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Introduction: Hemodialysis (HD) populations have a high prevalence of Obstructive Sleep Apnea (OSA), which was specifically linked with fluid overload. HD fluid management targeting a low dry weight was shown to reduce OSA severity, opening to novel therapeutic options. We assessed nephrologists' awareness of OSA diagnosis in HD patients and whether they integrate the current knowledge into their fluid management strategy. Material and methods: We performed a multicenter, cross-sectional study between July 2022 and July 2023, screening all HD patients of four HD units, and included those with confirmed OSA. We collected anthropometric parameters and fluid status from electronic dossiers. Predialysis fluid overload was measured by multifrequency bioelectrical impedance (BCM®). Nephrologists were asked to identify patients with known OSA, without consulting medical dossiers. The fluid management of patients identified as "OSA positive" was compared to that of patients misclassified as "OSA negative". Results: Among 193 HD patients, 23.0% (n=45) had confirmed OSA. The mean age was 76.0 ± 7.5 years, 82.2% were men. Only 60% were correctly identified as "OSA positive" by nephrologists; 14.7% of patients on CPAP were identified. BMI was the only factor associated with correct OSA identification. The predialysis fluid overload tended to be greater in "OSA positive" patients than in the "OSA negative" patients (2.2 ± 1.4 kg vs 1.5 ± 1.3 kg; p=0.08), but there was no difference in postdialysis achievement of dry weight between the groups (residual overweight 0.2 ± 1.0 kg and 0.1 ± 0.7 kg; p= 0.672). Conclusions: Our study suggests that the application of scientific evidence to the management of OSA in dialysis patients is not systematic. However, nephrologists have attempted to strictly achieve dry weight in all patients, regardless of OSA status. Sensibilization of nephrologists on the clinical and diagnostic peculiarities of OSA in HD patients may improve OSA diagnosis and therapeutic care.
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The Swiss Ticino regional pulmonary embolism response team (PERT) features direct access to various pharmacomechanical PE management options within a hub/spoke system, by integrating evidence, guidelines' recommendations and personal experiences. This system involves a collaborative management of patients among the hospitals distributed throughout the region, which refer selected intermediate-high or high PE patients to a second-level hub center, located in Lugano at Cardiocentro Ticino, belonging to the Ente Ospedaliero Cantonale (EOC). The hub provides 24/7 catheterization laboratory activation for catheter-based intervention (CBI), surgical embolectomy and/or a mechanical support system such as extracorporeal membrane oxygenation (ECMO). The hub hosts PE patients after percutaneous or surgical intervention in two intensive care units, one specialized in cardiovascular anesthesiology, to be preferred for patients without relevant comorbidities or with hemodynamic instability and one specialized in post-surgical care, to be preferred for PE patients after trauma or surgery or with relevant comorbidities, such as cancer. From April 2022 to December 2023, a total of 65 patients were referred to the hub for CBI, including ultrasound-assisted catheter-directed thrombolysis (USAT) or large-bore aspiration intervention. No patient received ECMO or underwent surgical embolectomy.
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During the COVID-19 pandemic, percutaneous tracheostomy proved to be an effective option in the management of patients with prolonged periods of intubation. In fact, among other things, it allowed early discharge from ICUs and contributed to reducing overcrowding in intensive care settings, a central and critical point in the COVID pandemic. As a direct consequence, the management and the weaning of frail, tracheostomized and ventilated patients was diverted to sub-intensive or normal hospitalization wards. One central challenge in this setting is the resumption of swallowing and oral feeding, which require interdisciplinary management involving a phoniatrician, ENT, pneumologist, and speech therapist. With this article, we aim to share the experience of a Swiss COVID-19 Center and to draw up a narrative review on the issues concerning the management of the tracheostomy cannula during swallowing resumption, integrating the most recent evidence from the literature with the clinical experiences of the professionals directly involved in the management of tracheostomized COVID-19 patients. In view of the heterogeneity of COVID-19 patients, we believe that the procedures described in the article are applicable to a larger population of patients undergoing tracheostomy weaning.
