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1.
PLoS One ; 19(7): e0306789, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39058719

RESUMO

Respiratory diseases significantly affect respiratory function, making them a considerable contributor to global mortality. The respiratory muscles play an important role in disease prognosis; as such, quantitative analysis of the respiratory muscles is crucial to assess the status of the respiratory system and the quality of life in patients. In this study, we aimed to develop an automated approach for the segmentation and classification of three types of respiratory muscles from computed tomography (CT) images using artificial intelligence. With a dataset of approximately 600,000 thoracic CT images from 3,200 individuals, we trained the model using the Attention U-Net architecture, optimized for detailed and focused segmentation. Subsequently, we calculated the volumes and densities from the muscle masks segmented by our model and performed correlation analysis with pulmonary function test (PFT) parameters. The segmentation models for muscle tissue and respiratory muscles obtained dice scores of 0.9823 and 0.9688, respectively. The classification model, achieving a generalized dice score of 0.9900, also demonstrated high accuracy in classifying thoracic region muscle types, as evidenced by its F1 scores: 0.9793 for the pectoralis muscle, 0.9975 for the erector spinae muscle, and 0.9839 for the intercostal muscle. In the correlation analysis, the volume of the respiratory muscles showed a strong correlation with PFT parameters, suggesting that respiratory muscle volume may serve as a potential novel biomarker for respiratory function. Although muscle density showed a weaker correlation with the PFT parameters, it has a potential significance in medical research.


Assuntos
Aprendizado Profundo , Testes de Função Respiratória , Músculos Respiratórios , Tomografia Computadorizada por Raios X , Humanos , Músculos Respiratórios/diagnóstico por imagem , Músculos Respiratórios/fisiologia , Tomografia Computadorizada por Raios X/métodos , Masculino , Feminino , Biomarcadores , Adulto , Pessoa de Meia-Idade , Idoso , Processamento de Imagem Assistida por Computador/métodos
2.
Respiration ; 101(9): 878-892, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35760051

RESUMO

INTRODUCTION: The purpose of this study was to evaluate the potential utility of respiratory muscles ultrasound (US) imaging for assessing respiratory function and identify US variables that best correlate with pulmonary parameters. MATERIALS AND METHODS: A search of 5 databases was conducted. Initially, there was no language, study design, or time frame restrictions. All studies assessing the relationship between pulmonary and US parameters were included. Two reviewers independently extracted and documented data regarding to examined population, age, gender, health condition, methodology, US, and pulmonary function measurements. All studies were qualitative synthesis. RESULTS: A total of 1,272 participants from 31 studies were included. Diaphragm thickness, diaphragm thickening ratio, and diaphragm excursion amplitude were mainly used as US parameters. Forced vital capacity, forced expiratory volume1sec, and maximal inspiratory pressure were mainly used as pulmonary parameters. The relationships between pulmonary and US parameters varied from negligible to strong (depend on examined population and methodology used). Data were not quantitatively synthesis due to high heterogeneity in terms of study design, population examined, and various pulmonary and US parameters. CONCLUSION: A strong relationship between US measurements and pulmonary parameters was demonstrated in some studies but not others. This review confirmed that US measurements can complement spirometry, but the exact role of the US remains to be confirmed. Further studies using standardized methodology are needed to obtain more conclusive evidence on the usefulness of US for assessing respiratory function.


Assuntos
Diafragma , Músculos Respiratórios , Diafragma/fisiologia , Humanos , Pressões Respiratórias Máximas , Músculos Respiratórios/diagnóstico por imagem , Ultrassonografia/métodos , Capacidade Vital
3.
Am J Surg ; 222(5): 1029-1033, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33941359

RESUMO

BACKGROUND: Postoperative respiratory failure (PRF) is a serious complication associated with significant morbidity and mortality. We propose a new method to predict PRF by utilizing computed tomography (CT) of the chest to assess degree of respiratory muscle wasting prior to surgery. METHODS: Patients who received a chest CT and required invasive mechanical ventilation (MV) after major non-cardiothoracic surgery were included. Exclusion criteria included cardiothoracic surgery. Respiratory muscle index (RMI) was calculated at the T6 vertebra measured on Slice-O-Matic® software. RESULTS: Thirty three patients met inclusion with a mean (±SD) age, BMI, and APACHE II score of 62.2 years (±12.1), 28.1 kg/m2 (±7.8), and 14.1 (±4.7). Most patients were female (n = 22 [67%]). Eleven patients (33%) developed PRF with a mean of 6.0 (±10.7) initial ventilation days. There was no difference in baseline demographics between groups. RMI values for the PRF group were significantly lower when compared to the non-PRF group: 22.7 cm2/m2 (±5.3) vs. 28.5 cm2/m2 (±5.9) (p = 0.008). CONCLUSION: Presence of respiratory muscle wasting prior to surgery was found to be associated with postoperative respiratory failure.


