Optimizing breathlessness management in amyotrophic lateral sclerosis: insights from a comprehensive systematic review.

BACKGROUND: Breathlessness is a prevalent symptom affecting the quality of life (QOL) of Amyotrophic Lateral Sclerosis (ALS) patients. This systematic review explored the interventions for controlling breathlessness in ALS patients, emphasizing palliative care (PALC), non-invasive ventilation (NIV), opioids, and non-pharmacological strategies. METHODS: A comprehensive search of PubMed, Cochrane Library, and Web of Science databases was conducted. Eligibility criteria encompassed adults with ALS or motor neuron disease experiencing breathlessness. Outcomes included QOL and symptom control. Study designs comprised qualitative studies, cohort studies, and randomized controlled trials. RESULTS: Eight studies were included, most exhibiting low bias risk, comprising one randomized controlled trial, three cohort studies, two comparative retrospective studies, and two qualitative studies (interviews). Most studies originated from Europe, with one from the United States of America. The participants totaled 3423, with ALS patients constituting 95.6%. PALC consultations significantly improved symptom assessment, advance care planning, and discussions about goals of care. NIV demonstrated efficacy in managing breathlessness, with considerations for device limitations. Opioids were effective, though predominantly studied in non-ALS patients. Non-pharmacological strategies varied in efficacy among patients. CONCLUSION: The findings underscore the need for individualized approaches in managing breathlessness in ALS. PALC, NIV, opioids, and non-pharmacological strategies each play a role, with unique considerations. Further research, especially ALS-specific self-management studies, is warranted.

The First Korean Case with Cardiac, Facial, and Digital Anomalies with Developmental Delay Caused by De Novo TRAF7 p.Arg655Gln Variant.

TRAF7-related disorders represent some of the rarest inherited disorders, exhibiting clinical features that overlap with cardiac, facial, and digital anomalies with developmental delay (CAFDADD) syndrome, as well as blepharophimosis-mental retardation syndrome (BMRS). A 36-year-old male, presenting with total blindness, blepharophimosis, and intellectual disability, was admitted for the assessment of resting dyspnea several months previously. He had a history of being diagnosed with obstructive sleep apnea (OSA). Transesophageal and transthoracic echocardiography unveiled right ventricular dilatation without significant pulmonary hypertension, bicuspid aortic valve with aortic root aneurysm, and aortic regurgitation in the proband. Sanger sequencing identified a de novo TRAF7 variant (c.1964G>A; p.Arg655Gln). Subsequently, aortic root replacement using the Bentall procedure was performed. However, despite the surgery, he continued to experience dyspnea. Upon re-evaluating OSA with polysomnography, it was discovered that continuous positive airway pressure support alleviated his symptoms. The underlying cause of his symptoms was attributed to OSA, likely exacerbated by the vertebral anomaly and short neck associated with CAFDADD syndrome. Clinicians should be attentive to the symptoms associated with OSA as it is a potentially serious medical condition in patients with TRAF7 variants.

Diagnostic performance of a clinical ultrasound-based algorithm for acute heart failure in patients presenting to the emergency department with dyspnea. / Rendimiento diagnóstico de un algoritmo basado en ecografía clínica para el diagnóstico de insuficiencia cardiaca aguda en pacientes que consultan en urgencias por disnea.

OBJECTIVES: To study the diagnostic performance of an ultrasound-based algorithm that includes the deceleration time (DT) of early mitral filling to establish a diagnosis of acute heart failure (AHF) in patients who come to an emergency department because of dyspnea. MATERIAL AND METHODS: Prospective analysis in a convenience sample of patients who came to a hospital emergency department with acute dyspnea. The algorithm included ultrasound findings and 4 echocardiographic findings as follows: mitral annular plane systolic excursion, Doppler mitral flow velocity, tissue Doppler imaging measure of the lateral annulus, and the DT of early mitral filling. The definitive diagnosis was made by 2 physicians blinded to each other's diagnosis and the ultrasound findings. RESULTS: A total of 166 adult patients with a mean (SD) age of 76 (13) years were included; 79 (48%) were women. AHF was the definitive diagnosis in 62 patients (37%). Diagnostic agreement was good between the 2 physicians (κ = 0.71). The algorithm classified all the patients, and there were no undetermined diagnoses. Diagnostic performance indicators for the ultrasound-based algorithm integrating early DT findings were as follows: area under the receiver operating characteristic curve, 0.91 (95% CI, 0.86-0.96); sensitivity, 87% (95% CI, 76%-94%); specificity, 95% (95% CI, 89%-98%); positive likelihood ratio, 18.1 (95% CI, 7.7-42.8); and negative likelihood ratio, 0.14 (95% CI, 0.07-0.26). CONCLUSION: The ultrasound-based algorithm integrating the DT of early mitral filling performs well for diagnosing AHF in emergency patients with dyspnea. The inclusion of early DT allows all patients to be diagnosed.