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COVID-19 , Deglutição , Humanos , Pandemias , Suíça , Traqueostomia/métodosRESUMO
Malnutrition and swallowing disorders are common in Duchenne muscular dystrophy (DMD) patients. We assessed, in adult DMD with home mechanical ventilation (HMV) and cough assist device, its prevalence and the relationships with respiratory muscle strength and long-term respiratory prognosis. We reviewed the patients (n = 117, age 18-39 years [median 24]), followed in a reference center, from 2006 to 2015, to obtain clinical baseline, nutritional status, vital capacity (VC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). The median body mass index (BMI) was low (15.6 kg/m²). Included patients had severe restrictive respiratory function with a median VC of 10.5% [7-17] of the predicted value. All patients were on HMV. Prevalence of malnutrition, swallowing disorders, and gastrostomy were respectively 62%, 34%, and 11%. BMI and serum albumin level were significantly associated with MIP, MEP, and VC. The 1-year/5-years cumulative incidences of respiratory events (pulmonary sepsis and acute respiratory distress) were, respectively, 20.7%/44.5%. Using univariate analysis, predictive factors for respiratory events were swallowing disorders (p = .001), transthyretinemia (p = .034), MIP (p = .039), and MEP (p = .03) but not BMI or albuminemia. Using multivariate analysis, only swallowing disorders remained significantly associated with respiratory events (OR = 4.2, IC 95% 1.31-12.2, p = .01). In conclusion, this study highlights the interrelationships between nutritional intake, swallowing function, airway clearance, and respiratory function in adult DMD. A multidisciplinary approach focusing on these previous factors is essential to optimize DMD patient health.
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Transtornos de Deglutição , Distrofia Muscular de Duchenne , Adolescente , Adulto , Deglutição , Transtornos de Deglutição/epidemiologia , Transtornos de Deglutição/etiologia , Humanos , Distrofia Muscular de Duchenne/complicações , Estado Nutricional , Prognóstico , Músculos Respiratórios , Adulto JovemRESUMO
In neuromuscular disorders (NMDs), nocturnal non-invasive ventilation (NIV) via a nasal mask is offered when hypercapnic respiratory failure occurs. With disease progression, nocturnal NIV needs to be extended into the daytime. Mouthpiece ventilation (MPV) is an option for daytime NIV. MPV represents a difficult task for home ventilators due to rapidly changing load conditions resulting from intermittent connections and disconnections from MPV circuit. The 252nd ENMC International Expert Workshop, held March 6th to 8th 2020 in Amsterdam, reported general guidelines for management of daytime MPV in NMDs. This report could not present all the detail regarding the technical issues important for clinical success of MPV. Based on the expert workshop discussions and the evidence from existing studies, the current narrative review aims to identify the technical issues of MPV and offers guidance via a decisional algorithm and educational figures providing relevant information that is important for successful implementation of MPV.
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Doenças Neuromusculares/complicações , Ventilação não Invasiva/métodos , Insuficiência Respiratória/prevenção & controle , Educação , Humanos , Educação de Pacientes como Assunto , Guias de Prática Clínica como Assunto , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapiaRESUMO
Long-term mechanical ventilation is a well-established treatment for chronic hypercapnic respiratory failure (CHRF). It is aimed at improving CHRF-related symptoms, health-related quality of life, survival, and decreasing hospital admissions. In Switzerland, long-term mechanical ventilation has been increasingly used since the 1980s in hospital and home care settings. Over the years, its application has considerably expanded with accumulating evidence of beneficial effects in a broad range of conditions associated with CHRF. Most frequent indications for long-term mechanical ventilation are chronic obstructive pulmonary disease, obesity hypoventilation syndrome, neuromuscular and chest wall diseases. In the current consensus document, the Special Interest Group of the Swiss Society of Pulmonology reviews the most recent scientific literature on long-term mechanical ventilation and provides recommendations adapted to the particular setting of the Swiss healthcare system with a focus on the practice of non-invasive and invasive home ventilation in adults.
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COVID-19/complicações , Síndrome do Desconforto Respiratório/etiologia , Síndrome do Desconforto Respiratório/terapia , Desmame do Respirador/métodos , Manuseio das Vias Aéreas/métodos , Métodos de Alimentação , Humanos , Pandemias , Síndrome do Desconforto Respiratório/reabilitação , SARS-CoV-2 , Suíça , Traqueotomia/métodos , Traqueotomia/reabilitaçãoRESUMO
Late-onset Pompe disease (LOPD) is an autosomal recessive lysosomal storage disease. Clinical features include skeletal muscle deficiency and diaphragm weakness. Clinical management relies on supportive treatment and mechanical ventilation in patients with chronic respiratory failure. M mode ultrasound and sniff tissue Doppler imaging can be used to assess and follow diaphragm function.