Assuntos
Complicações Pós-Operatórias/etiologia , Insuficiência Respiratória/etiologia , Músculos Respiratórios/diagnóstico por imagem , APACHE , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico por imagem , Valor Preditivo dos Testes , Radiografia Torácica , Respiração Artificial/efeitos adversos , Insuficiência Respiratória/diagnóstico por imagem , Músculos Respiratórios/fisiopatologia , Estudos Retrospectivos , Sarcopenia/complicações , Sarcopenia/diagnóstico por imagem , Procedimentos Cirúrgicos Operatórios/efeitos adversos , Tomografia Computadorizada por Raios X
4.
Minerva Anestesiol ; 87(4): 432-438, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33594871

RESUMO

BACKGROUND: The aim of this study was to evaluate whether measurement of diaphragm thickness (DT) by ultrasonography may be a clinically useful noninvasive method for identifying patients at risk of adverse outcomes defined as need of invasive mechanical ventilation or death. METHODS: We prospectively enrolled 77 patients with laboratory-confirmed COVID-19 infection admitted to our intermediate care unit in Pisa between March 5 and March 30, 2020, with follow-up until hospital discharge or death. Logistic regression was used identify variables potentially associated with adverse outcomes and those P<0.10 were entered into a multivariate logistic regression model. Cumulative probability for lack of adverse outcomes in patients with or without low baseline diaphragm muscle mass was calculated with the Kaplan-Meier product-limit estimator. RESULTS: The main findings of this study are that: 1) patients who developed adverse outcomes had thinner diaphragm than those who did not (2.0 vs. 2.2 mm, P=0.001); and 2) DT and lymphocyte count were independent significant predictors of adverse outcomes, with end-expiratory DT being the strongest (ß=-708; OR=0.492; P=0.018). CONCLUSIONS: Diaphragmatic ultrasound may be a valid tool to evaluate the risk of respiratory failure. Evaluating the need of mechanical ventilation treatment should be based not only on PaO2/FiO2, but on a more comprehensive assessment including DT because if the lungs become less compliant a thinner diaphragm, albeit free of intrinsic abnormality, may become exhausted, thus contributing to severe respiratory failure.


Assuntos
COVID-19/diagnóstico por imagem , Pneumonia Viral/diagnóstico por imagem , Músculos Respiratórios/anatomia & histologia , Idoso , Idoso de 80 Anos ou mais , COVID-19/mortalidade , COVID-19/terapia , Estudos de Coortes , Diafragma/anatomia & histologia , Diafragma/patologia , Feminino , Mortalidade Hospitalar , Humanos , Itália/epidemiologia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Pneumonia Viral/mortalidade , Pneumonia Viral/terapia , Valor Preditivo dos Testes , Respiração Artificial/estatística & dados numéricos , Insuficiência Respiratória/diagnóstico por imagem , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/mortalidade , Músculos Respiratórios/diagnóstico por imagem , Resultado do Tratamento , Ultrassonografia
5.
Rheumatology (Oxford) ; 60(1): 250-255, 2021 01 05.
Artigo em Inglês | MEDLINE | ID: mdl-32699895

RESUMO

OBJECTIVE: To investigate the potential contribution of accessory respiratory muscle atrophy to the decline of forced vital capacity (FVC) in patients with SSc-associated interstitial lung disease (ILD). METHODS: This single-centre, retrospective study enrolled 36 patients with SSc-ILD who underwent serial pulmonary function tests and chest high-resolution CT (HRCT) simultaneously at an interval of 1-3 years. The total extent of ILD and chest wall muscle area at the level of the ninth thoracic vertebra on CT images were evaluated by two independent evaluators blinded to the patient information. Changes in the FVC, ILD extent, and chest wall muscle area between the two measurements were assessed in terms of their correlations. Multiple regression analysis was conducted to identify the independent contributors to FVC decline. RESULTS: Interval changes in FVC and total ILD extent were variable among patients, whereas chest wall muscle area decreased significantly with time (P=0.0008). The FVC change was negatively correlated with the change in ILD extent (r=-0.48, P=0.003) and was positively correlated with the change in the chest wall muscle area (r = 0.53, P=0.001). Multivariate analysis revealed that changes in total ILD extent and chest wall muscle area were independent contributors to FVC decline. CONCLUSION: In patients with SSc-ILD, FVC decline is attributable not only to the progression of ILD but also to the atrophy of accessory respiratory muscles. Our findings call attention to the interpretation of FVC changes in patients with SSc-ILD.