OBJETIVO: Analizar el rendimiento diagnóstico de un algoritmo ecográfico que incluye el tiempo de desaceleración precoz del flujo mitral (TD) para establecer el diagnóstico de insuficiencia cardiaca aguda (ICA) en pacientes que consultan en un servicio de urgencias hospitalario (SUH) por disnea. METODO: Análisis prospectivo de una muestra de conveniencia de pacientes que consultan por disnea aguda en un SUH. El algoritmo ecográfico incluyó la ecografía pulmonar y cuatro parámetros ecocardiográficos, se midió MAPSE (desplazamiento sistólico del plano del anillo mitral), medidas doppler de flujo mitral, medidas doppler tisular en el anillo mitral lateral y TD. El diagnóstico final fue asignado por 2 médicos ciegos entre sí y a los hallazgos ecográficos. RESULTADOS: Se incluyeron 166 pacientes adultos, la edad media fue de 76 años (DE 13) y 79 eran mujeres (48%). Hubo 62 pacientes (37%) con un diagnóstico final de ICA. La concordancia entre asignadores fue buena para el diagnóstico de ICA (κ = 0,71). El algoritmo clasificó a todos los pacientes, no hubo ningún diagnóstico indeterminado. El rendimiento diagnóstico del algoritmo mostró un área bajo la curva de 0,91 (IC 95%: 0,86-0,96), sensibilidad del 87% (IC 95%: 76%-94%), especificidad del 95% (IC 95%: 89%-98%), razón de verosimilitud positiva del 18,1 (IC 95%: 7,7-42,8), razón de verosimilitud negativa del 0,14 (IC 95%: 0,07-0,26). CONCLUSIONES: Un algoritmo ecográfico que incluye el TD tiene un buen rendimiento para el diagnóstico de ICA en pacientes que acuden a SUH por disnea. Además, el uso de TD permite clasificar a todos los pacientes.

Impact of delayed mobile medical team dispatch for respiratory distress calls: a propensity score matched study from a French emergency communication center.

BACKGROUND: Shortness of breath is a common complaint among individuals contacting emergency communication center (EMCCs). In some prehospital system, emergency medical services include an advanced life support (ALS)-capable team. Whether such team should be dispatched during the phone call or delayed until the BLS-capable paramedic team reports from the scene is unclear. We aimed to evaluate the impact of delayed MMT dispatch until receiving the paramedic review compared to immediate dispatch at the time of the call on patient outcomes. METHODS: A cross-sectional study conducted in Lyon, France, using data obtained from the departmental EMCC during the period from January to December 2019. We included consecutive calls related to adult patients experiencing acute respiratory distress. Patients from the two groups (immediate mobile medical team (MMT) dispatch or delayed MMT dispatch) were matched on a propensity score, and a conditional weighted logistic regression assessed the adjusted odds ratios (ORs) for each outcome (mortality on days 0, 7 and 30). RESULTS: A total of 870 calls (median age 72 [57-84], male 466 53.6%) were sought for analysis [614 (70.6%) "immediate MMT dispatch" and 256 (29.4%) "delayed MMT" groups]. The median time before MMT dispatch was 25.1 min longer in the delayed MMT group (30.7 [26.4-36.1] vs. 5.6 [3.9-8.8] min, p < 0.001). Patients subjected to a delayed MMT intervention were older (median age 78 [66-87] vs. 69 [53-83], p < 0.001) and more frequently highly dependent (16.3% vs. 8.6%, p < 0.001). A higher proportion of patients in the delayed MMT group required bag valve mask ventilation (47.3% vs. 39.1%, p = 0.03), noninvasive ventilation (24.6% vs. 20.0%, p = 0.13), endotracheal intubation (7.0% vs. 4.1%, p = 0.07) and catecholamine infusion (3.9% vs. 1.3%, p = 0.01). After propensity score matching, mortality at day 0 was higher in the delayed MMT group (9.8% vs. 4.2%, p = 0.002). Immediate MMT dispatch at the call was associated with a lower risk of mortality on day 0 (0.60 [0.38;0.82], p < 0.001) day 7 (0.50 [0.27;0.72], p < 0.001) and day 30 (0.56 [0.35;0.78], p < 0.001) CONCLUSIONS: This study suggests that the deployment of an MMT at call in patients in acute respiratory distress may result in decreased short to medium-term mortality compared to a delayed MMT following initial first aid assessment.