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PURPOSE: The length of time a critically ill coronavirus disease 2019 (COVID-19) patient remains infectious and should therefore be isolated remains unknown. This prospective study was undertaken in critically ill patients to evaluate the reliability of single negative real-time polymerase chain reaction (RT-PCR) in lower tracheal aspirates (LTA) in predicting a second negative test and to analyze clinical factors potentially influencing the viral shedding. METHODS: From April 9, 2020 onwards, intubated COVID-19 patients treated in the intensive care unit were systematically evaluated for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by RT-PCR of nasopharyngeal swabs and LTA. The time to negativity was defined as the time between the onset of symptoms and the viral clearance in LTA. In order to identify risk factors for prolonged viral shedding, we used univariate and multivariate Cox proportional hazards models. RESULTS: Forty-eight intubated SARS-CoV-2 patients were enrolled. Overall, we observed that the association of the first negative RT-PCR with a second negative result was 96.7%. Median viral shedding was 25 (IQR: 21.5-28) days since symptoms' onset. In the univariate Cox model analysis, type 2 diabetes mellitus was associated with a prolonged viral RNA shedding (hazard ratio [HR]: 0.41, 95% CI: 0.06-3.11, p = 0.04). In the multivariate Cox model analysis, type 2 diabetes was associated with a prolonged viral RNA shedding (HR: 0.31, 95% CI: 0.11-0.89, p = 0.029). CONCLUSION: Intubated patients with type 2 diabetes mellitus may have prolonged SARS-CoV-2 shedding. In critically ill COVID-19 patients, one negative LTA should be sufficient to assess and exclude infectivity.
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Betacoronavirus/fisiologia , Infecções por Coronavirus/virologia , Estado Terminal , Diabetes Mellitus Tipo 2/virologia , Pneumonia Viral/virologia , Sistema Respiratório/virologia , Eliminação de Partículas Virais , Idoso , Betacoronavirus/genética , COVID-19 , Teste para COVID-19 , Vacinas contra COVID-19 , Técnicas de Laboratório Clínico/métodos , Técnicas de Laboratório Clínico/normas , Comorbidade , Infecções por Coronavirus/complicações , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/epidemiologia , Progressão da Doença , Feminino , Humanos , Unidades de Terapia Intensiva , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Sistema Respiratório/patologia , Reação em Cadeia da Polimerase Via Transcriptase Reversa/métodos , Reação em Cadeia da Polimerase Via Transcriptase Reversa/normas , Fatores de Risco , SARS-CoV-2 , Manejo de Espécimes/métodos , Manejo de Espécimes/normas , Suíça/epidemiologia , Fatores de TempoRESUMO
BACKGROUND AND PURPOSE: Early-stage Hodgkin lymphoma (HL) is a highly curable disease but the treatment can induce late complications many years later. Irradiation of the healthy heart is inevitable during radiation treatment of mediastinal sites. We developed a novel method to induce a prolonged apnea-like state that can help decrease the dose to organs at risk during radiation therapy. We present the results of the first 8 HL patients treated routinely with percussion assisted radiation therapy (PART) in our clinic. MATERIAL AND METHODS: We used a newly developed high-frequency non-invasive ventilation system to suppress respiratory motion for prolonged periods and push the heart away from the treated volume. RESULTS: All 8 patients were able to rapidly learn the technique and had an advantage to be treated by PART. We lowered the mean heart dose by an average of 3 Gy with similar target coverage compared to a classical free breathing treatment plan. They were all treated for 15 radiotherapy sessions by PART without any notable side effects. CONCLUSIONS: Percussion assisted radiation therapy can be used routinely to reduce the dose to the heart in Hodgkin lymphoma.
