Teleconsultation in respiratory medicine - A position paper of the Portuguese Pulmonology Society.

The COVID-19 pandemic crisis, among so many social, economic and health problems, also brought new opportunities. The potential of telemedicine to improve health outcomes had already been recognised in the last decades, but the pandemic crisis has accelerated the digital revolution. In 2020, a rapid increase in the use of remote consultations occurred due to the need to reduce attendance and overcrowding in outpatient clinics. However, the benefit of their use extends beyond the pandemic crisis, as an important tool to improve both the efficiency and capacity of future healthcare systems. This article reviews the literature regarding telemedicine and teleconsultation standards and recommendations, collects opinions of Portuguese experts in respiratory medicine and provides guidance in teleconsultation practices for Pulmonologists.

Non-invasive respiratory support paths in hospitalized patients with COVID-19: proposal of an algorithm.

COVID-19 related Acute Respiratory Failure, may be successfully treated with Conventional Oxygen therapy, High Flow Nasal Cannula, Continuous Positive Airway Pressure or Bi-level Positive-Pressure ventilation. Despite the accumulated data in favor of the use of different Non-invasive Respiratory therapies in COVID-19 related Acute Respiratory Failure, it is not fully understood when start, escalate and de-escalate the best respiratory supportive option for the different timing of the disease. Based on the current published experience with Non-invasive Respiratory therapies in COVID-19 related Acute Respiratory Failure, we propose an algorithm in deciding when to start, when to stop and when to wean different NIRT. This strategy may help clinicians in better choosing NIRT during this second COVID-19 wave and beyond.

Continuous noninvasive ventilatory support outcomes for patients with neuromuscular disease: a multicenter data collaboration.

BACKGROUND: Typically, patients with progressive neuromuscular disorders (NMDs) develop acute respiratory failure (ARF), are intubated, and when failing spontaneous breathing trials (SBTs) undergo a tracheotomy and receive tracheostomy mechanical ventilation (TMV). However, increasing numbers of patients use nasal noninvasive ventilation (NIV), initially for sleep and this is extended to continuous dependence (CNVS). This can be used as a strategy to assist in successful extubation . We retrospectively reviewed 19 centers offering CNVS and mechanical insufflation-exsufflation (MI-E) as an alternative to TMV. METHODS: Centers with publications or presentations concerning CNVS outcomes data were pooled for amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy type 1 (SMA1). Progression to CNVS dependence without hospitalization, duration of dependence, and extubations and decannulations to CNVS were recorded. Prolongation of life was defined by duration of CNVS dependence without ventilator free breathing ability (VFBA). RESULTS: There were 1623 part time (<23 h/day) NVS users with ALS, DMD, and SMA1 from 19 centers in 16 countries of whom 761 (47%) were CNVS dependent for 2218 patient-years. This included: 335 ALS patients for a mean 1.2 ± 1.0 (range to 8) years each; 385 DMD patients for 5.4 ± 1.6 (range to 29) years; and 41 SMA1 patients for 5.9 ± 1.8 (range to 20) years. Thirty-five DMD and ALS TMV users were decannulated to CNVS and MI-E. At data collection 494 (65%) patients were CNVS dependent but 110 (74 of whom with bulbar ALS), had undergone tracheotomies. CONCLUSIONS: ALS, DMD, and SMA1 patients can become CNVS dependent without requiring hospitalization but CNVS cannot be used indefinitely for many patients with advanced upper motor neuron diseases.

COVID-19 pandemic and non invasive respiratory management: Every Goliath needs a David. An evidence based evaluation of problems.

BACKGROUND AND AIM: The war against Covid-19 is far from won. This narrative review attempts to describe some problems with the management of Covid-19 induced acute respiratory failure (ARF) by pulmonologists. METHODS: We searched the following databases: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials and reviewed the references of retrieved articles for additional studies. The search was limited to the terms: Covid-19 AND: acute respiratory distress syndrome (ARDS), SARS, MERS, non invasive ventilation (NIV), high flow nasal cannula (HFNC), pronation (PP), health care workers (HCW). RESULTS: Protection of Health care workers should be paramount, so full Personal Protective Equipment and Negative pressure rooms are warranted. HFNC alone or with PP could be offered for mild cases (PaO2/FiO2 between 200-300); NIV alone or with PP may work in moderate cases (PaO2/FiO2 between 100-200). Rotation and coupled (HFNC/NIV) strategy can be beneficial. A window of opportunity of 1-2h is advised. If PaO2/FIO2 significantly increases, Respiratory Rate decreases with a relatively low Exhaled Tidal Volume, the non-invasive strategy could be working and intubation delayed. CONCLUSION: Although there is a role for non-invasive respiratory therapies in the context of COVID-19 ARF, more research is still needed to define the balance of benefits and risks to patients and HCW. Indirectly, non invasive respiratory therapies may be of particular benefit in reducing the risks to healthcare workers by obviating the need for intubation, a potentially highly infectious procedure.

