de Anestesiologia

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Fev 2013, Volume 63 Nº 1 Páginas 20 - 26

Resumo: Pt En Es | Texto: Pt En Es | PDF: Pt | PDF: En | PDF: Es

JUSTIFICATIVA E OBJETIVOS: Hipertermia maligna (HM) é doença farmacogenética, com reação hipermetabólica anormal a anestésicos halogenados e/ou relaxantes musculares despolarizantes. Desde 1991 há um serviço hotline de atendimento telefônico para HM no Brasil, disponível 24 horas por dia, em São Paulo. Este artigo analisa a atividade do serviço brasileiro de hotline para HM em 2009. MÉTODOS: Análise prospectiva de todas chamadas telefônicas paria maligna (HM) é doença farmacogenética, com reação hipermetabólica anormal a anestésicos halogenados e/ou relaxantes musculares despolarizantes. Desde 1991 há um serviço hotline de atendimento telefônico para HM no Brasil, disponível 24 horas por dia, em São Paulo. Este art go analisa a at vidade do serviço brasileiro de hotline para HM em 2009a o serviço brasileiro de hotline para HM, de janeiro a dezembro de 2009. RESULTADOS: Foram recebidas 22 ligações; 21 provenientes do Sul-Sudeste do Brasil e uma do Norte. Quinze eram pedidos de informações gerais sobre HM. Sete foram suspeitas de crises agudas de HM, das quais duas não foram consideradas como HM. Nas cinco crises compatíveis com HM, todos os pacientes receberam anestésicos inalatórios halogenados (2 isoflurano, 3 sevoflurano) e um usou também succinilcolina; havia quatro homens e uma mulher, com média de idade de 18 anos (2-27). Problemas descritos nas cinco crises de HM: taquicardia (cinco), aumento do gás carbônico expirado (quatro), hipertermia (três), acidemia (um), rabdomiólise (um) e mioglobinúria (um). Um paciente recebeu dantrolene. Todos os cinco pacientes com crises de HM foram seguidos em unidade de terapia intensiva e recuperaram-se sem sequelas. A suscetibilidade à HM foi posteriormente confirmada em dois pacientes por meio do teste de contratura muscular in vitro. CONCLUSÕES: O número de chamadas por ano no serviço brasileiro de hotline para HM ainda é reduzido. As características das crises foram similares às descritas em outros países. É preciso aumentar o conhecimento sobre HM no Brasil.

BACKGROUND AND OBJECTIVES: Malignant hyperthermia (MH) is a pharmacogenetic disease that causes abnormal hypermetabolic reaction to halogenated anesthetics and/or depolarizing muscle relaxants. In Brazil, there is a hotline telephone service for MH since 1991, available 24 hours a day in São Paulo. This article analyzes the activity of the Brazilian hotline service for MH in 2009. METHODS: Prospective analysis of all phone calls made to the Brazilian hotline service for MH from January to December 2009. RESULTS: Twenty-two phone calls were received: 21 from the South/Southeast region of Brazil and one from the North region. Fifteen calls were requests for general information about MH. Seven were about suspected MH acute episodes, two of which were not considered as MH. In five episodes compatible with MH, all patients received halogenated volatile anesthetics (2, isoflurane; 3, sevoflurane) and one also used succinylcholine; there were four men and one woman, with a mean age of 18 years (2-27). The problems described in the five MH episodes were tachycardia (5), increased expired carbon dioxide (4), hyperthermia (3), acidemia (1), rhabdomyolysis (1), and myoglobinuria (1). One patient received dantrolene. All five patients with MH episodes were follow-up in the intensive care unit and recovered without sequelae. Susceptibility to MH was later confirmed in two patients by in vitro muscle contracture test. CONCLUSIONS: The number of calls per year in the Brazilian hotline service for MH is still low. The characteristics of MH episode were similar to those reported in other countries. The knowledge of MH in Brazil needs to be increased.

JUSTIFICATIVA Y OBJETIVOS: La Hipertermia Maligna (HM) es una enfermedad farmacogenética, con una reacción hipermetabólica anormal a los anestésicos halogenados y/o relajantes musculares despolarizantes. Desde 1991 existe un servicio hotline de atención telefónica para la HM en Brasil a disposición las 24 horas del día en São Paulo. Este artículo analiza la actividad del servicio brasileño de hotline para la HM en el 2009. MÉTODOS: Análisis prospectivo de todas las llamadas telefónicas realizadas al servicio brasileño de hotline para la HM, desde enero a diciembre de 2009. RESULTADOS: Se recibieron 22 llamadas; 21 provenientes del Sur y Sudeste de Brasil y una del Norte. Quince eran solicitudes de informaciones generales sobre la HM. Siete fueron sobre sospechas de crisis agudas de HM, de las cuales dos no fueron consideradas como HM. En las cinco crisis compatibles con la HM, todos los pacientes recibieron anestésicos inhalatorios halogenados (2 isoflurano, 3 sevoflurano) y uno también usó succinilcolina. Había cuatro hombres y una mujer, con un promedio de edad de 18 años (2-27). Los problemas descritos en las cinco crisis de HM: taquicardia (cinco), aumento del gas carbónico espirado (cuatro), hipertermia (tres), acidemia (uno), rabdomiólisis (uno) y mioglobinuria (uno). Un paciente recibió dantrolene. Todos los cinco pacientes con crisis de HM recibieron acompañamiento en la unidad de cuidados intensivos y se recuperaron sin secuelas. La susceptibilidad a la HM fue posteriormente confirmada en dos pacientes por medio del test de la contractura muscular in vitro. CONCLUSIONES: El número de llamadas por año al servicio brasileño de hotline para la HM todavía es pequeño. Las características de las crisis fueron similares a las descritas en otros países. Es necesario aumentar el conocimiento que se tiene sobre la HM en Brasil.

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