Effects of a cardiopulmonary rehabilitation protocol on functional capacity, dyspnea, fatigue, and body composition in individuals with post-COVID-19 syndrome: A randomized controlled trial.

BACKGROUND AND OBJECTIVE: Reduced functional capacity, dyspnea, fatigue, and changes in body composition are common in patients with post-COVID-19 syndrome (PCS), and cardiopulmonary rehabilitation may improve these parameters. Thus, the present study verified the effects of cardiopulmonary rehabilitation (respiratory, aerobic, and resistance muscle training) on submaximal exercise tolerance, dyspnea, fatigue, and body composition. METHODS: This controlled and randomized clinical trial applied a six-week outpatient intervention protocol in individuals over 18 years old (n = 33) with a diagnosis of COVID-19 confirmed by polymerase chain reaction. These individuals were allocated to cardiopulmonary rehabilitation (n = 17) or control groups (i.e., educational lectures; n = 16). The cardiopulmonary rehabilitation group performed respiratory, aerobic, and resistance muscle training. Submaximal exercise tolerance, dyspnea, fatigue, and body composition were assessed before and after the protocol. RESULTS: After 6 weeks, the cardiopulmonary rehabilitation group increased the tolerance to submaximal exercise, with a difference of 100.46 m (95% confidence interval [CI]: 7.40-193 m) in the distance walked on the six-minute walk test, reduced dyspnea (-1.45, 95% CI: -1.98--0.92) in the modified Medical Research Council, and increased 0.63 kg (95% CI: 0.09-1.18 kg) of muscle mass in the upper limbs compared with the control group. CONCLUSION: The six-week cardiopulmonary rehabilitation protocol improved functional capacity, reduced dyspnea, and increased muscle mass in the upper limbs in individuals with PCS. Thus, these results supported the protocol use in this population and encourage further studies to assess its effectiveness in a large sample.

Impact of pulmonary rehabilitation on patients with different chronic respiratory diseases during hospitalization.

The impact of pulmonary rehabilitation (PR) on patients with different chronic respiratory diseases (CRDs) during hospitalization has not been thoroughly evaluated before. The objectives of the current research were to assess the effect of comprehensive PR management on inpatients' self-management skills, exercise capacity, nutrition assessment and mental health issues and explore whether impacts of PR vary in different CRDs. This retrospective study analyzed the clinical data from 272 inpatients with CRDs receiving PR management during hospitalization between October 2020 and March 2022 in Beijing Chao-Yang Hospital. Significant improvements were found in the patients' ability of daily living (ADL), dyspnea (assessed by modified medical research council dyspnea scale (MMRC)), handgrip strength, maximal inspiratory and expiratory pressure, anxiety (using the 7-item generalized anxiety disorder scale (GAD-7)) and depression (the 9-item patient health questionnaire score (PHQ-9)). There was no significant change in nutrition assessment pre-post PR management during hospitalization. The subgroup analyses were conducted on hospitalized patients with chronic obstructive pulmonary disease (COPD), bronchiectasis, asthma, interstitial lung diseases (ILDs) and other CRDs (e.g., lung cancer, diaphragm hemiparesis, obesity, etc.). The results showed that ADL, MMRC score, MIP, MEP, PHQ-9 score improved in all subgroups with CRDs. Handgrip strength of left hand was increased in COPD inpatients and anxiety was improved in all subgroups except for ILDs. Comprehensive PR management was necessary and beneficial for patients with different CRDs during hospitalization.

Early predictors of intensive care unit admission among COVID-19 patients in Qatar.

