Evaluation of respiratory impairment in patients with systemic lupus erythematosus with the six-minute walk test

AbstractObjectiveEvaluate SLE stable patients, without overt respiratory compromise, by means of 6MWT.Casuistic and methodsForty-five stable SLE patients were enrolled. The ATS/ERS protocol for 6MWT, was used and two parameters with cut-off points were chosen.ResultsForty-two patients were women. The mean age was 39 ± 11.4 years; mean duration of disease, 121 ± 93.1 months; mean value of MRC, 2 ± 0; mean FVC, 85.9 ± 34.2%; mean FEV1, 67.5 ± 21.6%; mean MIP, 82 ± 58.4%; mean MEP, 78 ± 37.3%; mean heart rate at rest, 75 ± 12.8 bpm; mean respiratory rate at rest, 19 ± 5.3 bpm; mean 6MWD, 478 ± 82 m; mean SpO2 at rest was 98 ± 0.8%; mean fall in SpO2, 4 ± 6 points. When the study population was divided according to the 400-m walk distance cut-off value, the heart rate immediately before the test was significant lower in those participants who walked less than 400 m (p = 0.0043), just like the value of Borg scale (p = 0.0036); according to the presence of saturation ≥ 4, heart rate at the end of the test was significantly higher in those participants who were showing desaturation (p = 0.0170); MEP (p = 0.0282) and 6MWD (p = 0.0291) were signifi- cantly lower, and MIP showed a tendency towards being smaller (p = 0.0504). FVC < normal inferior limit was significantly associated with the group with desaturation (p = 0.0274).ConclusionCompared to 6MWD, desaturation was better suited to find the patients with the most compromised indexes in respiratory function tests

Idiopathic Bilateral Diaphragmatic Paresis.

We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis.32481-

Evaluation of pulmonary function in amyotrophic lateral sclerosis holders through forced vital capacityin the seated and supine position