Assuntos
Doenças Pulmonares Intersticiais/fisiopatologia , Atrofia Muscular/fisiopatologia , Músculos Respiratórios/patologia , Escleroderma Sistêmico/fisiopatologia , Capacidade Vital , Progressão da Doença , Feminino , Humanos , Músculos Intermediários do Dorso/diagnóstico por imagem , Músculos Intermediários do Dorso/patologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Atrofia Muscular/diagnóstico por imagem , Análise de Regressão , Testes de Função Respiratória , Músculos Respiratórios/diagnóstico por imagem , Estudos Retrospectivos , Escleroderma Sistêmico/complicações , Músculos Superficiais do Dorso/diagnóstico por imagem , Músculos Superficiais do Dorso/patologia , Vértebras Torácicas , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos
6.
Respir Physiol Neurobiol ; 285: 103596, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33301966

RESUMO

Ultrasound imaging is a non-invasive technique to assess organ function. Its potential application in rodents to evaluate respiratory function remains poorly investigated. We aimed to assess and validate ultrasound technique in rats to analyze inspiratory and expiratory muscles. We measured respiratory parameters to provide normal eupneic values. Histological studies and plethysmography were used to validate the technique and assess the physiological implications. A linear relationship was observed between ultrasound and histological data for diaphragm and rectus abdominis (RA) measurement. The tidal volume was significantly correlated with the right + left RA area (r = 0.76, p < 0.001), and the rapid shallow breathing index was significantly and inversely correlated with the right + left RA area (r=-0.53, p < 0.05). In the supine position, the right and left diaphragm expiratory thickness were not associated with tidal volume obtained in the physiological position. Ultrasound imaging is highly accurate and reproducible to assess and follow up diaphragm and RA structure and function in rats.


Assuntos
Reto do Abdome/diagnóstico por imagem , Músculos Respiratórios/diagnóstico por imagem , Volume de Ventilação Pulmonar/fisiologia , Ultrassonografia/normas , Animais , Diafragma/diagnóstico por imagem , Técnicas Histológicas , Masculino , Pletismografia , Ratos , Ratos Sprague-Dawley , Reprodutibilidade dos Testes
7.
Thorax ; 76(3): 295-297, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-32868293

RESUMO

Multiple CT indices are associated with disease progression and mortality in patients with COPD, but which indices have the strongest association remain unestablished. This longitudinal 10-year observational study (n=247) showed that the emphysema severity on CT is more closely associated with the progression of airflow limitation and that a reduction in the cross-sectional area of erector spinae muscles (ESMCSA) on CT is more closely associated with mortality than the other CT indices, independent of patient demographics and pulmonary function. ESMCSA is a useful CT index that is more closely associated with long-term mortality than emphysema and airway disease in patients with COPD.


Assuntos
Remodelação das Vias Aéreas , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Enfisema Pulmonar/diagnóstico , Músculos Respiratórios/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Idoso , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Estudos Prospectivos , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Enfisema Pulmonar/fisiopatologia , Músculos Respiratórios/fisiopatologia , Índice de Gravidade de Doença , Fatores de Tempo
8.
Nagoya J Med Sci ; 82(3): 585-593, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33132442