Background: COVID-19 is associated with significant morbidity and mortality. This study aimed to explore the early predictors of intensive care unit (ICU) admission among patients with COVID-19. Methods: This was a case-control study of adult patients with confirmed COVID-19. Cases were defined as patients admitted to ICU during the period February 29-May 29, 2020. For each case enrolled, one control was matched by age and gender. Results: A total of 1,560 patients with confirmed COVID-19 were included. Each group included 780 patients with a predominant male gender (89.7%) and a median age of 49 years (interquartile range = 18). Predictors independently associated with ICU admission were cardiovascular disease (adjusted odds ratio (aOR) = 1.64, 95% confidence interval (CI): 1.16-2.32, p = 0.005), diabetes (aOR = 1.52, 95% CI: 1.08-2.13, p = 0.016), obesity (aOR = 1.46, 95% CI: 1.03-2.08, p = 0.034), lymphopenia (aOR = 2.69, 95% CI: 1.80-4.02, p < 0.001), high AST (aOR = 2.59, 95% CI: 1.53-4.36, p < 0.001), high ferritin (aOR = 1.96, 95% CI: 1.40-2.74, p < 0.001), high CRP (aOR = 4.09, 95% CI: 2.81-5.96, p < 0.001), and dyspnea (aOR = 2.50, 95% CI: 1.77-3.54, p < 0.001). Conclusion: Having cardiovascular disease, diabetes, obesity, lymphopenia, dyspnea, and increased AST, ferritin, and CRP were independent predictors for ICU admission in patients with COVID-19.

Associations between respiratory function, balance, postural control, and fatigue in persons with multiple sclerosis: an observational study.

Introduction: Fatigue, postural control impairments, and reduced respiratory capacities are common symptoms in persons diagnosed with Multiple Sclerosis (MS). However, there is a paucity of evidence establishing correlations among these factors. The aim of this study is to analyze respiratory function in persons with MS compared to the control group as well as to analyze the relationship between fatigue, respiratory function and postural control in persons with MS. Materials and methods: A total of 17 persons with MS and 17 healthy individuals were enrolled for this cross-sectional study. The evaluated parameters included fatigue assessed using the Visual Analog Scale-fatigue (VAS-F) and the Borg Dyspnea Scale, postural control assessed through the Mini Balance Evaluation System Test (Mini-BESTest), Berg Balance Scale (BBS), Timed Up and Go (TUG) test, and Trunk Impairment Scale (TIS); and respiratory capacities measured by Maximum Inspiratory Pressure (MIP), Maximum Expiratory Pressure (MEP), Forced Vital Capacity (FVC), Forced Expiratory Volume in the first second (FEV1), FEV1/FVC ratio, Diaphragmatic excursion and diaphragmatic thickness. Results: A very high correlation was observed between the Borg Dyspnoea Scale and the BBS (r = -0.768), TUG (0.867), and Mini-BESTest (r = -0.775). The VAS-F exhibited an almost perfect correlation solely with the TUG (0.927). However, none of the variables related to fatigue exhibited any correlation with the respiratory variables under study. Balance-related variables such as BBS and Mini-BESTest demonstrated a very high and high correlation. Respectively, with respiratory function variables MEP (r = 0.783; r = 0.686), FVC (r = 0.709; r = 0.596), FEV1 (r = 0.615; r = 0.518). BBS exhibited a high correlation with diaphragmatic excursion (r = 0.591). Statistically significant differences were noted between the persons with MS group and the control group in all respiratory and ultrasound parameters except for diaphragmatic thickness. Conclusion: The findings suggest that decreased postural control and balance are associated with both respiratory capacity impairments and the presence of fatigue in persons with MS. However, it is important to note that the alterations in respiratory capacities and fatigue are not mutually related, as indicated by the data obtained in this study. Discrepancies were identified in abdominal wall thickness, diaphragmatic excursion, and respiratory capacities between persons with MS and their healthy counterparts.

Long COVID outcomes following omicron wave in non-hospital population.