Orientador: Ilma Aparecida PaschoalDissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciências MédicasResumo: Os objetivos deste trabalho foram avaliar as variações da medida da capacidade vital forçada e da ventilação voluntária máxima na mudança de decúbito de sentado para deitado em pacientes com Esclerose Lateral Amiotrófica (ELA) e determinar as pressões inspiratória e expiratória máximas, para medir força muscular respiratória. Participaram do estudo 21 pacientes portadores de ELA, 11 homens e 10 mulheres com idade entre 20 e 74 anos, sem nenhum antecedente de doença respiratória prévia. Dez pacientes apresentavam acometimento de membros superiores e inferiores, 9 pacientes tinham fraqueza muscular de membros superiores, inferiores e de músculos bulbares, 1 paciente só tinha alterações bulbares e 1 paciente só sintomas de membros superiores. Treze pacientes nunca fumaram e 8 tinham antecedentes de tabagismo. (6 ex-fumantes e 2 ainda fumantes). Treze pacientes se queixavam de dispnéia aos esforços. Para verificar a significância estatística das variações da CVF e da VVM foi aplicado o teste não paramétrico de Wilcoxon (para amostras pareadas). Valores de p '< ou =¿ 0.05 indicaram que os valores medidos na posição sentada menos 25%, menos 20% e menos 15% e supina são significativamente diferentes. Neste trabalho, a utilização de 25% como faixa de corte para variação da CVF com o decúbito não produziu resultados significativos.No entanto, faixas de corte de 15% e 20% resultaram em freqüências significativas de variação da CVF com o decúbito. Na avaliação descritiva da PIM, 12 pacientes apresentaram medidas dentro da normalidade prevista e 6 deles atingiram valores menores que 60% do normal. Na medida da PEM, 7 pacientes estavam dentro da referência de normalidade e 11 deles estavam 60% abaixo da normalidade. Concluiu-se que na espirometria houve queda significativa na capacidade vital forçada medida no decúbito supino em 9 pacientes quando o valor de corte foi de 15% e em 8 pacientes quando o valor de corte foi 20%; a utilização do valor de corte de 25% não produziu queda na CVF com freqüência significativa; o limite de 25% a partir do qual a queda da CVF é considerada como causada por fraqueza muscular diafragmática pode ser questionado. Na avaliação de outras variações concomitantes na espirometria a VVM mostrou significantes alterações com o decúbito sendo significativa nos índices de 25%, 20% e 15%. A avaliação da força muscular respiratória através da PIM e PEM revelou maior sequência de alterações nas medidas da Pressão Expiratória Máxima, sugerindo um comprometimento importante da musculatura expiratóriaAbstract: Amyotrophic lateral sclerosis ( ALS ) is a progressive fatal neurodegenerative disorder of unknown origin, characterized by degeneration of espinal motor neurons, motor nuclei of the lower brain stem and upper neurons of the motor cortex. Eventually these lesions may produce respiratory failure. The aim of this study was to assess the variations of the measurements of forced vital capacity and maximum voluntary ventilation in patients with Amyotrophic Lateral Sclerosis when the subjects moved from the sitting to supine position and also to determine the maximum inspiratory and expiratory pressures, in order to evaluate repiratory muscle strength. 21 ALS patients participated of the study ¿ 11 females and 10 males, whose ages ranged from 20 to 74 years. Only patients with an ALS diagnosis confirmed, who undergone a careful clinical evaluation complemented by an electromyographic exam and a nerve conduction study, that texcluded all other neuromuscular diseases, were included. All the patients undergone physiotherapeutic assessment and the Manuvacumeter Recorder (IMEBRAS) with a capacity for 150 cc of water was used to measure the maximum inspiratory (MIP) and expiratory pressures (MEP). Spirometry was performed with the ANAMED AM 4000 PC spirometer. All the patients were measured for the Forced Vital Capacity (FVC), the Forced Expiratory Volume during the first second (FEV 1) and Maximum Voluntary Ventilation (M V V) in sitting and supine position, comparatively. The Wilcoxon non-parametric test was used to check if there was a statistical difference between the variables measured in the sitting position minus 25%, 20% and 15% and the same values measured in the supine position. Values of p '< or =¿ 0.05 indicated that the variables in the sitting position and lying position were significantly different. In this study, the use of 25% as the cut offline for FVC variation with decubitus did not produce significant results. However the cutoff line of 15% and 20% resulted in significant frequencies of FVC variations with decubitus. These results raise the possibility that the 25% decrease that appears in the literature about the subject as indicative of respiratory muscle weakness may not be appropriate. As to the manuvacumeter measurements alterations were observed mainly in the MEPvalues, even before FVC and MIP variations were evident. Conclusion: 1. In the spirometric evaluation there was a significant decrease of the forced vital capacity in the supine decubitus for 9 patients, when the cutoff value used was 15%, and for 8 patients, when the cutoff value was 20%. When the 25% cutoff was used the decrease in CVF was not significantly frequent. 2. The VVM showed significant decubitus alterations. They were significant with the 25%, 20% and 15% cutoff limits. 3. MEP deterioration was more frequent and easier to detect than MIP. The normality indexes of Black and HyattMestradoCiencias BiomedicasMestre em Ciências Médica