Organization of Home Mechanical Ventilation in Portugal: Characterization of current centers and a pathway to uniformization.

INTRODUCTION: Home Mechanical Ventilation (HMV) is increasing worldwide. OBJECTIVE: Characterization of the Portuguese HMV Units. METHODS: The HMV Team Group of the Portuguese Pulmonology Society prepared a questionnaire that was sent by e-mail addressed to Pneumology Department Directors throughout the country, and the responses were then analyzed. The results enabled a provisional classification of the Units, which followed specific criteria. RESULTS: Thirty centers were surveyed, of which 60% (18) sent the answers to the questionnaire. As for the results obtained, only one center was considered as a basic unit. Most centers (14/18) were considered specialized units. 3/18 centers were classified as highly complex multidisciplinary units. Of the 12 centers that did not answer the questionnaire, one refused to do it and another center was in transition period. CONCLUSIONS: Analysis of the results reveals the high number of patients treated with HMV in Portugal, supports the importance of creating protocols to standardize HMV countrywide, and audit its practice through the creation of a national register.

Tracheostomy prevalence at Skilled Nursing Facilities.

The incidence of chronically ill subjects with prolonged mechanical ventilation has significantly increased over the last decade. Many patients get discharge to Skilled Nursing Facilities with an artificial airway, which do not have the means to properly progress on weaning. In Portugal this prevalence is unknown. Our aim was to establish the prevalence of tracheostomized patients at SNF in the North of Portugal, characterizing these units and its population, in a cross-sectional study, through an online questionnaire answered on the same day. Of the 75 SNF, 30 answered: 13 long-term, 2 medium-term, 2 short-term, 12 had beds of both medium and long-term and 1 had the three typologies. 33 had tracheostomy ventilation (prevalence 3.36%), all admitted at long-term units, the majority transferred from previous hospital admission (n=27, 90%). Only one was under mechanical ventilation. The most frequent reason for tracheostomy placement was acute respiratory failure (n=10, 33.3%). The most commonly presented cannula was the fenestrated non-cuffed (n=17, 59%). Only 4 were performing occlusion training, 21 needed frequent secretion suctioning and 1 used the mechanical in-exsufflation. Regarding motor function, 16 (53.3%) were unable to achieve sitting balance and 20 (66.7%) had no orthostatic balance or walking ability. 14 (46.7%) had percutaneous endoscopic gastrostomy. Although low response rate may induce some bias, this study revealed a significant prevalence of tracheostomized patients at SNF. These facilities do not have the resources to safely and effectively progress on ventilatory weaning. It is essential to establish new referral criteria and create specialized weaning units.

Upper Airway Video Endoscopy: Assessment of the response to positive pressure ventilation and mechanical in-exsufflation.

Upper airways (UA) include the nasal cavities, pharynx, and larynx, and its main function is to warm and filter the inspired air. UA dysfunction is in the pathogenesis of various disorders, such as obstructive sleep apnea syndrome (OSAS) and vocal cord dysfunction. In addition, in some neurodegenerative diseases (e.g. Amyotrophic Lateral Sclerosis - ALS), UA dysfunction may also compromise the effective use of ventilatory support (VS). In this context, the endoscopic evaluation of UA may be useful in understanding the OSAS mechanisms, in determining the causes for treatment-induced airway obstruction and even in helping to titrate noninvasive ventilation (NIV) in ALS patients with bulbar or pseudo-bulbar (spastic) dysfunction. Specifically, in OSAS patients, when residual obstructive events persist, although an optimal ventilatory mode has been apparently achieved, along with interface and equipment, the endoscopic evaluation of UA seems to be a valuable tool in understanding its mechanisms, even assisting adjustments to NIV parameters. In addition, it has also been described as being useful in laryngeal response to mechanical in-exsufflation (MI-E) and Exercise-Induced Laryngeal Obstruction (EILO). However, no protocol has yet been published or validated for this. For this reason, a literature review was conducted on UA function and its response to positive pressure and MI-E. Special emphasis has also been given to the current indication for video endoscopy in chronically ventilated patients.