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Doença de Hodgkin , Lesões por Radiação , Radioterapia de Intensidade Modulada , Coração , Doença de Hodgkin/radioterapia , Humanos , Órgãos em Risco , Percussão , Lesões por Radiação/etiologia , Lesões por Radiação/prevenção & controle , Dosagem Radioterapêutica , Planejamento da Radioterapia Assistida por ComputadorRESUMO
INTRODUCTION: Patients with chronic pulmonary obstructive disease (COPD) are at risk of acute exacerbation. Diaphragm muscle is classically highly solicited in COPD exacerbation. PATIENT CONCERNS: A COPD patient was admitted because of acute dyspnea with wheezing. DIAGNOSIS: acute COPD exacerbation. INTERVENTIONS: A diaphragm ultrasound and a Doppler echocardiography were performed at bedside. OUTCOMES: We measured diaphragm thickening at the apposition zone and diaphragm inspiratory motion from the subcostal view, in addition with classical echocardiographic parameters. CONCLUSION: Despite a normal diaphragm thickening, diaphragm motion during inspiration is reduced in acute COPD exacerbation. These apparently discrepant findings may be explained by the alterations of the respiratory mechanics during COPD exacerbations, which should be considered when evaluating the diaphragmatic function by imaging.
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Diafragma/anormalidades , Doença Pulmonar Obstrutiva Crônica/complicações , Idoso de 80 Anos ou mais , Diafragma/fisiopatologia , Progressão da Doença , Transtornos Dissociativos , Humanos , Masculino , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Ultrassonografia/métodosRESUMO
Fluid overload has been associated with a high prevalence of sleep apnea (SA) in patients with end-stage kidney disease (ESKD). In this prospective study, we hypothesized that improvement in kidney function and hydration status after kidney transplantation (Tx) may result in an improvement in SA severity. A total of 196 patients on the kidney Tx waiting list were screened for SA using home nocturnal polysomnography (PSG) to measure the apnea-hypopnea index (AHI) and underwent bioimpedance to assess body composition. Of 88 participants (44.9%) with SA (AHI ≥ 15/h), 42 were reassessed 6 months post-Tx and were compared with 27 control patients. There was a significant, but small, post-Tx improvement in AHI (from 44.2 ± 24.3 to 34.7 ± 20.9/h, P = .02) that significantly correlated with a reduction in fluid overload (from 1.8 ± 2.0 to 1.2 ± 1.2 L, P = .02) and body water (from 54.9% to 51.6%, P = .003). A post-Tx increase in body fat mass (from 26% to 30%, P = .003) possibly blunted the beneficial impact of kidney Tx on SA. All parameters remained unchanged in the control group. In conclusion, SA is a frequent condition in ESKD patients and partially improved by kidney Tx. We suggest that SA should be systematically assessed before and after kidney Tx. ClinicalTrials.gov Identifier: NCT02020642.
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Falência Renal Crônica , Transplante de Rim , Síndromes da Apneia do Sono , Humanos , Falência Renal Crônica/cirurgia , Transplante de Rim/efeitos adversos , Polissonografia , Estudos Prospectivos , Síndromes da Apneia do Sono/diagnóstico , Síndromes da Apneia do Sono/epidemiologia , Síndromes da Apneia do Sono/etiologiaRESUMO
Obstructive sleep apnea syndrome (OSAS) and arterial hypertension (HT) are two frequent, often concomitant diseases, who are both associated with an increased cardiovascular risk. In the last years, an association between these two entities has been established. The purpose of this article is to review the current knowledge about the link between HT and OSAS, the pathophysiological mechanisms involved in the common genesis of the two conditions and the characteristics suggesting an underlying OSAS in a hypertensive subject. We will also update readers about the current screening of OSAS in which primary care physicians are directly involved, and review the pros and cons of different treatment options for HT in OSAS.
Le syndrome des apnées obstructives du sommeil (SAOS) et l'hypertension artérielle (HTA) sont deux pathologies fréquentes, souvent concomitantes, qui sont toutes deux associées à un risque cardiovasculaire augmenté. Le but de cet article est de revoir l'état actuel des connaissances sur l'association entre HTA et SAOS, en se basant sur les mécanismes physiopathologiques impliqués dans la genèse commune aux deux conditions. L'article passe en revue les caractéristiques qui devraient faire suspecter un SAOS chez un sujet hypertendu et fait le point sur la démarche de dépistage du SAOS par le médecin traitant et sur les différentes options thérapeutiques à disposition pour le traitement de l'HTA associée au SAOS.