Six minute walk test evaluation of pulmonary involvement in scleroderma patients

Orientador: Ilma Aparecida PaschoalTese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciencias MedicasResumo: INTRODUÇÃO - O envolvimento pulmonar é a principal causa de morte relacionada a Esclerose Sistêmica ( ES ). Um teste simples para avaliar a capacidade de exercício é o teste da caminhada de 6 minutos (TC6), e a distância percorrida é usada como desfecho primário em experimentos clínicos. A variação da saturação da hemoglobina (? Sat) durante o TC6 é preditiva de mortalidade nos pacientes com Hipertensão Arterial Pulmonar (HAP). O objetivo deste trabalho foi avaliar a distância percorrida e a queda na saturação (? Sat), no TC6 em pacientes com ES e estabelecer associações entre os resultados do TC6 com outras variáveis clínicas. MÉTODOS - Foram avaliados 110 pacientes com ES. A variação da saturação foi determinada pela diferença entre a saturação de repouso e a saturação ao final dos seis minutos. Foram consideradas como dessaturação variações iguais ou maiores que 4 pontos percentuais. Os dados clínicos e demográficos foram coletados. Todos os pacientes foram submetidos a radiograma de tórax, tomografia computadorizada de alta resolução, teste de função pulmonar, ecocardiograma e pesquisa de marcadores imunológicos no sangue (Scl70 e FAN). RESULTADOS - As variáveis que se associaram com uma distância da caminhada 30 mm Hg e a dessaturação; as variáveis associadas com o ? Sat (p 30 mm Hg e o escore de opacidade reticular e vidro fosco na tomografia computadorizada. Na análise de regressão logística multivariada, três variáveis foram significativas quando testadas com a distância percorrida: idade, raça e dispnéia; e quatro variáveis foram significativas quando testadas com ? Sat: idade, índice de dispnéia, anti Scl 70 positivo e CVF < 80%. CONCLUSÃO - A dessaturação durante o TC6 fornece informação adicional a respeito da doença pulmonar em pacientes com ESAbstract: Six minute walk test evaluation of pulmonary involvement in scleroderma patients Pulmonary involvement is the leading cause of systemic sclerosis (SSc) related deaths. A simple test to evaluate exercise capacity is the 6-minute walk test (6MWT), and the walk distance is increasingly used as a primary outcome in clinical trials. Hemoglobin desaturation during a 6MWT is predictive of mortality in patients with primary pulmonary hipertension. Objectives: To evaluate the walk distance and oxygen desaturation (?sat) during the 6MWT in patients with SSc and to establish correlations between the 6MWT results and other clinical variables. Methods: This study analysed 110 SSc patients who underwent 6MWT. ?sat was defined as a decrease of 4 or more points in saturation between the resting point and the end of the test. Clinical and demographic data was collected. All the patients underwent radiological evaluations (X-rays and HRCT), had pulmonary function tests and echocardiograms performed, and the presence of autoantibodies determined. Results: The variables associated with a walk distance < 400 m (p<0.05) were age, dyspnea index, fibrosis on X-ray, PASP =30 mm Hg, desaturation; the variables associated with ? sat (p<0.05) were age, positive anti-Scl 70, dyspnea index, fibrosis on X-ray, FVC < 80%, PASP= 30 mm Hg, ground-glass or reticular opacities on HRCT. In the multivariate logistic regression analysis, 3 variables were significant when tested with walk distance: age, race and dyspnea index; and 4 variables were significant when tested with ? sat: age, dyspnea index, positive anti-Scl-70 and FVC< 80%. (91) Conclusions: Desaturation during a 6MWT provides additional information regarding severity of disease in scleroderma patients with pulmonary manifestationsDoutoradoClinica MedicaDoutor em Clínica Médic

Cystic Fibrosis In Adults.

The aim of this cross-sectional retrospective study was to describe clinical and functional characteristics and the microbiological profile of an adult population with cystic fibrosis. The study was performed at the Pulmonary Diseases Service of the Hospital of the Medical School of the State University of Campinas. The charts of 54 adults (27 males, 27 females) with cystic fibrosis were reviewed. Demographic, clinical, and microbiological data were collected. Clinical and functional characteristics of patients with chronic Pseudomonas infection were compared with those without it. The mean age of the patients was 41.8 years and the mean sweat chloride concentration was 106.4 mEq/L. Forty-nine percent had chronic respiratory symptoms only after 18 years of age, and 85% reported chronic production of purulent sputum. Six percent had diarrhea or fat in stools, and no patient suffered from diabetes mellitus. Pseudomonas was present in 48%, and 73% of them had the mucoid strain. Others agents found were Staphylococcus aureus (28%), Hemophylus sp. (34%), Aspergillus sp. (22%), Stenotrophomonas maltophilia (9%), Acinetobacter sp. (7%), and Burkholderia cepacia (2%). Nontuberculous mycobacteria were found in sputum from 9 patients (11%); 34 patients (67%) had obstructive ventilatory defect; and 47% had obstruction and restriction. Concerning the groups with and without Pseudomonas colonization, only the values of sweat chloride were significantly higher in chronic colonized patients. Less severe phenotypes of cystic fibrosis may be found among patients previously diagnosed as having bronchiectasis. Chronic colonization with Pseudomonas aeruginosa in this study was not associated with worse clinical status but colonized patients did show significantly higher levels of chloride in sweat.18581-

Avaliação do comprometimento respiratório em pacientes com lúpus eritematoso sistêmico com o teste de caminhada de seis minutos

