[Proposals from a French expert panel for respiratory care in ALS patients]. / Propositions formulées par un groupe d'experts français concernant la prise en charge respiratoire chez les patients atteints de sclérose latérale amyotrophique.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive diaphragm weakness and deteriorating lung function. Bulbar involvement and cough weakness contribute to respiratory morbidity and mortality. ALS-related respiratory failure significantly affects quality of life and is the leading cause of death. Non-invasive ventilation (NIV), which is the main recognized treatment for alleviating the symptoms of respiratory failure, prolongs survival and improves quality of life. However, the optimal timing for the initiation of NIV is still a matter of debate. NIV is a complex intervention. Multiple factors influence the efficacy of NIV and patient adherence. The aim of this work was to develop practical evidence-based advices to standardize the respiratory care of ALS patients in French tertiary care centres. METHODS: For each proposal, a French expert panel systematically searched an indexed bibliography and prepared a written literature review that was then shared and discussed. A combined draft was prepared by the chairman for further discussion. All of the proposals were unanimously approved by the expert panel. RESULTS: The French expert panel updated the criteria for initiating NIV in ALS patients. The most recent criteria were established in 2005. Practical advice for NIV initiation were included and the value of each tool available for NIV monitoring was reviewed. A strategy to optimize NIV parameters was suggested. Revisions were also suggested for the use of mechanically assisted cough devices in ALS patients. CONCLUSION: Our French expert panel proposes an evidence-based review to update the respiratory care recommendations for ALS patients in daily practice.

[Diaphragmatic ultrasonography for the pulmonologist: Technique and clinical use]. / L'échographie diaphragmatique pour le pneumologue : méthodologie et intérêt clinique.

INTRODUCTION: Ultrasonography is an emerging tool that helps to assess diaphragmatic function. It is now widely used in ICUs to predict weaning from mechanical ventilation. Ultrasonography is readily available, harmless (no radiation), and repeatable with good interoperator reproducibility. Over the past few years, ultrasonography has seen increasing use in patients with chronic pulmonary pathologies. STATE OF THE ART: The aim of this review is (1) to describe the ultrasound techniques used to assess diaphragmatic excursion and thickening, (2) to indicate the expected, normal values in healthy patients, and (3) to summarize the main findings and clinical applications in treatment of chronic respiratory disorders. CONCLUSIONS: Chronic pulmonary diseases are associated with diaphragmatic dysfunction that can be assessed with ultrasound. Diaphragmatic dysfunction is primary in neuromuscular disorders and secondary to respiratory disease in other chronic pulmonary conditions (COPD, ILD). Ultrasound is correlated with the severity of the underlying disease (functional and clinical parameters). PERSPECTIVES: The prognostic interest of diaphragm ultrasonography remains to be established, after which its utilization should become routine.

Proposals from a French expert panel for respiratory care in ALS patients.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive diaphragm weakness and deteriorating lung function. Bulbar involvement and cough weakness contribute to respiratory morbidity and mortality. ALS-related respiratory failure significantly affects quality of life and is the leading cause of death. Non-invasive ventilation (NIV), which is the main recognized treatment for alleviating the symptoms of respiratory failure, prolongs survival and improves quality of life. However, the optimal timing for the initiation of NIV is still a matter of debate. NIV is a complex intervention. Multiple factors influence the efficacy of NIV and patient adherence. The aim of this work was to develop practical evidence-based advices to standardize the respiratory care of ALS patients in French tertiary care centres. METHODS: For each proposal, a French expert panel systematically searched an indexed bibliography and prepared a written literature review that was then shared and discussed. A combined draft was prepared by the chairman for further discussion. All of the proposals were unanimously approved by the expert panel. RESULTS: The French expert panel updated the criteria for initiating NIV in ALS patients. The most recent criteria were established in 2005. Practical advice for NIV initiation were included and the value of each tool available for NIV monitoring was reviewed. A strategy to optimize NIV parameters was suggested. Revisions were also suggested for the use of mechanically assisted cough devices in ALS patients. CONCLUSION: Our French expert panel proposes an evidence-based review to update the respiratory care recommendations for ALS patients in daily practice.