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Hipertensão/complicações , Hipertensão/terapia , Apneia Obstrutiva do Sono/complicações , Apneia Obstrutiva do Sono/diagnóstico , Humanos , Hipertensão/fisiopatologia , Apneia Obstrutiva do Sono/fisiopatologiaRESUMO
The aim of this study was to describe the endotracheal respiratory flora in a population of adults suffering from neuromuscular or neurological disorders requiring a long-term tracheostomy and to identify risk factors for colonization. We conducted a prospective and single-center observational study among patients with chronic tracheostomy admitted for planned respiratory assessment between February 2015 and December 2016. Data were collected from patient interview and medical charts with a standardized questionnaire. A tracheal aspiration was performed for each patient. Humidifiers were analysed for bacteriological contamination. Overall 77 tracheal aspirates (TA) were obtained from patients included. Pathogenic bacteria were found in 90% of cases (69/77) with a majority of Pseudomonas aeruginosa (32/77, 41%), Staphylococcus aureus (34/77, 44%) and Serratia marcesens. (22/79, 38%) Amoxicillin + Clavulanic-acid and Cefotaxime were adapted for respectively in only 28% and 35% of the subjects due to the natural resistance of organisms. No pathogenic bacteria were isolated from humidifier samples. Risk factors significantly associated with P. aeruginosa colonization were residence in a medical-care home (p = 0.01, OR = 3.8 [1.1; 15.1]) and the presence of a cuff (p = 0.003, OR = 4.4 [1.1; 20.6]). Significant quantities of pathogenic bacteria are frequently isolated from TA of tracheostomised patients in the absence of infection. The frequent resistance of these pathogens to Amoxicillin + Clavulanic-acid precludes the use of this antibiotic in the empiric treatment of pneumonia in this population.
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Bactérias/patogenicidade , Infecções Respiratórias/microbiologia , Traqueia/microbiologia , Traqueostomia/efeitos adversos , Adulto , Idoso , Antibacterianos/uso terapêutico , Bactérias/isolamento & purificação , Contagem de Colônia Microbiana , Resistência Microbiana a Medicamentos , Feminino , Hospitalização , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/complicações , Doenças do Sistema Nervoso/epidemiologia , Doenças Neuromusculares/complicações , Doenças Neuromusculares/epidemiologia , Pneumonia/complicações , Pneumonia/diagnóstico , Pneumonia/tratamento farmacológico , Estudos Prospectivos , Pseudomonas aeruginosa/isolamento & purificação , Infecções Respiratórias/epidemiologia , Fatores de Risco , Serratia marcescens/isolamento & purificação , Staphylococcus aureus/isolamento & purificaçãoRESUMO
BACKGROUND: Although ultrashort echo time (UTE) sequences allow excellent assessment of lung parenchyma, image quality remains lower than that of computed tomography (CT). PURPOSE: To investigate a high-frequency noninvasive ventilation (HF-NIV) technique allowing a stabilized inspiration and to compare image quality with current dedicated MR sequences. STUDY TYPE: Prospective. POPULATION: Ten healthy volunteers. FIELD STRENGTH/SEQUENCE: 3D radial UTE sequence at 1.5T. ASSESSMENT: UTE-HF-NIV sequence was compared with UTE-free-breathing (UTE-FB), reconstructed at end expiration (UTE-Exp) and average (UTE-Avg), and breath-hold VIBE sequences. The distance from lung apex to the dome of the right hemidiaphragm was measured. Visual assessment of the visibility and sharpness of normal anatomical structures was carried out. Dedicated software also quantitatively evaluated vessel-lung and right lung-liver interface sharpness. Apparent signal ratio (Sr) and contrast ratios (Cr) were quantitatively evaluated. STATISTICAL TESTS: Wilcoxon signed rank test for visual scores, paired t-test for continuous variables, significance at P < 0.05. RESULTS: The distance between apex and the right hemidiaphragmatic dome was significantly larger (P < 0.001) with UTE-HF-NIV compared with UTE-FB and VIBE acquisitions. Vessel and airway visibility had identical median visual scores with all UTE methods. Median visual scores for sharpness of vessels and airways were significantly higher (P < 0.001) with HF-NIV (vessels = 3; airways = 2) than in UTE-FB (vessels = 2; airways = 1) and VIBE (vessels = 1; airways = 1). Software-based vessel sharpness evaluation resulted in larger values in 8/10 volunteers with UTE-HF-NIV (67.3 ± 9.8) compared with UTE-Avg (62.3 ± 12.6) but the average difference was not significant (P = 0.28). The sharpness of the lung-liver interface was significantly higher (P < 0.001) with HF-NIV (17.3 ± 5.3) compared with UTE-Avg (14.1 ± 3.9). Significantly higher values (P < 0.01) of Sr and Cr were observed with UTE-HF-NIV compared with UTE-FB and VIBE. DATA CONCLUSION: HF-NIV allowing acquisition at full inspiration significantly improves image quality for lung imaging. This could offer the option to alternate some follow-up CT studies by using this technique. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2019;50:1789-1797.