RESUMO

A 46-year-old Japanese woman visited a nearby hospital because of diplopia after flu-like symptoms. One month later, she presented with blepharoptosis and external ophthalmoplegia. Laboratory tests showed a high creatine kinase concentration (3146 U/L). She underwent intravenous immunoglobulin therapy; however, her symptoms did not improve, prompting transfer to our institute. On admission, transthoracic echocardiography revealed 30% of left ventricular ejection fraction and edema of the left ventricular wall. Coronary angiography showed no significant coronary stenosis. An endomyocardial biopsy resulted in a diagnosis of acute myocarditis. On the following day, she needed a temporary pacemaker because she had complete atrioventricular block and intra-aortic balloon pump because of cardiogenic shock. Intravenous immunoglobulin therapy was again administered and her cardiac function gradually recovered. She was successfully weaned off her temporary pacemaker and intra-aortic balloon pump on Day 5 after improvement in her complete atrioventricular block. Steroid therapy administered from Day 9 was effective in reducing her creatine kinase concentrations. However, contrast-enhanced magnetic resonance imaging revealed inflammation of the scalene, semispinalis cervicis, sternocleidomastoid, and intercostal muscles. On Day 25, her cardiac function had recovered to a left ventricular ejection fraction of 59%. Finally, she was successfully discharged on Day 45 after undergoing rehabilitation.


Assuntos
Miocardite/diagnóstico por imagem , Miosite/diagnóstico por imagem , Angiografia Coronária , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Músculos Respiratórios/diagnóstico por imagem
10.
Br J Anaesth ; 125(1): e148-e157, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32386831

RESUMO

BACKGROUND: Bedside measures of patient effort are essential to properly titrate the level of pressure support ventilation. We investigated whether the tidal swing in oesophageal (ΔPes) and transdiaphragmatic pressure (ΔPdi), and ultrasonographic changes in diaphragm (TFdi) and parasternal intercostal (TFic) thickening are reliable estimates of respiratory effort. The effect of diaphragm dysfunction was also considered. METHODS: Twenty-one critically ill patients were enrolled: age 73 (14) yr, BMI 27 (7) kg m-2, and Pao2/Fio2 33.3 (9.2) kPa. A three-level pressure support trial was performed: baseline, 25% (PS-medium), and 50% reduction (PS-low). We recorded the oesophageal and transdiaphragmatic pressure-time products (PTPs), work of breathing (WOB), and diaphragm and intercostal ultrasonography. Diaphragm dysfunction was defined by the Gilbert index. RESULTS: Pressure support was 9.0 (1.6) cm H2O at baseline, 6.7 (1.3) (PS-medium), and 4.4 (1.0) (PS-low). ΔPes was significantly associated with the oesophageal PTP (R2=0.868; P<0.001) and the WOB (R2=0.683; P<0.001). ΔPdi was significantly associated with the transdiaphragmatic PTP (R2=0.820; P<0.001). TFdi was only weakly correlated with the oesophageal PTP (R2=0.326; P<0.001), and the correlation improved after excluding patients with diaphragm dysfunction (R2=0.887; P<0.001). TFdi was higher and TFic lower in patients without diaphragm dysfunction: 33.6 (18.2)% vs 13.2 (9.2)% and 2.1 (1.7)% vs 12.7 (9.1)%; P<0.0001. CONCLUSIONS: ΔPes and ΔPdi are adequate estimates of inspiratory effort. Diaphragm ultrasonography is a reliable indicator of inspiratory effort in the absence of diaphragm dysfunction. Additional measurement of parasternal intercostal thickening may discriminate a low inspiratory effort or a high effort in the presence of a dysfunctional diaphragm.


Assuntos
Esôfago/fisiologia , Respiração com Pressão Positiva/métodos , Músculos Respiratórios/fisiologia , Ultrassonografia/métodos , Trabalho Respiratório/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Cuidados Críticos/métodos , Estado Terminal , Diafragma/fisiologia , Esôfago/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Respiratórios/diagnóstico por imagem
11.
Respiration ; 99(5): 369-381, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32396905