Sleep bruxism associated with obstructive sleep apnoea syndrome - A pilot study using a new portable device.

Sleep bruxism (SB) and obstructive sleep apnoea syndrome (OSAS) share common pathophysiologic pathways. We aimed to study the presence and relationship of SB in a OSAS population. Patients referred with OSAS suspicion and concomitant SB complains were evaluated using a specific questionnaire, orofacial evaluation and cardio-respiratory polygraphy that could also monitor audio and EMG of the masseter muscles. From 11 patients studied 9 had OSAS. 55.6% were male, mean age was 46.3±11.3 years, and apnea hypopnea index of 11.1±5.7/h. Through specific questionnaire 55.6% had SB criteria. Orofacial examination (only feasible in 3) confirmed tooth wear in all. 77.8% had polygraphic SB criteria (SB index>2/h). Mean SB index was 5.12±3.6/h, phasic events predominated (72.7%). Concerning tooth grinding episodes, we found a mean of 10.7±9.2 per night. All OSAS patients except two (77.8%) had more than two audible tooth-grinding episodes. These two patients were the ones with the lowest SB index (1.0 and 1.4 per hour). Only in one patient could we not detect tooth grinding episodes. There was a statistically significant positive correlation between tooth grinding episodes and SB index and phasic event index (R=0.755, p=0.019 and R=0.737, p=0.023 respectively, Pearson correlation). Mean apnoea to bruxism index was 0.4/h, meaning that only a minority of SB events were not secondary to OSAS. We could not find any significant correlation between AHI and bruxism index or phasic bruxism index (R=-0.632 and R=-0.611, p>0.05, Pearson correlation). This pilot study shows that SB is a very common phenomenon in a group of mild OSAS patients, probably being secondary to it in the majority of cases. The new portable device used may add diagnostic accuracy and help to tailor therapy in this setting.

Prevalence of late-onset pompe disease in Portuguese patients with diaphragmatic paralysis - DIPPER study.

Pompe disease is a rare autosomal recessive neuromuscular disorder caused by acid α-glucosidase enzyme (GAA) deficiency and divided into two distinct variants, infantile- and late-onset. The late-onset variant is characterized by a spectrum of phenotypic variation that may range from asymptomatic, to reduced muscle strength and/or diaphragmatic paralysis. Since muscle strength loss is characteristic of several different conditions, which may also cause diaphragmatic paralysis, a protocol was created to search for the diagnosis of Pompe disease and exclude other possible causes. METHODS: We collected a sample size of 18 patients (10 females, 8 males) with a median age of 60 years and diagnosis of diaphragmatic paralysis of unknown etiology, followed in the Pulmonology outpatient consultation of 9 centers in Portugal, over a 24-month study period. We evaluated data from patient's clinical and demographic characteristics as well as complementary diagnostic tests including blood tests, imaging, neurophysiologic and respiratory function evaluation. All patients were evaluated for GAA activity with DBS (dried blood test) or serum quantification and positive results confirmed by serum quantification and sequencing. RESULTS: Three patients were diagnosed with Pompe's disease and recommended for enzyme replacement therapy. The prevalence of Pompe, a rare disease, in our diaphragmatic paralysis patient sample was 16.8%. CONCLUSION: We conclude that DBS test for GAA activity should be recommended for all patients with diaphragmatic paralysis which, despite looking at all the most common causes, remains of unknown etiology; this would improve both the timing and accuracy of diagnosis for Pompe disease in this patient population. Accurate diagnosis will lead to improved care for this rare, progressively debilitating but treatable neuromuscular disease.

The value of cough peak flow measurements in the assessment of extubation or decannulation readiness.

Insufficient cough strength has a major role in extubation and decannulation outcomes. Cough capacity can be easily evaluated by measuring flows during coughing. Values vary depending on whether cough flows are measured through the mouth or through a tracheostomy or endotracheal tube. It is important to standardize these measurements and start using them routinely in the extubation and decannulation processes. Values of cough peak flow >160 L/min measured at the mouth or a value of cough PEF >60 L/min measured at the endotracheal tube suggest successful decannulation or extubation.

Suberosis and bird fancier's disease: a comparative study of radiological, functional and bronchoalveolar lavage profiles.