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Processamento de Imagem Assistida por Computador/métodos , Pulmão/fisiologia , Imageamento por Ressonância Magnética/métodos , Ventilação não Invasiva/métodos , Adulto , Feminino , Humanos , Imageamento Tridimensional/métodos , Masculino , Estudos Prospectivos , Valores de Referência , Adulto JovemRESUMO
BACKGROUND: In patients with neuromuscular disorders, assessment of respiratory function relies on forced vital capacity (FVC) measurements. Providing complementary respiratory outcomes may be useful for clinical trials. Diaphragm sniff ultrasound (US) is a noninvasive technique that can assess diaphragm function that may be affected in patients with neuromuscular disorders. PURPOSE: We aimed to provide normal values of sniff diaphragm ultrasound, to assess the relationship between sniff diaphragm US, vital capacity (VC) and sniff nasal pressure. Additionally, we aimed to evaluate the diagnostic accuracy of sniff diaphragm US for predicting restrictive pulmonary insufficiency. MATERIALS AND METHODS: We included patients with neuromuscular disorders that had been tested with a sniff diaphragm US and functional respiratory tests. Healthy subjects were also included to obtain normal diaphragm sniff ultrasound. We performed diaphragm tissue Doppler imaging (TDI) and time movement (TM) diaphragm echography combined with sniff maneuver. RESULTS: A total of 89 patients with neuromuscular diseases and 27 healthy subjects were included in our study. In patients, the median age was 32 years [25; 50] and the median FVC was 34% of predicted [18; 55]. Sniff diaphragm motion using TM ultrasound was significantly associated with sniff nasal pressure, both for the right hemidiaphragm (r = 0.6 p <0.0001) and the left hemidiaphragm (r = 0.63 p = 0.0008). Right sniff peak TDI velocity was also significantly associated with FVC (r = 0.72, p<0.0001) and with sniff nasal pressure (r = 0.66 p<0.0001). Sniff diaphragm ultrasound using either TM mode or TDI displayed significant accuracy for predicting FVC<60% with an area under curve (AUC) reaching 0.93 (p<0.0001) for the right sniff diaphragm ultrasound in TM mode and 0.86 (p<0.001) for right peak diaphragm TDI velocity. CONCLUSION: Sniff diaphragm TM and TDI measures were significantly associated with sniff nasal pressure. Sniff diaphragm TM and TDI had a high level of accuracy to reveal respiratory involvement in patients with neuromuscular disorders. This technique is useful to assess and follow up diaphragm function in patients with neuromuscular disorders. It may be used as a respiratory outcome for clinical trials.
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Diafragma/diagnóstico por imagem , Diafragma/fisiopatologia , Doenças Neuromusculares/diagnóstico por imagem , Doenças Neuromusculares/fisiopatologia , Nariz/fisiopatologia , Pressão , Respiração , Ultrassonografia Doppler , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Movimento , Curva ROC , Volume de Ventilação Pulmonar , Fatores de Tempo , Capacidade VitalRESUMO
RATIONALE: Diaphragm is the main inspiratory respiratory muscle and little is known about diaphragm ultrasound in facio-scapula-humeral muscular dystrophy, a neuromuscular disease characterized by an asymmetric skeletal muscle involvement. PATIENT CONCERNS: Diaphragm function evaluation DIAGNOSIS:: Diaphragm muscle weakness attested by the drop of vital capacity (VC) value from sitting position (74%) to supine position (46%). INTERVENTIONS: A diaphragm ultrasound was performed in supine position, from the anterior subcostal window OUTCOMES:: We found an opposite side to side hemi diaphragm displacement, either during sniff maneuver or during deep inspiration maneuver, showing a cranial abnormal reduced motion of the right hemi diaphragm whereas the left hemi diaphragm moved caudally. LESSONS: Diaphragm weakness may be present with an asymmetric pattern and an opposite motion during inspiration or sniff manoeuver in facio-scapula-humeral muscular dystrophy. A future study with a systematic evaluation of a greater number of FSHD1 patients will be necessary to characterize this population.