RESUMO

BACKGROUND: Reference values derived from existing diaphragm ultrasound protocols are inconsistent, and the association between sonographic measures of diaphragm function and volitional tests of respiratory muscle strength is still ambiguous. OBJECTIVE: To propose a standardized and comprehensive protocol for diaphragm ultrasound in order to determine lower limits of normal (LLN) for both diaphragm excursion and thickness in healthy subjects and to explore the association between volitional tests of respiratory muscle strength and diaphragm ultrasound parameters. METHODS: Seventy healthy adult subjects (25 men, 45 women; age 34 ± 13 years) underwent spirometric lung function testing, determination of maximal inspiratory and expiratory pressure along with ultrasound evaluation of diaphragm excursion and thickness during tidal breathing, deep breathing, and maximum voluntary sniff. Excursion data were collected for amplitude and velocity of diaphragm displacement. Diaphragm thickness was measured in the zone of apposition at total lung capacity (TLC) and functional residual capacity (FRC). All participants underwent invasive measurement of transdiaphragmatic pressure (Pdi) during different voluntary breathing maneuvers. RESULTS: Ultrasound data were successfully obtained in all participants (procedure duration 12 ± 3 min). LLNs (defined as the 5th percentile) for diaphragm excursion were as follows: (a) during tidal breathing: 1.2 cm (males; M) and 1.2 cm (females; F) for amplitude, and 0.8 cm/s (M) and 0.8 cm/s (F) for velocity, (b) during maximum voluntary sniff: 2.0 cm (M) and 1.5 cm (F) for amplitude, and 6.7 (M) cm/s and 5.2 cm/s (F) for velocity, and (c) at TLC: 7.9 cm (M) and 6.4 cm (F) for amplitude. LLN for diaphragm thickness was 0.17 cm (M) and 0.15 cm (F) at FRC, and 0.46 cm (M) and 0.35 cm (F) at TLC. Values for males were consistently higher than for females, independent of age. LLN for diaphragmatic thickening ratio was 2.2 with no difference between genders. LLN for invasively measured Pdi during different breathing maneuvers are presented. Voluntary Pdi showed only weak correlation with both diaphragm excursion velocity and amplitude during forced inspiration. CONCLUSIONS: Diaphragm ultrasound is an easy-to-perform and reproducible diagnostic tool for noninvasive assessment of diaphragm excursion and thickness. It supplements but does not replace respiratory muscle strength testing.


Assuntos
Diafragma/diagnóstico por imagem , Força Muscular/fisiologia , Espirometria/métodos , Adulto , Diafragma/fisiologia , Feminino , Capacidade Residual Funcional , Voluntários Saudáveis , Humanos , Masculino , Pressões Respiratórias Máximas/métodos , Pessoa de Meia-Idade , Valores de Referência , Músculos Respiratórios/diagnóstico por imagem , Músculos Respiratórios/fisiologia , Capacidade Pulmonar Total , Ultrassonografia , Adulto Jovem
12.
Am J Speech Lang Pathol ; 29(1): 335-356, 2020 02 07.
Artigo em Inglês | MEDLINE | ID: mdl-31999193

RESUMO

Purpose Expiratory muscle strength training (EMST) is increasingly utilized in dysphagia rehabilitation; however, little is known about the effects of this approach on swallowing function or physiology. We conducted a systematic review to appraise and synthesize evidence regarding the effects of EMST on videofluoroscopic measures of swallowing in individuals with medical diagnoses, in which dysphagia is a concern. Method A literature search was conducted according to Cochrane guidelines. Of 292 nonduplicate articles, 11 were judged to be relevant for review. These underwent detailed review for study quality, risk of bias evaluation, and synthesis of swallowing outcomes. Results The selected articles described EMST in a variety of patient populations using either the EMST150 or the Phillips Threshold positive expiratory pressure device. The typical protocol involved five sets of five breaths through the device (25 breaths/day), 5 days per week for 4 weeks. Exercise loads were set between 50% and 75% depending on the population, and treatment was typically supervised by a clinician weekly. The Penetration-Aspiration Scale was the most commonly reported videofluoroscopic outcome measure. Conclusions differed as to whether or not swallowing improved following a course of EMST. Differences in videofluoroscopy protocols, methods of summarizing participant performance, and statistical approaches across studies meant that meta-analysis of swallowing outcomes could not be completed. Conclusion This review failed to find clear evidence regarding the effects of EMST on videofluoroscopic measures of swallowing. Heterogeneity in the etiologies and baseline severity of dysphagia across studies and in the methods used to measure swallowing outcomes was a particular barrier to data synthesis.


Assuntos
Transtornos de Deglutição/terapia , Deglutição/fisiologia , Treinamento Resistido/métodos , Músculos Respiratórios/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Transtornos de Deglutição/diagnóstico por imagem , Transtornos de Deglutição/etiologia , Fluoroscopia/métodos , Humanos , Pessoa de Meia-Idade , Força Muscular/fisiologia , Músculos Respiratórios/diagnóstico por imagem
13.
Neuromuscul Disord ; 30(1): 17-27, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31839403