Respiratory support in patients with COVID-19 (outside intensive care unit). A position paper of the Respiratory Support and Chronic Care Group of the French Society of Respiratory Diseases.

With first cases noted towards the end of 2019 in China, COVID-19 infection was rapidly become a devastating pandemic. Even if most patients present with a mild to moderate form of the disease, the estimated prevalence of COVID-19-related severe acute respiratory failure (ARF) is 15-20% and 2-12% needed intubation and mechanical ventilation. In addition to mechanical ventilation some other techniques of respiratory support could be used in some forms of COVID-19 related ARF. This position paper of the Respiratory Support and Chronic Care Group of the French Society of Respiratory Diseases is intended to help respiratory clinicians involved in care of COVID-19 pandemic in the rational use of non-invasive techniques such as oxygen therapy, CPAP, non-invasive ventilation and high flow oxygen therapy in managing patients outside intensive care unit (ICU). The aims are: (1) to focus both on the place of each technique and in describing practical tips (types of devices and circuit assemblies) aimed to limit the risk of caregivers when using those techniques at high risk spreading of viral particles; (2) to propose a step-by-step strategy to manage ARF outside ICU.

[Cohort of patients initiated to home ventilation. Observational and prospective study]. / Cohorte de patients nouvellement traités par ventilation non invasive à domicile. Étude observationnelle, prospective et multicentrique.

Cohort of patients initiated to home ventilation. Observational and prospective study. The effectiveness of home noninvasive ventilation (NIV) for chronic respiratory failure (CRF) is well established. However, few data are available about home NIV prescription and utilization according to the different etiologies of respiratory failure. The ANTADIR Federation, in partnership with the Ventilatory Support Group of the French Speaking Pulmonary Society, has set up a national, observational and multicenter cohort study. The main goal of this study is to analyze the clinical data justifying home NIV prescription in patients with chronic respiratory insufficiency. The secondary objectives will be to assess: the evolution of comorbidities or their occurrence, hospitalizations, NIV compliance, dropout and survival. The population includes patients with chronic respiratory failure newly initiated onto NIV, both in a stable state and following an acute exacerbation who qualify for long-term NIV. Data collected include: diagnosis and comorbidities, age, sex, BMI, biomarkers (hematocrit, arterial blood gases, total CO2) and functional data (FEV1, VC, TLC), nocturnal results (SaO2, PtcCO2), type of ventilator used, ventilator parameters and mask type. Follow-up data will be collected at 4 months, 1 year and 2 years and will include: hospitalizations, changes in prescription, adherence, dropouts and deaths. This work will make it possible to obtain new scientific information on long-term NIV use in France.

[Noninvasive ventilation. The 2015 guidelines from the Groupe Assistance Ventilatoire (GAV) of the Société de Pneumologie de Langue Française (SPLF)]. / Ventilation non invasive. Les conseils 2015 du Groupe assistance ventilatoire (GAV) de la Société de pneumologie de langue française (SPLF).

A task force issued from the Groupe Assistance Ventilatoire (GAV) of the Société de Pneumologie de Langue Française (SPLF) was committed to develop a series of expert advice concerning various practical topics related to long-term non invasive ventilation by applying the Choosing Wisely® methodology. Three topics were selected: monitoring of noninvasive ventilation, the interpretation of data obtained from built-in devices coupled to home ventilators and the role of hybrid modes (target volume with variable pressure support. For each topic, the experts have developed practical tips based on a comprehensive analysis of recent insights and evidence from the literature and from clinical experience.

[Noninvasive ventilation: efficacy of a new ventilatory mode in patients with obesity-hypoventilation syndrome]. / Ventilation non invasive : efficacité d'un nouveau mode ventilatoire chez les patients atteints du syndrome obésité-hypoventilation.