Hypersensitivity Pneumonitis (HP) is an immunologically mediated interstitial lung disease that may result from repeated inhalation of many different environmental agents. Heterogeneity of the clinical presentation and bronchoalveolar lavage profiles have been described, possibly related to different occupational exposures. The aim of our study was to compare bronchoalveolar lavage fluid (BALF), clinical, functional and radiological characteristics of the two most frequent forms of HP seen in our practice: Suberosis (an HP related to moldy cork dust exposure) and bird fancier's disease (BFD). We included 81 patients with Suberosis, with a mean age of 38.8 +/- 11.3 years and a mean exposure of 20.0 +/- 10.5 years and 32 patients with BFD, with a mean age of 46.3 +/- 11.8 years and mean exposure of 10.5 +/- 1.0 years. Patients with BFD had more acute forms, while subacute and chronic presentations predominated in Suberosis. Restrictive defect was the most frequent pattern of lung function impairment, and more severe in BFD. Ground glass opacities were the most frequent pattern in high-resolution computed tomography. A normal chest x-ray was more frequently seen in Suberosis. Both types of HP had lymphocytic alveolitis in BALF: Suberosis - 6.6 +/- 5.7 x 10(5) ml-l cells, 58.8 +/- 18.9% lymphocytes; bird fancier's disease - 9.0 +/- 6.5 x 105 ml-l cells, 61.7 +/- 22.2% lymphocytes. Although BALF CD8+ lymphocytes predominated in both diseases, the proportion of CD4+ and CD4/CD8 ratios were significantly higher in bird fancier's disease (Suberosis: 0.47 +/- 0.33 versus BFD: 1.1 +/- 1.5; p < 0.005). Moreover, BALF cellularity and mast cell counts were also significantly higher in BFD. In conclusion, Suberosis and bird fancier's disease are HP with different clinical and laboratory profiles, suggesting that despite their pathophysiological similarities, different antigenic exposures may cause different immune and inflammatory response dynamics in the lung.

[Suberosis: involvement of bronchoalveolar +mastocytes in the genesis of interstitial involvement]. / La suberosis: implicación de los mastocitos broncoalveolares en la génesis de la afectación intersticial.

UNLABELLED: Suberosis--the lung disease suffered by cork industry workers--may present in the form of either hypersensitivity pneumonitis (HP) or obstructive pulmonary disease (OPD) with asthma-like symptoms or chronic bronchitis. Mast cells play an important role in pulmonary inflammation and are particularly implicated in the rapid release of mediators in bronchoconstriction and the production of cytokines and mediators of fibroblast activity. Increased numbers of mast cells are present in bronchoalveolar lavage (BAL) fluid in interstitial lung diseases, suggesting that these cells also participate in chronic inflammatory processes and in pulmonary fibrosis. OBJECTIVES: To assess the participation of mas cells in interstitial pulmonary inflammation in cork industry workers by histochemically analyzing their presence in BAL fluid. Foreseeing the possible implication of bronchoalveolar mast cells in the pathogenesis of suberosis, we also studied their relation to various signs and symptoms of the disease, to respiratory function parameters and to degree of alveolitis. PATIENTS AND METHODS: Thirty-one cork industry workers with respiratory symptoms related to occupational exposure were enrolled. Occupational and case histories were taken. Physical examinations were complemented by chest X-rays, plethysmography/spirometry, fiberoptic bronchoscopy with BAL, and determination of carbon monoxide diffusing capacity (DLCO) and arterial blood gases at rest. Patient classification (20 with HP and 11 with OPD) was based on clinical and functional criteria and analysis of BAL fluid. Mast cells in cytospinned samples treated with two different stains [May-Grunwald-Giemsa (MGG) and Toluidine Blue (Tol.Bl.)] were counted by two observers and the results were compared. MAIN RESULTS: Good correlation between the two staining methods was confirmed (rs = 0.86, p < 0.0001). Correlation between the two observers was also good (MGG rs = 0.86, Yol.Bl. rs = 0.87, p < 0.0001). The number of mast cells in BAL fluid was significantly higher in patients with HP [13.4 +/- 4.5 (x +/- SEM)] than in those with OPD (0.9 +/- 0.3; p < 0.002, Mann Whitney test). The subgroup of eight patients with poorer respiratory function (CV and/or DLCO < 80% of reference value) also had higher mast cell counts in BAL (19.9 +/- 7.7 versus 3.5 +/- 1.7; p = 0.002). We also saw a negative relation between mast cell counts in BAL fluid and lung function parameters: total lung capacity (rs = -0.68, p = 0.005) and DLCO (rs = -0.54, p = 0.008). Mast cell recovery from BAL fluid was positively related to severity of alveolitis in terms of total cell counts (rs = 0.62, p = 0.002), absolute lymphocyte counts (rs = 0.56, p = 0.006) and albumin levels (rs = 0.68, p = 0.003). CONCLUSIONS: Our findings suggest that mast cells participate in interstitial lung cell response to the inhalation of organic cork dust, particularly when HP is the form of presentation. Moreover, mas cell recruitment on the alveolar surface seems to be related to the intersity of lymphocytosis and interstitial pulmonary inflammation and to lung function deterioration in affected patients.