RESUMO

The pathophysiology of respiratory muscle weakness in myotonic dystrophy type 1 (DM1) remains incompletely understood. 21 adult patients with DM1 (11 men, 42 ± 13 years) and 21 healthy matched controls underwent spirometry, manometry, and diaphragm ultrasound. In addition, surface electromyography of the diaphragm and the obliquus abdominis muscle was performed following cortical and posterior cervical magnetic stimulation (CMS) of the phrenic nerves or magnetic stimulation of the lower thoracic nerve roots. Magnetic stimulation was combined with invasive recording of the twitch transdiaphragmatic and gastric pressure (twPdi and twPgas) in 10 subjects per group. The following parameters were reduced in DM1 patients compared to control subjects: maximum inspiratory pressure (MIP; 40.3 ± 19.2 vs. 95.8 ± 28.5 cmH2O, p < 0.01), diaphragm thickening ratio (DTR; 2.0 ± 0.4 vs. 2.7 ± 0.6, p < 0.01), twPdi following CMS (10.8 ± 8.3 vs. 21.4 ± 10.1 cmH2O, p = 0.03), and amplitude of diaphragm compound muscle action potentials (0.10 ± 0.25 vs. 0.46 ± 0.35 mV; p = 0.04). MIP and DTR were significantly correlated with the muscular impairment rating scale (MIRS) score. Maximum expiratory pressure (MEP) was reduced in DM1 patients compared to controls (41.3 ± 13.4 vs. 133.8 ± 28.0 cmH2O, p < 0.01) and showed negative correlation with the MIRS score. Pgas following a maximum cough was markedly lower in patients than in controls (71.9 ± 43.2 vs. 102.4 ± 35.5 cmH2O) but without statistical significance (p = 0.06). In DM1, respiratory muscle weakness relates to clinical disease severity and involves inspiratory and probably expiratory muscle strength. Axonal phrenic nerve pathology may contribute to diaphragm dysfunction.


Assuntos
Debilidade Muscular/fisiopatologia , Distrofia Miotônica/fisiopatologia , Nervo Frênico/fisiopatologia , Músculos Respiratórios/fisiopatologia , Adulto , Estudos de Casos e Controles , Estudos Transversais , Diafragma/diagnóstico por imagem , Diafragma/fisiopatologia , Eletromiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Força Muscular/fisiologia , Debilidade Muscular/etiologia , Distrofia Miotônica/complicações , Condução Nervosa/fisiologia , Testes de Função Respiratória , Músculos Respiratórios/diagnóstico por imagem , Índice de Gravidade de Doença , Espirometria , Ultrassonografia
15.
Sci Rep ; 9(1): 19006, 2019 12 12.
Artigo em Inglês | MEDLINE | ID: mdl-31831753

RESUMO

Maximal inspiratory pressure (PIMAX) reflects inspiratory weakness in late-onset Pompe disease (LOPD). However, static pressure tests may not reveal specific respiratory muscle adaptations to disruptions in breathing. We hypothesized that dynamic respiratory muscle functional tests reflect distinct ventilatory compensations in LOPD. We evaluated LOPD (n = 7) and healthy controls (CON, n = 7) during pulmonary function tests, inspiratory endurance testing, dynamic kinematic MRI of the thorax, and ventilatory adjustments to single-breath inspiratory loads (inspiratory load compensation, ILC). We observed significantly lower static and dynamic respiratory function in LOPD. PIMAX, spirometry, endurance time, and maximal diaphragm descent were significantly correlated. During single-breath inspiratory loads, inspiratory time and airflow acceleration increased to preserve volume, and in LOPD, the response magnitudes correlated to maximal chest wall kinematics. The results indicate that changes in diaphragmatic motor function and strength among LOPD subjects could be detected through dynamic respiratory testing. We concluded that neuromuscular function significantly influenced breathing endurance, timing and loading compensations.


Assuntos
Doença de Depósito de Glicogênio Tipo II/fisiopatologia , Músculos Respiratórios/fisiopatologia , Adulto , Idade de Início , Fenômenos Biomecânicos , Estudos de Casos e Controles , Feminino , Doença de Depósito de Glicogênio Tipo II/diagnóstico por imagem , Humanos , Inalação , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Músculos Respiratórios/diagnóstico por imagem , Tórax/diagnóstico por imagem , Fatores de Tempo , Adulto Jovem
16.
J Peripher Nerv Syst ; 24(3): 283-293, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31393643