Background: The persistence of symptoms or the development of new symptoms following a diagnosis of SARS-CoV-2 has given rise to a multifaceted clinical condition referred to as "long COVID" (LC). The understanding of LC among China's non-hospitalized population continues to be insufficient. This investigation was designed to evaluate the protracted consequences amongst this demographic, as well as to identify the associated risk factors. Methods: This research constitutes a prospective cohort study focusing on non-hospitalized individuals, aged between 18 and 59, who have been positively diagnosed with COVID-19. Each participant was subjected to a sequence of questionnaire-based surveys, designed to evaluate symptoms as well as the status of depression and anxiety. A logistic regression model, adjusted for multiple variables, was employed to scrutinize the correlation between demographic elements, lifestyle attributes, and health-related risk factors in relation to conditions and symptoms post COVID-19 infection. Results: A total of 706 individuals participated in the 3 months follow-up, with 620 continuing on to the 6 months follow-up. The median age was 35 (28, 43) years, and 597 (85%) are female. Upon follow-up, Compared with patients without LC, patients with LC have a higher proportion of females (420 (87%) vs. 177 (79%); p = 0.010), were older (35 (29, 44) years vs. 33 (27, 41) years; p = 0.010) and have more comorbidities. Out of all participants, 483 (68.4%) reported experiencing at least one symptom at the 3 months mark, while 49.7% reported symptoms persisting at the 6 months mark. At the 3 months follow-up, the most prevalent persistent symptoms were cough (46%), fatigue (38%), and shortness of breath (34%). By the 6 months follow-up, fatigue (25%), shortness of breath (22%), and sleep disorders (16%) were the most commonly reported symptoms. Anxiety and depression were consistently reported as prevalent symptoms throughout the follow-up period. Most patient symptoms fade over time, with the quickest decreases observed in cough (from 46 to 9%), expectoration (from 26 to 6.3%), smell disorder (from 16 to 3.9%), and taste disorder (from 18 to 3.5%). Male and those possessing advanced educational qualifications exhibit a decreased susceptibility to the sustained incidence of coughing. Conversely, older age and the presence of comorbidities were identified as risk factors for persistent fatigue and shortness of breath. Conclusion: In the after of COVID-19, it has been observed that the majority of patient symptoms tend to decrease over time. The primary residual symptoms noticed after a 6 month follow-up were fatigue, dyspnea, and sleep disturbances. However, it's noteworthy that the risk factors associated with these symptoms exhibit subtle variations. Furthermore, psychological sequelae, namely depression and anxiety, are frequently reported among COVID-19 survivors.

Effect of pulmonary rehabilitation in patients with post-tuberculosis sequelae with functional limitation.

BACKGROUND: Pulmonary rehabilitation improves dyspnea, functional limitation and quality of life in patients with chronic respiratory disease especially Chronic obstructive pulmonary disease (COPD). Whether Pulmonary rehabilitation (PR) will have similar effect in patients with post-tuberculosis sequelae or not and whether the two morphological variants will respond similarly or not was the purpose of our study. METHODS: Adult patients fulfilling the inclusion criteria of a diagnosis of post-tuberculosis sequelae with functional limitation (modified medical research council [mMRC] grade 1 or more) were recruited over a period of two years. A baseline health assessment in the different domains of health was done at the beginning and repeated just after the completion of the rehabilitation program at 6 weeks. RESULTS: 26 patients completed the PR protocol of our study. Dyspnea improved from an mMRC grade of 1.5 to 0.7 post-PR (p < 0.001). 6MWD increased by 34 meters from a baseline value of 408.6 meters to 442.7 meters post-PR. (p-value 0.3) St. George's Respiratory Questionnaire (SGRQ) symptom score decreased by 13 points, SGRQ activity score decreased by 18 points, SGRQ impact score decreased by 18 and SGRQ total score decreased by 17 points with p-values of 0.037, 0.002, 0.004 and 0.002 for SGRQ symptom score, SGRQ activity score, SGRQ impact score, SGRQ total score respectively. Depression Anxiety Stress Scale (DASS) Stress score decreased by 6.7, DASS Anxiety score decreased by 6.6, and DASS Depression score decreased by 5.5 points. Intergroup comparison revealed both bronchiectasis predominant group and fibrosis predominant group responded similarly to PR. CONCLUSION: PR improved parameters assessing dyspnea, quality of life and mental health indices significantly. Improvement in functional capacity was not statically significant. Both the morphological variants responded similarly to the PR.

Weight loss with GLP-1 analogues in preparation for transplantation.

This case describes a woman in her 20s with a 6-month history of progressive exertional dyspnoea and cough. Examination revealed hypoxia on room air, sinus tachycardia, finger clubbing and bibasal inspiratory crackles. Inflammatory markers were mildly elevated and empirical antimicrobial therapy was commenced. A multidisciplinary discussion consensus diagnosis of acute interstitial pneumonitis was made based on the findings of high-resolution CT of the chest, macrophage predominant bronchoalveolar lavage cell differential and surgical lung biopsy. There was clinical and radiological deterioration despite glucocorticoids and antifibrotic therapy. A body mass index of 37.5 kg/m2 precluded her from lung transplant assessment and consideration. Following consultation with the weight management service, she was commenced on glucagon-like peptide 1 (GLP-1) analogue therapy. She had a remarkable response within 6 months, was listed for lung transplantation, and within 18 months of her initial presentation, a double lung transplantation was performed.