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Diafragma/patologia , Distrofia Muscular Facioescapuloumeral/patologia , Diafragma/diagnóstico por imagem , Feminino , Humanos , Pessoa de Meia-Idade , Postura Sentada , Decúbito Dorsal , UltrassonografiaRESUMO
BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic muscle disorder. Respiratory muscle function is classically affected in this disease. Ultrasound recently emerged as a non-invasive tool to assess diaphragm function. However, there are only a few studies using diaphragm ultrasound (US) in DMD. PURPOSE: We aimed to assess diaphragm ultrasound patterns in DMD, their relationship with age and their association with home mechanical ventilation (HMV). METHODS: We included DMD patients followed at Raymond Poincaré Hospital who benefited from diaphragm ultrasound and pulmonary function tests. RESULTS: There were 110 DMD patients and 17 male sex-matched healthy subjects included. In all, 94% of patients were permanent wheelchair users. Median body mass index (BMI) was 18âkg/m2. DMD patients disclosed a reduced forced vital capacity (VC) (12% of predicted value), and 78% of patients were on HMV. In patients, right and left diaphragmatic motions on deep inspiration were reduced and end expiratory diaphragm thickness was borderline normal. In patients, right and left diaphragmatic thickening fractions (TF) were reduced 12.7% and 15.5%, respectively. Age and end expiratory thickness were significantly inversely associated (pâ=â0.005 for the right diaphragm, pâ=â0.018 for the left diaphragm). Diaphragm TF was significantly inversely associated with age (pâ=â0.001 for the right side, pâ<â0.0001 for the left side). Right and left inspiratory diaphragm motions were significantly inversely associated with age (pâ<â0.0001). CONCLUSION: This study describes the severity of diaphragm dysfunction in patients with DMD. Diaphragm US may be a non-invasive outcome measure for DMD.
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Diafragma/diagnóstico por imagem , Distrofia Muscular de Duchenne/diagnóstico por imagem , Ultrassonografia , Adolescente , Adulto , Criança , Estudos Transversais , Diafragma/crescimento & desenvolvimento , Diafragma/patologia , Diafragma/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Distrofia Muscular de Duchenne/fisiopatologia , Distrofia Muscular de Duchenne/terapia , Tamanho do Órgão , Respiração , Respiração Artificial , Estudos Retrospectivos , Adulto JovemRESUMO
STUDY OBJECTIVES: To determine the prevalence and clinical associations of respiratory effort-related arousals (RERA) in a general population sample. METHODS: A total of 2,162 participants (51.2% women, 58.5 ± 11.0 years old, body mass index [BMI] 25.6 ± 4.2 kg/m2) of a general population-based cohort (HypnoLaus, Switzerland) underwent full polysomnography at home. Each subject with a RERA index ≥ 5 events/h was compared with an age-, sex- and apnea-hypopnea index (AHI)-matched control without RERA. RESULTS: A RERA index ≥ 5 events/h was present in 84 participants (3.8%; 95% confidence interval: 3.2-4.8%). In 17 participants (0.8%; 95% confidence interval: 0.5-1.3%), RERAs were the predominant sleep breathing disorder and only one of them complained of excessive daytime sleepiness. Compared to matched controls, subjects with a RERA index ≥ 5 events/h were similar in terms of BMI (26.5 ± 3.5 versus 26.3 ± 4.8 kg/m2, P = .73), neck circumference (38.5 ± 3.3 versus 37.6 ± 3.7 cm, P = .10) and Epworth Sleepiness Scale score (6.7 ± 3.7 versus 6.0 ± 3.7, P = .22). Also, no differences were found for hypertension (21.4% versus 27.4%, P = .47), diabetes (7.1% versus 7.1%, P = 1.00), or metabolic syndrome (31.0% versus 23.8%, P = .39). CONCLUSIONS: In a middle-aged population-based cohort, the prevalence of a RERA index ≥ 5 events/h was low (3.8%) and was not associated with negative clinical outcomes when using the currently recommended scoring criteria of the American Academy of Sleep Medicine.