RESUMO

Diaphragm weakness in Charcot-Marie-Tooth disease 1A (CMT1A) is usually associated with severe disease manifestation. This study comprehensively investigated phrenic nerve conductivity, inspiratory and expiratory muscle function in ambulatory CMT1A patients. Nineteen adults with CMT1A (13 females, 47 ± 12 years) underwent spiromanometry, diaphragm ultrasound, and magnetic stimulation of the phrenic nerves and the lower thoracic nerve roots, with recording of diaphragm compound muscle action potentials (dCMAP, n = 15), transdiaphragmatic and gastric pressures (twPdi and twPgas, n = 12). Diaphragm motor evoked potentials (dMEP, n = 15) were recorded following cortical magnetic stimulation. Patients had not been selected for respiratory complaints. Disease severity was assessed using the CMT Neuropathy Scale version 2 (CMT-NSv2). Healthy control subjects were matched for age, sex, and body mass index. The following parameters were significantly lower in CMT1A patients than in controls (all P < .05): forced vital capacity (91 ± 16 vs 110 ± 15% predicted), maximum inspiratory pressure (68 ± 22 vs 88 ± 29 cmH2 O), maximum expiratory pressure (91 ± 23 vs 123 ± 24 cmH2 O), and peak cough flow (377 ± 135 vs 492 ± 130 L/min). In CMT1A patients, dMEP and dCMAP were delayed. Patients vs controls showed lower diaphragm excursion (5 ± 2 vs 8 ± 2 cm), diaphragm thickening ratio (DTR, 1.9 [1.6-2.2] vs 2.5 [2.1-3.1]), and twPdi (8 ± 6 vs 19 ± 7 cmH2 O; all P < .05). DTR inversely correlated with the CMT-NSv2 score (r = -.59, P = .02). There was no group difference in twPgas following abdominal muscle stimulation. Ambulatory CMT1A patients may show phrenic nerve involvement and reduced respiratory muscle strength. Respiratory muscle weakness can be attributed to diaphragm dysfunction alone. It relates to neurological impairment and likely reflects a disease continuum.


Assuntos
Doença de Charcot-Marie-Tooth/fisiopatologia , Debilidade Muscular/fisiopatologia , Nervo Frênico/fisiopatologia , Músculos Respiratórios/fisiopatologia , Adulto , Doença de Charcot-Marie-Tooth/diagnóstico por imagem , Diafragma/diagnóstico por imagem , Diafragma/fisiopatologia , Estimulação Elétrica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/diagnóstico por imagem , Nervo Frênico/diagnóstico por imagem , Músculos Respiratórios/diagnóstico por imagem , Ultrassonografia
17.
Neuromuscul Disord ; 29(8): 618-627, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31327549

RESUMO

Late-onset Pompe disease (LOPD) causes myopathy of skeletal and respiratory muscles, and phrenic nerve pathology putatively contributes to diaphragm weakness. The aim of this study was to investigate neural contributions to diaphragm dysfunction, usefulness of diaphragm ultrasound, and involvement of expiratory abdominal muscles in LOPD. Thirteen patients with LOPD (7 male, 51±17 years) and 13 age- and gender-matched controls underwent respiratory muscle strength testing, ultrasound evaluation of diaphragm excursion and thickness, cortical and cervical magnetic stimulation (MS) of the diaphragm with simultaneous recording of surface electromyogram and twitch transdiaphragmatic pressure (twPdi; n = 6), and MS of the abdominal muscles with recording of twitch gastric pressure (twPgas; n = 6). The following parameters were significantly reduced in LOPD patients versus controls: forced vital capacity (p<0.01), maximum inspiratory and expiratory pressure (both p<0.001), diaphragm excursion velocity (p<0.05), diaphragm thickening ratio (1.8 ±â€¯0.4 vs. 2.6 ±â€¯0.6, p<0.01), twPdi following cervical MS (12.0 ±â€¯6.2 vs. 19.4 ±â€¯4.8 cmH2O, p<0.05), and twPgas following abdominal muscle stimulation (8.8 ±â€¯8.1 vs. 34.6 ±â€¯17.1 cmH2O, p<0.01). Diaphragm motor evoked potentials and compound muscle action potentials showed no between-group differences. In conclusion, phrenic nerve involvement in LOPD could not be electrophysiologically confirmed. Ultrasound supports assessment of diaphragm function. Abdominal expiratory muscles are functionally involved in LOPD.