The paradox of platypnoea-orthodeoxia syndrome.

Platypnoea-orthodeoxia is a rare clinical syndrome characterised by dyspnoea and oxygen desaturation in the upright position which improves when supine. It requires two components: a sufficiently sized anatomical vascular defect (typically intra-cardiac or intra-pulmonary) combined with a functional component that promotes positional right-to-left shunting. We describe the rare occurrence of a patient with platypnoea-orthodeoxia syndrome (POS) because of a paradoxical shunt through a patent foramen ovale caused by a large right atrial line-associated thrombus in a male with metastatic oesophageal cancer undergoing chemotherapy. This case is a timely reminder to consider POS amongst differentials for hypoxia as it is often treatable if recognised.

Treatment with systemic corticosteroid versus placebo for exacerbations of COPD: A systematic review and meta-analysis.

BACKGROUND: For the treatment of COPD exacerbations, systemic corticosteroids are recommended in addition to short-acting bronchodilators. Although there have been several systemic reviews, many of the included studies were conducted before 2007 and a re-evaluation has not been performed since 2014. Therefore, we conducted a systematic review and meta-analysis to evaluate the efficacy and safety profile of systemic corticosteroids in patients with COPD during exacerbations. METHODS: We searched relevant randomized control trials (RCTs) and analyzed the treatment failure, relapse, lung function, improvement in PaO2 and PaCO2, dyspnea, quality of life (QOL), length of stay in hospital and adverse events including hyperglycemia and mortality as the outcomes of interest. RESULTS: We identified a total of 12 RCTs (N = 1336). Systemic corticosteroids significantly reduced the treatment failure (odds ratios; OR 0.41, 95% confidence intervals; CI 0.25 to 0.67) and hospital length of stay (mean difference; MD -1.57 days, 95% CI -2.36 to -0.78) and improved FEV1 (MD 0.18 L, 95% CI 0.08 to 0.28) and dyspnea (transitional dyspnea index; MD 1.90, 95% CI 0.26 to 3.54) in COPD exacerbations compared to placebo. However, systemic corticosteroids were associated with a significantly higher incidence of adverse events (OR 1.83, 95% CI 1.25 to 2.69) and hyperglycemia (OR 2.94, 95% CI 1.68 to 5.14). CONCLUSIONS: In patients with moderate and severe COPD and severe obstructive impairment during exacerbations, systemic corticosteroids cause more adverse events, including hyperglycemia, than placebo but significantly reduce the treatment failure and hospital length of stay and improve FEV1 and dyspnea.

A first look at the reliability, validity and responsiveness of L-PF-35 dyspnea domain scores in fibrotic hypersensitivity pneumonitis.

BACKGROUND: Dyspnea impairs quality of life (QOL) in patients with fibrotic hypersensitivity pneumonitis (FHP). The Living with Pulmonary Fibrosis questionnaire (L-PF) assesses symptoms, their impacts and PF-related QOL in patients with any form of PF. Its scores have not undergone validation analyses in an FHP cohort. METHODS: We used data from the Pirfenidone in FHP trial to examine reliability, validity and responsiveness of the L-PF-35 Dyspnea domain score (Dyspnea) and to estimate its meaningful within-patient change (MWPC) threshold for worsening. Lack of suitable anchors precluded conducting analyses for other L-PF-35 scores. RESULTS: At baseline, Dyspnea's internal consistency (Cronbach's coefficient alpha) was 0.85; there were significant correlations with all four anchors (University of California San Diego Shortness of Breath Questionnaire scores r = 0.81, St. George's Activity domain score r = 0.82, percent predicted forced vital capacity r = 0.37, and percent predicted diffusing capacity of the lung for carbon monoxide r = 0.37). Dyspnea was significantly different between anchor subgroups (e.g., lowest percent predicted forced vital capacity (FVC%) vs. highest, 33.5 ± 18.5 vs. 11.1 ± 9.8, p = 0.01). There were significant correlations between changes in Dyspnea and changes in anchor scores at all trial time points. Longitudinal models further confirmed responsiveness. The MWPC threshold estimate for worsening was 6.6 points (range 5-8). CONCLUSION: The L-PF-35 Dyspnea domain appears to possess acceptable psychometric properties for assessing dyspnea in patients with FHP. Because instrument validation is never accomplished with one study, additional research is needed to build on the foundation these analyses provide. TRIAL REGISTRATION: The data for the analyses presented in this manuscript were generated in a trial registered on ClinicalTrials.gov; the identifier was NCT02958917.