Objetivo: Avaliar pacientes com LES est&#225;vel, sem comprometimento respirat&#243;rio evidente, por meio do TC6M. Casu&#237;stica e m&#233;todos: Foram recrutados 45 pacientes com LES est&#225;vel. Foi utilizado o protocolo ATS/ERS para TC6M, tendo sido escolhidos dois par&#226;metros com pontos de corte. Resultados: Quarenta e dois dos pacientes eram mulheres.A m&#233;dia de idade foi 39 &#177; 11,4 anos; a dura&#231;&#227;o m&#233;dia da doen&#231;a, 121 &#177; 93,1 meses; valor m&#233;dio de MRC 2 &#177; 0; CVF m&#233;dia 85,9 &#177; 34,2%; VEF1 m&#233;dio 67,5 &#177; 21,6%; PIM m&#233;dia 82 &#177; 58,4%; PEM m&#233;dia 78 &#177; 37,3%; frequ&#234;ncia card&#237;aca m&#233;dia em repouso 75 &#177; 12,8 bpm; frequ&#234;ncia respirat&#243;ria m&#233;dia em repouso 19 &#177; 5,3 bpm; dist&#226;ncia m&#233;dia no TC6M 478 &#177; 82 m; SpO2 m&#233;dia em repouso 98 &#177; 0,8%; queda m&#233;dia em SpO2 4 &#177; 6 pontos. Quando a popula&#231;&#227;o em estudo foi dividida de acordo com o valor de corte de 400 m de dist&#226;ncia caminhada, a frequ&#234;ncia card&#237;aca imediatamente antes do teste foi significativamente menor naqueles participantes que caminharam menos de 400 m (p = 0,0043), da mesma forma que o valor da escala de Borg (p = 0,0036). De acordo com a presen&#231;a de satura&#231;&#227;o &#8805; 4, a frequ&#234;ncia card&#237;aca ao final do teste estava significativamente mais elevada naqueles participantes exibindo dessatura&#231;&#227;o (p = 0,0170); PEM (p = 0,0282) e TC6M (p = 0,0291) estavam significativamente menores e PIM revelou uma tend&#234;ncia para diminuir (p = 0,0504). CVF < limite inferior do normal foi achado significativamente associado com o grupo com dessatura&#231;&#227;o (p = 0,0274). Conclus&#227;o: Comparado com TC6M, a dessatura&#231;&#227;o foi o indicador mais apropriado para localizar os pacientes com os &#237;ndices mais comprometidos nos testes de fun&#231;&#227;o respirat&#243;ria

Paresia diafragmática bilateral idiopática Idiopathic bilateral diaphragmatic paresis

Relata-se o caso de um paciente com dispnéia intensa ao se deitar, em que foram excluídas doenças pulmonares, neuromusculares ou cardíacas, cuja investigação revelou paresia diafragmática bilateral. Um sinal chave para o diagnóstico foi a evidência de respiração paradoxal com o doente em decúbito supino. Havia piora da oxigenação e da capacidade vital forçada com a mudança da posição ortostática para supina. A fluoroscopia ortostática foi normal. A pressão inspiratória máxima estava muito reduzida. A estimulação elétrica transcutânea do diafragma foi normal, e a eletroestimulação do nervo frênico mostrou ausência de resposta, permitindo o diagnóstico de paresia bilateral do diafragma.<br>We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis

Insuficiência respiratória crônica nas doenças neuromusculares: diagnóstico e tratamento Chronic respiratory failure in patients with neuromuscular diseases: diagnosis and treatment