Assuntos
Doença de Depósito de Glicogênio Tipo II/fisiopatologia , Debilidade Muscular/diagnóstico , Debilidade Muscular/fisiopatologia , Músculos Respiratórios/fisiopatologia , Adulto , Idade de Início , Idoso , Estudos de Casos e Controles , Estudos Transversais , Diafragma/diagnóstico por imagem , Diafragma/fisiopatologia , Feminino , Doença de Depósito de Glicogênio Tipo II/complicações , Humanos , Campos Magnéticos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Condução Nervosa/fisiologia , Nervo Frênico/fisiologia , Estimulação Física , Músculos Respiratórios/diagnóstico por imagem , Espirometria
18.
J Neurol ; 266(11): 2752-2763, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31350642

RESUMO

OBJECTIVE: Duchenne muscular dystrophy (DMD) is characterized by damage to muscles including the muscles involved in respiration. Dystrophic muscles become weak and infiltrated with fatty tissue, resulting in progressive respiratory impairment. The objective of this study was to assess respiratory muscle quality and function in DMD using magnetic resonance imaging and to determine the relationship to clinical respiratory function. METHODS: Individuals with DMD (n = 36) and unaffected controls (n = 12) participated in this cross sectional magnetic resonance imaging study. Participants underwent dynamic imaging of the thorax to assess diaphragm and chest wall mobility and chemical shift-encoded imaging of the chest and abdomen to determine fatty infiltration of the accessory respiratory muscles. Additionally, clinical pulmonary function measures were obtained. RESULTS: Thoracic cavity area was decreased in individuals with DMD compared to controls during tidal and maximal breathing. Individuals with DMD had reduced chest wall movement in the anterior-posterior direction during maximal inspirations and expirations, but diaphragm descent during maximal inspirations (normalized to height) was only decreased in a subset of individuals with maximal inspiratory pressures less than 60% predicted. Muscle fat fraction was elevated in all three expiratory muscles assessed (p < 0.001), and the degree of fatty infiltration correlated with percent predicted maximal expiratory pressures (r = - 0.70, p < 0.001). The intercostal muscles demonstrated minimal visible fatty infiltration; however, this analysis was qualitative and resolution limited. INTERPRETATION: This magnetic resonance imaging investigation of diaphragm movement, chest wall movement, and accessory respiratory muscle fatty infiltration provides new insights into the relationship between disease progression and clinical respiratory function.


Assuntos
Distrofia Muscular de Duchenne/diagnóstico por imagem , Distrofia Muscular de Duchenne/fisiopatologia , Músculos Respiratórios/diagnóstico por imagem , Estudos Transversais , Diagnóstico por Imagem , Humanos , Imageamento por Ressonância Magnética , Movimento , Músculos Respiratórios/fisiopatologia , Cavidade Torácica/diagnóstico por imagem , Cavidade Torácica/fisiopatologia
19.
Eur Respir J ; 53(6)2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30956204

RESUMO

Assessing respiratory mechanics and muscle function is critical for both clinical practice and research purposes. Several methodological developments over the past two decades have enhanced our understanding of respiratory muscle function and responses to interventions across the spectrum of health and disease. They are especially useful in diagnosing, phenotyping and assessing treatment efficacy in patients with respiratory symptoms and neuromuscular diseases. Considerable research has been undertaken over the past 17 years, since the publication of the previous American Thoracic Society (ATS)/European Respiratory Society (ERS) statement on respiratory muscle testing in 2002. Key advances have been made in the field of mechanics of breathing, respiratory muscle neurophysiology (electromyography, electroencephalography and transcranial magnetic stimulation) and on respiratory muscle imaging (ultrasound, optoelectronic plethysmography and structured light plethysmography). Accordingly, this ERS task force reviewed the field of respiratory muscle testing in health and disease, with particular reference to data obtained since the previous ATS/ERS statement. It summarises the most recent scientific and methodological developments regarding respiratory mechanics and respiratory muscle assessment by addressing the validity, precision, reproducibility, prognostic value and responsiveness to interventions of various methods. A particular emphasis is placed on assessment during exercise, which is a useful condition to stress the respiratory system.


Assuntos
Força Muscular , Mecânica Respiratória , Músculos Respiratórios/diagnóstico por imagem , Músculos Respiratórios/fisiologia , Eletromiografia , Europa (Continente) , Exercício Físico , Humanos , Testes de Função Respiratória , Músculos Respiratórios/anatomia & histologia , Descanso , Sociedades Médicas , Estimulação Magnética Transcraniana
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