Severe acute respiratory syndrome coronavirus 2 infection rate among pediatric patients with respiratory symptoms.

BACKGROUND: The incidence of coronavirus disease 2019 (COVID-19) in children has been increasing worldwide since the onset of the pandemic. This study examined the risk factors and characteristics of COVID-19 among pediatric patients compared to other respiratory viral infections. METHODS: This was a prospective cross-sectional study. Patients aged 0-18 years presenting with respiratory symptoms from October 2020 to December 2021 were included. Demographic and clinical data were reviewed. RESULTS: In total, 738 pediatric patients were enrolled. Of these, 48.5% had COVID-19, and 41.3% were infected with another respiratory virus. The COVID-19 incidence increased from 0.5% during the original strain outbreak (October 2020 to March 2021) to 56.5% and 73.4% during the alpha (April to June 2021) and delta (July to December 2021) periods, respectively. Children aged 6-18 years, being female, obesity, exposure to household members with COVID-19, and the delta period were risk factors for COVID-19. Being aged 1-5 years, obesity, shortness of breath, productive cough, and chest pain were associated with COVID-19 pneumonia. Children aged 5-18 years, underlying neurological disease, a history of COVID-19 pneumonia, and the delta period were associated with long COVID. CONCLUSIONS: Pediatric COVID-19 patients presenting with respiratory symptoms who are obese or have been exposed to household members with COVID-19 should be tested for COVID-19. COVID-19 patients who are obese, younger than five years old, or who present with shortness of breath, productive cough, or chest pain should be evaluated for pneumonia. COVID-19 patients with a history of COVID-19 pneumonia or underlying neurological disease should receive follow-up for long COVID.

Systematic review of the effects of patient errors using inhaled delivery systems on clinical outcomes in COPD.

BACKGROUND: Errors using inhaled delivery systems for COPD are common and it is assumed that these lead to worse clinical outcomes. Previous systematic reviews have included patients with both asthma and COPD and much of the evidence related to asthma. More studies in COPD have now been published. Through systematic review, the relationship between errors using inhalers and clinical outcomes in COPD, including the importance of specific errors, was assessed.MethodsElectronic databases were searched on 27 October 2023 to identify cohort, case-control or randomised controlled studies, which included patients with COPD, an objective assessment of inhaler errors and data on at least one outcome of interest (forced expiratory volume in 1 s, (FEV1), dyspnoea, health status and exacerbations). Study quality was assessed using the Newcastle and Ottawa scales. A narrative synthesis of the results was performed as there was insufficient detail in the publications to allow quantitative synthesis. There was no funding for the review. RESULTS: 19 publications were included (7 cohort and 12 case-control) reporting outcomes on 6487 patients. 15 were considered low quality, and most were confounded by the absence of adherence data. There was weak evidence that lower error rates are associated with better FEV1, symptoms and health status and fewer exacerbations. Only one considered the effects of individual errors and found that only some were related to worse outcomes. CONCLUSION: Evidence about the importance of specific errors using inhalers and outcomes would optimise the education and training of patients with COPD. Prospective studies, including objective monitoring of inhalation technique and adherence, are needed. PROSPERO REGISTRATION NUMBER: CRD42023393120.

Severe autoimmune hemolytic anemia following immunotherapy with checkpoint inhibitors in two patients with metastatic melanoma: a case report.

Introduction: Over the past decade, immune checkpoint inhibitors such as antibodies against cytotoxicity T-lymphocyte-associated protein 4 (CTLA-4) and programmed cell death protein 1 (PD-1) have become an important armamentarium against a broad spectrum of malignancies. However, these specific inhibitors can cause adverse autoimmune reactions by impairing self-tolerance. Hematologic side effects of immune checkpoint inhibitors, including autoimmune hemolytic anemia (AIHA), are rare but can be life-threatening. Case report: Herein, we report two patients on immune checkpoint inhibitors for metastatic melanoma who developed AIHA with symptoms of dyspnea and fatigue. In the first patient, symptoms alleviated after discontinuation of combined anti CTLA-4 and anti-PD-1 therapy, initiation of corticosteroids and application of a single red blood cell transfusion. Due to subsequent progress of melanoma, combinational anti-PD-1 and tyrosine kinase inhibitor therapy was initiated based on multidisciplinary tumor board decision. After two months, she again developed the described hematological and clinical signs of AIHA leading to cessation of anti-PD-1 therapy and initiation of corticosteroids, which again resulted in an alleviation of her symptoms. Due to further progression, the patient received dacarbazine for several months before she decided to stop any therapy other than palliative supportive care. In the second patient, discontinuation of anti-PD-1 therapy and initiation of corticosteroids entailed a complete alleviation of his symptoms. After refusing chemotherapy due to subsequent melanoma progression, he received radiotherapy of bone metastases and is currently enrolled in a clinical trial. The patient did not develop AIHA ever since. Conclusion: Hematologic immune-related adverse events due to treatment with immune checkpoint inhibitors are rare but can have life-threatening consequences. If dyspnea and other clinical symptoms are present, AIHA should be considered as a potential cause and treated promptly in a multidisciplinary setting. An expanded comprehension of risk factors and pathogenesis of AIHA is needed to identify high-risk patients beforehand, leading to more effective predictive and reactive treatment approaches.