Noninvasive ventilation is recommended to correct the nocturnal hypoventilation and relieve the symptoms of patients with the obesity-hypoventilation syndrome (OHS). The benefits of fixed pressure ventilation (S/T technology) are recognized but limited on account of the variability of nocturnal ventilatory requirements. The new technique AVAPS-AE (automatic EPAP) allows adjustment of the pressure according to the volume currently targeted. Its efficacy has not yet been evaluated. Our objectives are to evaluate firstly, whether AVAPS-AE optimizes the benefits of S/T technology on sleep architecture and quality, secondly, whether these benefits are associated with an improvement in gas exchange, symptoms, exercise tolerance, level of physical activity and quality of life of patients with OHS. In this multicenter trial, 60 newly diagnosed patients with OHS will be randomized to the control (S/T) and trial (AVAPS-AE) groups. A standardized titration procedure will be followed for the calibration of the ventilators. Functional evaluations (polysomnography, blood gases, impedance measurements and walking tests), questionnaires (physical activity, quality of life, quality of sleep and daytime somnolence) visual scales (fatigue, headaches) and a recording of activity will be undertaken after two months of ventilation.

[Proposal for a systematic analysis of polygraphy or polysomnography for identifying and scoring abnormal events occurring during non-invasive ventilation]. / Proposition pour une analyse systématique de la polygraphie et de la polysomnographie afin d'identifier les évènements anormaux survenant pendant la ventilation non invasive.

Non-invasive ventilation (NIV) is recognised as an effective treatment for chronic hypercapnic respiratory failure. Monitoring NIV during sleep may be preferable to daytime assessment. This paper reports the findings of an international consensus group, which systematically analysed nocturnal polygraphic or polysomnographic tracings recorded with either volume-cycled or pressure-cycled ventilators. A systematic description of nocturnal respiratory events, which occur during NIV, is proposed: leaks, obstruction at different levels of the upper airway (glottis and/or pharynx), with or without decrease of respiratory drive and asynchrony.

[Ventilator modes and settings during non-invasive ventilation: effects on respiratory events and implications for their identification. 2011]. / Modes ventilatoires et réglages en ventilation non invasive : retentissement sur les évènements respiratoires et implications dans leur identification.

Compared with invasive ventilation, non-invasive ventilation (NIV) has two unique characteristics: its non-hermetic nature and the fact that the ventilator-lung assembly cannot be considered as a single-compartment model because of the presence of variable resistance represented by the upper airways. When NIV is initiated, the ventilator settings are determined empirically based on clinical evaluation and blood gas variations. However, NIV is predominantly applied during sleep. Consequently, to assess overnight patient-machine "agreement" and efficacy of ventilation, more specific and sophisticated monitoring is needed. The effectiveness of NIV might therefore be more correctly assessed by sleep studies than by daytime assessment. The simplest monitoring can be done from flow and pressure curves from the mask or the ventilator circuit. Examination of these tracings can give useful information to evaluate if the settings chosen by the operator were the right ones for that patient. However, as NIV allows a large range of ventilatory parameters and settings, it is mandatory to have information about this to better understand patient-ventilator interaction. Ventilatory modality, mode of triggering, pressurization slope, use or not of positive end expiratory pressure and type of exhalation as well as ventilator performances may all have physiological consequences. Leaks and upper airway resistance variations may, in turn, modify these patterns. This article discusses the equipment available for NIV, analyses the effect of different ventilator modes and settings and of exhalation and connecting circuits on ventilatory traces and gives the background necessary to understand their impact on nocturnal monitoring of NIV.

Proposal for a systematic analysis of polygraphy or polysomnography for identifying and scoring abnormal events occurring during non-invasive ventilation.

Non-invasive ventilation (NIV) is recognised as an effective treatment for chronic hypercapnic respiratory failure. Monitoring NIV during sleep may be preferable to daytime assessment. This paper reports the findings of an international consensus group which systematically analysed nocturnal polygraphic or polysomnographic tracings recorded with either volume-cycled or pressure-cycled ventilators. A systematic description of nocturnal respiratory events which occur during NIV is proposed: leaks, obstruction at different levels of the upper airway (glottis and/or pharynx), with or without decrease of respiratory drive and asynchrony.