Sarcoidosis-tuberculosis association: a case report.

The association of sarcoidosis and tuberculosis is rare and, its differentiation is sometimes very difficult. We report the case of a young man who presented with type III sarcoidosis complicated by pulmonary tuberculosis within 17 months of the diagnosis, leading to a fatal outcome. A change in antimycobacterial antigen complex A60 immunoglobulin G (IgG) titres, as seen in our case, may be helpful in differentiating between the two diseases.

Broncho-alveolar inflammation in cork worker's asthma.

UNLABELLED: Suberosis is an occupational lung disease of cork workers associated with repeated exposure to mouldy cork dust in the cork industry, usually presenting as an interstitial lung disorder (Extrinsic Allergic Alveolitis). However, Occupational Asthma can also be associated with cork dust exposure and demonstrated by serial peak expiratory flow changes. AIM: To investigate broncho-alveolar inflammation in patients with cork work-related occupational asthma, evaluated by Broncho-alveolar fluid cellular profiles and serial peak expiratory flow (PEF) rates monitoring. RESULTS: We studied 14 patients with respiratory symptoms associated with occupational exposure in the cork industry. Positive PEF monitoring occurred in 7 cases (Occupational Asthma-OA), and in 7 (Non-occupational asthmatics-NOA) PEF records were negative. There were no differences in age, lung function (FEV1%, RV%), bronchial hyperresponsiveness, years of exposure, and atopy between the two patients groups. However, patients with work-related asthma had higher BAL eosinophil counts than NOA (1.9 +/- 2.6% versus 0.2 +/- 0.3%; p < 0.05, Wilcoxon test). CONCLUSIONS: Cork worker's asthma, demonstrated by work related changes in serial PEF recordings, is associated with eosinophilic lung inflammation as described in other forms of occupational asthma.

[Obstructive sleep apnoea and cardiovascular disease--a retrospective study]. / Síndroma de apneia obstrutiva do sono e doença cardiovascular--estudo retrospectivo.

INTRODUCTION: Obstructive Sleep Apnea (OSA) is associated with adverse effects in cardiovascular physiology. Some studies have connected this pathology with Cardiovascular Disease (CVD). AIM: Determine the prevalence of CVD in patients with OSA of different severity and verify the association between CVD and OSA. MATERIAL AND METHODS: Retrospective study based on data from 155 consecutive patients with OSA referred to a Sleep Disordered Breathing Clinic in an University Hospital during the year of 2001. RESULTS: The majority of patients were male (83,2%), the mean age was 53,6 +/- 11,9 years. Severe OSA was observed in 52,9% patients, moderate in 20% and mild in 27,1% patients (mean Apnea Hypopnea Index of 35,2 +/- 23,8/hour). Among the studied individuals 52,3% showed CVD, being Arterial Hypertension (AH) the most common disease (45,8%). Acute Myocardial Infarction appeared in 6,5% and Angina in 3,9% cases. The majority of the patients (67,1%) were obese (Body Mass Index - BMI>30). The mean BMI was 33,1 +/- 6,34. A significant (31%) number of patients reported Lipid Disorders and 11% reported Diabetes mellitus. The majority (51,3%) of patients referred smoking habits. Severity of OSA (evaluated by AHI) was significantly higher in women with AH (p= 0,033) and significantly lower in patients who developed Cerebrovascular Disease (CeVD) (p = 0,036). CONCLUSIONS: The studied population presented a high prevalence of CVD, being the severity of OSA significantly higher in women with AH. The increased prevalence of CVD in patients with OSA has been documented in recent epidemiologic studies. The physiopathologic mechanisms underlying this association, certainly, deserve further investigation.