High-flow nasal cannula during pulmonary rehabilitation for people with chronic obstructive pulmonary disease: A systematic review and meta-analysis.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is an increasingly concerning global public health issue due to its high burden of morbidity and mortality. Pulmonary rehabilitation (PR) is a comprehensive intervention to improve patients' physical and psychological conditions, commonly involving oxygen supplementation. The potential benefits of high-flow nasal cannula (HFNC) have recently sparked interest as oxygen therapy. In this context, this study aims to assess the effects of HFNC during the exercise training component of a PR program in people with COPD. METHODS: Systematic review (CRD42022330929). We included randomised controlled trials (RCTs), including crossover RCTs with adults with stable COPD. We included trials using oxygen therapy with HFNC during the exercise training component of a PR programme. PRIMARY OUTCOMES: disease-specific health-related quality of life (HRQoL), exercise capacity (EC) and adverse events. SECONDARY OUTCOMES: treatment adherence, breathlessness and future exacerbations. RESULTS: We included five studies with 300 participants with moderate to severe COPD. The certainty of the evidence was primarily low or very low for all outcomes of interest due to risk of bias, inconsistency or imprecision. HFNC has little to no difference in HRQoL (4 studies, 129 participants, MD 0.17, 95% CI -1.20 to 1.54; I2 50%). HFNC may result in little to no difference in EC (3 studies, 212 participants, mean difference 18.73, 95% CI -20.49 to 28.94; I2 56%), and we are uncertain about the effect of HFNC on breathlessness (4 studies; 244 participants, MD of -0.07, 95% CI -0.4 to 0.26; I2 63%). Only one study with 44 participants reported a participant's withdrawal because of progressive dyspnoea during lower limb exercise. CONCLUSIONS: We are uncertain about the effect of HFNC during the exercise component of a PR programme in HRQoL, EC or dyspnoea compared to usual care or conventional supplementary oxygen. Non-domiciliary oxygen patients showed improvements in HRQoL, EC and dyspnoea.

Relationship between dyspnoea and related factors in patients with cancer: a cross-sectional study.

OBJECTIVES: Dyspnoea is a common and distressing symptom in patients with cancer. We aimed to analyse the association between dyspnoea and related factors and to estimate their causal relationship. METHODS: A cross-sectional study was conducted. Patients with cancer with dyspnoea and a mean Numerical Rating Scale (NRS) of ≥3 over 24 hours were enrolled at 10 institutions in Japan from December 2019 to February 2021. The outcomes included dyspnoea, cough and pain NRS over 24 hours, Eastern Cooperative Oncology Group Performance Status, Hospital Anxiety and Depression Scale, Somatosensory Amplification Scale, opioids for dyspnoea and respiratory failure. Path analyses were conducted to estimate the direct and indirect paths with reference to dyspnoea and related factors. RESULTS: A total of 209 patients were enrolled and 208 patients were included in the analysis. Cough worsened dyspnoea (ß=0.136), dyspnoea increased emotional distress (ß=1.104), emotional distress increased somatosensory amplification (ß=0.249) and somatosensory amplification worsened cough (ß=0.053) according to path analysis. CONCLUSION: There may be a vicious circle among dyspnoea and related factors: cough worsened dyspnoea, dyspnoea increased emotional distress, emotional distress increased somatosensory amplification and somatosensory amplification worsened cough. When treating dyspnoea in patients with cancer, managing these factors aimed at interrupting this vicious circle may be useful. TRIAL REGISTRATION NUMBER: UMIN Clinical Trials Registry (UMIN000038820).