[Initiation of long-term non-invasive ventilation at home: current practices and expected issues. Surveys from the CasaVNI working party]. / Mise en route d'une ventilation non invasive : pratiques actuelles et évolutions attendues. Enquêtes du groupe de travail CasaVNI.

INTRODUCTION: At the initiative of the Antadir Federation, the expert group CasaVNI has undertaken a study of the current situation and the medium term outlook of the initiation of long term non-invasive ventilation (NIV) in France and other countries. METHODS: Three surveys have been undertaken: (1) the first concerning the modalities of installation and surveillance of long-term NIV in France, (2) a second concerning the same modalities in other countries, (3) a third concerning the current prevalence and expected incidence of long-term NIV over the next few years. RESULTS: The initiation of long-term NIV takes place in hospital in the majority of cases (France 76%, elsewhere 79%) and the surveillance mainly at a day hospital (France 59%, elsewhere 43%). Despite the interrogation of many sources, the working party has been unable to determine the exact incidence and prevalence of patients receiving long-term NIV in France. CONCLUSION: With regard to the initiation of long-term NIV, clinical studies and guidelines are still necessary to validate the alternatives that will permit a response to the increasing demand while maintaining high standards.

[Initiation of domiciliary non-invasive ventilation: proposals of the Casavni working party]. / Mise en route d'une ventilation non invasive au domicile: propositions du groupe de travail Casavni.

INTRODUCTION: In France, there are no good practice guidelines or a regulatory framework for the initiation of long term non-invasive ventilation (NIV). METHODS: The ANTADIR federation set up a working party to examine the feasibility of new methods of initiation of NIV and to consider the possibility of initiation in the home. Two problems were considered: (1) logistical and technical requirements, (2) the responsibilities of the professions involved and the regulatory framework. RESULTS: Clinical effectiveness, improvement in quality of life and adherence to treatment are similar whether NIV is initiated in hospital or at home. Domiciliary management is possible only when the patient is clinically stable. It should be undertaken by a competent physician or, on medical prescription, by a physiotherapist. A nurse or trained technician may check the apparatus but should not initiate NIV alone. Installation of domiciliary NIV should be considered under the following conditions: patients with spontaneous ventilation, availability of urgent assistance and appropriate family support. Close surveillance of the quality of ventilation is necessary, whether in hospital or at home. For the initiation of domiciliary NIV each professional involved needs a clear understanding of his/her role. CONCLUSION: Academic societies should propose good practice guidelines for the initiation of NIV. Domiciliary initiation is possible under certain conditions and the results are as good as those obtained in hospital.

[The obesity-hypoventilation syndrome]. / Le syndrome obésité-hypoventilation.

Obesity, well-known as a cardiovascular risk factor is also a "respiratory" risk factor and can have profound adverse effects on the respiratory system, such as alterations in pulmonary function tests, respiratory mechanics, respiratory muscle strength and endurance, gas exchange, control of breathing and exercise capacity. ABG are frequently altered in obese subjects and abnormalities are directly proportional to BMI. Two main pathophysiological mechanisms may account for gas exchange abnormalities: V/Q inequality, responsible for isolated hypoxemia, and alveolar hypoventilation responsible for the also called "obesity hypoventilation syndrome" (OHS). Hypoventilation in obese patients includes a diversity of mechanisms frequently imbricated, among which the two most frequent are mechanical limitation and blunted ventilatory drive. Two other clinical entities (COPD and OSA) frequently present in the obese patients may potentiate or aggravate this hypoventilation. OHS is frequently underappreciated and diagnosis is rarely made at the steady state. Such diagnosis is frequently made in two situations: either during an exacerbation or when in front of symptoms of respiratory sleep disturbances. The patient is referred to sleep laboratory for screening for OSA. Ventilatory management of these patients will depend on the patient's underlying condition and on sleep study results. It includes CPAP or NIPPV but frequently additional O(2) addition is necessary. OHS represents today one of the most frequent indications of NIV worldwide.