As doenças neuromusculares prejudicam a renovação do ar alveolar e, por esta razão, produzem insuficiência respiratória crônica. A instalação da insuficiência respiratória pode acontecer de modo agudo, como nos traumas, ou ser lenta ou rapidamente progressiva, como na esclerose lateral amiotrófica, distrofias musculares, doença da placa mioneural, etc. O comprometimento da musculatura respiratória prejudica também a eficiência da tosse e, no estado atual da terapêutica disponível no Brasil para estes doentes, pode-se dizer que a morbimortalidade nestes indivíduos está mais associada ao fato de que eles tossem mal do que de que ventilam mal. Nesta revisão, uma breve compilação histórica procura mostrar a evolução das órteses e próteses respiratórias, desde o final do século XIX até agora, com o objetivo de apresentar as opções de máquinas disponíveis para o suporte e substituição da ventilação nas doenças neuromusculares. Além disso, são enfatizados os elementos fundamentais para o diagnóstico da hipoventilação alveolar e da falência do mecanismo protetor da tosse: história clínica, determinação do pico de fluxo da tosse, medida da pressão expiratória máxima e da pressão inspiratória máxima, espirometria em dois decúbitos (sentado e supino), oximetria de pulso, capnografia e polissonografia. São apresentados os valores limites disponíveis na literatura tanto para a indicação do suporte noturno da ventilação como para a extensão do suporte para o período diurno. As manobras para incremento da eficiência da tosse são aqui também discutidas, assim como o momento adequado para sua introdução.<br>Neuromuscular diseases affect alveolar air exchange and therefore cause chronic respiratory failure. The onset of respiratory failure can be acute, as in traumas, or progressive (slow or rapid), as in amyotrophic lateral sclerosis, muscular dystrophies, diseases of the myoneural junction, etc. Respiratory muscle impairment also affects cough efficiency and, according to the current knowledge regarding the type of treatment available in Brazil to these patients, it can be said that the high rates of morbidity and mortality in these individuals are more often related to the fact that they cough inefficiently rather than to the fact that they ventilate poorly. In this review, with the objective of presenting the options of devices available to support and substitute for natural ventilation in patients with neuromuscular diseases, we have compiled a brief history of the evolution of orthopedic braces and prostheses used to aid respiration since the end of the 19th century. In addition, we highlight the elements that are fundamental to the diagnosis of alveolar hypoventilation and of failure of the protective cough mechanism: taking of a clinical history; determination of peak cough flow; measurement of maximal inspiratory and expiratory pressures; spirometry in two positions (sitting and supine); pulse oximetry; capnography; and polysomnography. Furthermore, the threshold values available in the literature for the use of nocturnal ventilatory support and for the extension of this support through the daytime period are presented. Moreover, the maneuvers used to increase cough efficiency, as well as the proper timing of their introduction, are discussed

Measurement of C ⁣PC\!P violation observables in D+→K−K+π+D^+\rightarrow K^-K^+\pi^+ decays

International audienceA search for violation of the charge-parity $C\!P$ symmetry in the $D^+\rightarrow K^-K^+\pi^+$ decay is presented, with proton-proton collision data corresponding to an integrated luminosity of 5.4 fb$^{-1}$, collected at a center-of-mass energy of $13$ TeV with the LHCb detector. A novel model-independent technique is used to compare the $D^+$ and $D^-$ phase-space distributions, with instrumental asymmetries subtracted using the $D^+_{s}\rightarrow K^-K^+\pi^+$ decay as a control channel. The $p$-value for the hypothesis of $C\!P$ conservation is $8.1\%$. The $C\!P$ asymmetry observables $A_{C\!P|S}^{\phi\pi^+} = (0.95 \pm 0.43_{stat} \pm 0.26_{syst})\times 10^{-3}$ and $A_{C\!P|S}^{\overline{K}^{*0}K^+} = (-0.26 \pm 0.56_{ stat} \pm 0.18_{syst})\times 10^{-3}$ are also measured. These results show no evidence of $C\!P$ violation and represent the most sensitive search performed through the phase space of a multibody decay

Measurement of C ⁣PC\!P violation observables in D+→K−K+π+D^+\rightarrow K^-K^+\pi^+ decays

International audienceA search for violation of the charge-parity $C\!P$ symmetry in the $D^+\rightarrow K^-K^+\pi^+$ decay is presented, with proton-proton collision data corresponding to an integrated luminosity of 5.4 fb$^{-1}$, collected at a center-of-mass energy of $13$ TeV with the LHCb detector. A novel model-independent technique is used to compare the $D^+$ and $D^-$ phase-space distributions, with instrumental asymmetries subtracted using the $D^+_{s}\rightarrow K^-K^+\pi^+$ decay as a control channel. The $p$-value for the hypothesis of $C\!P$ conservation is $8.1\%$. The $C\!P$ asymmetry observables $A_{C\!P|S}^{\phi\pi^+} = (0.95 \pm 0.43_{stat} \pm 0.26_{syst})\times 10^{-3}$ and $A_{C\!P|S}^{\overline{K}^{*0}K^+} = (-0.26 \pm 0.56_{ stat} \pm 0.18_{syst})\times 10^{-3}$ are also measured. These results show no evidence of $C\!P$ violation and represent the most sensitive search performed through the phase space of a multibody decay