Evaluating noninvasive ventilation using a monitoring system coupled to a ventilator: a bench-to-bedside study.

Empirically determined noninvasive ventilation (NIV) settings may not achieve optimal ventilatory support. Some ventilators include monitoring modules to assess ventilatory quality. We conducted a bench-to-bedside study to assess the ventilatory quality of the VPAPIII-ResLink (ResMed, North Ryde, Australia). We tested the accuracy of minute ventilation (MV) and leak calculations given by VPAPIII-ResLink compared to those measured by a bench model at varied leak levels and ventilator settings. We systematically assessed NIV efficacy using this system from 2003 to 2006. Ventilation was considered inadequate if leak (>24 L x min(-1)), continuous desaturation (>30% of the trace) or desaturation dips (>3%) were present. On the bench test, both methods were highly correlated (r = 0.947, p>0.0001 and r = 0.959, p<0.0001 for leak and MV, respectively). We performed 222 assessments in 169 patients (aged 66.42+/-16 yrs, 100 males). Abnormalities were detected on 147 (66%) out of 222 occasions. Leak was the most common abnormality (34.2%) followed by desaturation dips (23.8%). The most effective therapeutic solutions were a chin strap if leak was detected (61.2%) and expiratory positive airway pressure increase for desaturation dips (59.5%). In 15.7% of cases, when abnormalities persisted, a polygraphy was performed. The systematic use of this device enables NIV to be optimised, limiting the indication of sleep studies to complex cases.

[Difficult weaning due to tracheal compression by an esophageal bezoar]. / Difficulté de sevrage par compression trachéale due à un bézoard.

INTRODUCTION: Bezoars result from the aggregation of ingested materials (food, drugs, hair) that accumulate at a certain anatomic level in the gastrointestinal tract. It is a rare condition, which is favoured by a reduction in intestinal motility, or by a primary abnormality reducing the patency of gastrointestinal tract. CASE REPORT: We present a case when acute respiratory symptoms revealed an oesophageal bezoar. The patient presented with compression of the posterior tracheal wall by an oesophageal bezoar. The diagnosis was confirmed by oesophageal endoscopy. Treatment consisted in endoscopy-guided fragmentation and removal of the bezoar followed by topical lavage. CONCLUSION: Oesophageal bezoars may account for compression of the posterior tracheal wall causing acute respiratory failure or difficult weaning from the ventilator. The close anatomic proximity between the gastrointestinal and respiratory tract may explain the impact of oesophageal bezoars on the respiratory tract.

[Severe drug rash with eosinophilia and systemic symptoms after treatment with minocycline]. / Syndrome d'hypersensibilité médicamenteuse avec manifestations systémiques sévères après traitement par minocycline.

INTRODUCTION: Lung involvement is rarely observed in the DRESS syndrome (Drug rash with eosinophilia and systemic symptoms). We report here a severe minocycline induced hypersensitivity syndrome with initial respiratory distress. CASE REPORT: A 19 year old man was admitted to the intensive care unit for acute respiratory distress with fever (400C), lymph node enlargement, hepatomegaly, splenomegaly and eosinophilia (1640/mm3). Bilateral alveolar opacities were observed on the chest x-ray. Sedation and mechanical ventilation rapidly became necessary because of severe hypoxaemia (47 mm Hg) and the sudden onset of severe aggressive behaviour. The diagnosis of DRESS was immediately suspected as the patient had been treated for acne with minocycline for 28 days, and IV corticosteroids (2 mmg/kg/day) were initiated. Skin lesions were delayed and appeared 3 days later. The outcome was uncertain for the following 6 weeks with serious disturbance of hepatic and renal function. Serology for human herpes virus (HHV6) was initially negative but became positive. One year later, after progressive withdrawal of corticosteroid therapy, the patient had made a complete recovery with no sequelae. CONCLUSION: The DRESS syndrome can cause considerable morbidity with multiple, severe visceral functional disturbances. Respiratory physicians should be aware of this syndrome as lung involvement can be serious and may precede cutaneous symptoms.

[Leak monitoring in noninvasive ventilation]. / Monitorización de las fugas en ventilación no invasiva.

Nasal mask ventilation has been shown to be effective, but outcomes do not always match expectations because of mouth leaks, patient-ventilator asynchrony, or decreased upper airway patency. These developments are detected when they lead ultimately to circuit leaks that lower the effectiveness of ventilation through pressure loss, poor inspiratory triggering, and prolonged inspiratory time. The quality of sleep is affected, and adverse effects and treatment intolerance may arise. A number of ways to detect leaks and their practical consequences are proposed in this article. We applied 310 leak-detection procedures to 177 patients who had disappointing clinical, gasometric, or polysomnographic outcomes of ventilation. The leak-detection procedures varied according to the type of ventilation and the supposed underlying pathophysiological mechanism. Significant leaks were detected in 132 patients (76%); therapeutic changes were then prescribed to optimize outcomes. We present a practical method to apply in patients with suboptimal ventilation outcomes. If leaks can be detected during treatment, the probable cause of treatment failure can sometimes be established and possible pathophysiological mechanisms better understood. With this knowledge, it may be possible to improve ventilation.

Monitorización de las fugas en ventilación no invasiva / Leak monitoring in noninvasive ventilation

La ventilación por mascarilla nasal ha dado sobradas pruebas de su eficacia. Sin embargo, en ciertos casos los resultados no son los esperados. Tres mecanismos pueden explicar estos fallos: apertura bucal, desincronización paciente-respirador y disminución de la permeabilidad de la vía respiratoria superior. Éstos pueden detectarse por su manifestación última: las fugas en el circuito, que reducen la eficacia de la ventilación (fallo de presurización, disfunción del trigger inspiratorio y prolongación del tiempo inspiratorio), alteran la calidad del sueño y producen efectos adversos e intolerancia al tratamiento. Proponemos aquí varias técnicas de detección de fugas y sus consecuencias prácticas. Se sometió a 177 pacientes, con resultados de la ventilación inferiores a los esperados (clínicos, gasométricos o poligráficos), a 310 procedimientos de detección de fugas, con montajes que variaron según la modalidad ventilatoria y el mecanismo fisiopatológico juzgado como responsable. Se detectaron fugas significativas en 132 pacientes (76 por ciento), lo cual impuso modificaciones terapéuticas para optimizar los resultados. Presentamos un método de aplicación práctica en casos en que se asista a resultados insuficientes de la ventilación. La detección de fugas bajo tratamiento ofrece la posibilidad de establecer la causa probable del fracaso, comprender el mecanismo fisiopatológico potencialmente responsable e intervenir en consecuencia (AU)

[Alveolar hemorrhage associated with intestinal inflammatory disease and Hashimoto thyroiditis]. / Hemorragia alveolar asociada a enfermedad inflamatoria intestinal y tiroiditis de Hashimoto.

Diffuse alveolar hemorrhage (DAH) is characterized by diffuse bleeding into alveolar spaces. Three histopathological patterns may be seen: 1) pulmonary capillaritis due to immunological aggression to the membrane, 2) diffuse alveolar damage within the context of acute respiratory distress syndrome, and 3) and "bland" DAH without alveolar or capillary damage. In the first two groups, pulmonary damage usually occurs within the context of a systemic disease. In the last, injury is usually found only in the lung, an entity called pulmonary hemosiderosis. We present a case of DAH with neither capillaritis nor diffuse alveolar damage in association with inflammatory bowel disease and Hashimoto thyroiditis. The case is interesting both because the association has not yet been described in the literature and because the presence of alveolar bleeding without evident tissue damage within the context of known autoimmune diseases may extend the field to include a new pathophysiological mechanism of pulmonary hemorrhage.