Deventilation Syndrome in COPD Patients Receiving Long-Term Home Noninvasive Ventilation: A Systematic Scoping Review.

The treatment of patients with COPD and chronic hypercapnic respiratory failure using noninvasive ventilation (NIV) is well established. A "deventilation syndrome" (DVS) has been described as acute dyspnea after cessation of NIV therapy. A systematic scoping review reporting according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) searching Embase was conducted in September 2021. A final manual search followed in February 2023. Literature synthesis was blinded using Rayyan by three different reviewers. A total of 2,009 studies were screened. Five studies met the eligibility criteria. Four articles presented original data. Three articles examined potential treatment options. Three studies were prospective; none were randomized. A total of 122 patients were included. DVS was defined differently in all studies. Seventy-four patients were identified to suffer from DVS (48 controls). Patients were evaluated by blood gas analysis, transcutaneous TcCO2 measurement, spirometry, whole-body plethysmography, respiratory muscle assessments, diaphragmatic electromyography, ultrasound, 6-min walk test, polysomnography, and questionnaires. Treatment approaches studied were minimization of "patient-ventilator asynchrony" (PVA) and use of pursed- lip breathing ventilation. Pathophysiological mechanisms discussed were PVA, high inspiratory positive airway pressure, hyperinflation, respiratory muscle impairment, and increased respiratory rates. Compared with controls, patients with DVS appeared to suffer from more severe airway obstruction, hyperinflation, and PaCO2 retention; worse exercise test scores; and poorer quality of life. The available evidence does not allow for definite conclusions about pathophysiological mechanisms, ethology, or therapeutic options. Future studies should focus on a consistent definition and possible pathomechanisms.

Ambulatory Long-Term Oxygen Therapy in Patients with Severe COPD: A Randomized Crossover Trial to Compare Constant-Minute-Volume and Constant-Bolus Systems.

Background and Methods: Constant-minute-volume and constant-bolus devices serve as two different means of portable oxygen conservation. A prospective randomised crossover study was conducted in COPD GOLD IV patients to investigate the effect of these two devices on dyspnea, oxygenation and 6-minute walking test (6MWT) distance. The primary endpoint was the final operating level required (operating level range 1-5 for both devices) by either device to meet the success criteria for mobile oxygen therapy, as outlined in the British Thoracic Society guidelines (SpO2 ≥90% throughout 6MWT; ≥10% increase in walking distance from baseline; improvement in BORG of at least 1 point from baseline). Results: Twenty-five patients were enrolled in the study and randomly assigned to one of two sequences involving the use of each type of portable oxygen conservation device. 14 female, 67.9 years (±7.8); FEV1: 27.3%pred. (±8.4); PaO2 at rest without oxygen: 50.3mmHg (±5.9). For both systems, 24/25 patients (96%) were successfully recruited. The mean operating-level difference when success criteria were met was -0.58 in favor of the constant bolus device (95% CI: -0.88 to -0.28, P <0.001). Secondary endpoints (walking distance, respiratory rate and BORG dyspnea) showed no statistically significant or clinically relevant differences. An algorithm created especially for this study showed a high success rate in terms of titration for the required operating level. Conclusion: Both portable oxygen-conserving devices met the success criteria in 96% of patients in the 6MWT when they were titrated to the correct level. The constant-bolus device required a significantly lower operating level to achieve the success criteria, hereby reducing energy consumption. Individual titration of the respective device is recommended, which can be facilitated by the novel titration algorithm described here.

[Neuralgic amyotrophy: a common cause of unilateral and bilateral diaphragmatic pareses]. / Die neuralgische Amyotrophie: eine häufige Ursache uni- oder bilateraler Zwerchfellparesen.

There are several causes for unilateral or bilateral diaphragmatic paresis. The most common cause is an (intraoperative) injury to the phrenic nerve.However, in up to 20% of cases, no explanation can be found despite extensive workup. Neuralgic amyotrophy (NA, also known as Parsonage-Turner syndrome) is a common underdiagnosed multifocal autoimmune-inflammatory disease that predominantly affects proximal nerve segments of the upper extremities. Classic symptoms include acute onset of severe pain in the shoulder girdle with delayed onset of paresis of the shoulder and arm muscles. In at least 7% of cases, the phrenic nerve is also affected. Based on the annual incidence of NA of 1:1000, the entity as a cause of diaphragmatic dysfunction is probably not as uncommon as previously thought. However, clinical experience shows that this diagnosis is often not considered, and diaphragmatic paresis gets wrongly classified as idiopathic.This is particularly disastrous because in the early stage of NA, medical therapy with corticosteroids is mostly not considered and the possibility that surgical repair of the diaphragm may be performed prematurely, given that the condition may resolve spontaneously many months after symptom onset.The aim of the present article is to raise awareness of the entity of NA as a cause of diaphragmatic paresis and to establish a standardized approach to diagnosis and treatment.

The use of digital web-based video training for correct inhalation technique during the COVID-19 pandemic.

Scientifically validated web-based training videos for proper inhalation technique were increasingly used by an international audience during the pandemic. Translations into additional languages would support a larger patient population. https://bit.ly/3lYQwsD.

[Treatment alternatives for amyopathic MDA5-positive dermatomyositis]. / Therapiealternativen bei amyopathischer MDA5-positiver Dermatomyositis.

We report a case of an atypical course of therapy in amyopathic MDA5-antibody-positive dermatomyositis with interstitial lung disease. Due to the poor prognosis, early therapy with cyclophosphamide followed by rituximab was carried out initially in addition to the administration of prednisolone. Due to therapy failure, treatment was switched to mycophenolate mofetil. This showed a surprisingly rapid positive course in terms of interstitial lung disease, skin manifestation, and general disease activity.

[The digitalization of pneumology and the role of medical societies]. / Die Digitalisierung der Pneumologie und die Rolle der Fachgesellschaften.

BACKGROUND: Medicine is becoming increasingly digitalized. Digital applications are finding their way into health care. The aim of the study was to record the attitudes of members of the German Respiratory Society (DGP) towards digitalization. METHODS: The study was conducted in cooperation with the DGP, the German Respiratory League and the Health Innovation Hub (HIH) of the German Federal Ministry of Health. Data were gathered with the help of an online questionnaire (July/August 2021), analyzed descriptively and supplemented with a subgroup comparison regarding proactivity of the members. RESULTS: 284 questionnaires were complete and included in the analysis (31% female); 76.4% believed that digitalization would change their daily professional life within five years. 47.2% had prescribed or planned to prescribe Digital Health Applications (DiGA). Lack of technology skills of patients and the time required for health professionals were seen as critical (49.3 and 47.5%). Regarding DiGA, scientific proof of efficacy (48.9%) and ease of use for patients (47.9%) were rated as most important. The subgroup comparison showed that 42.7% actively informed themselves about digital medicine and that this group saw more opportunities for meaningful use of DiGA. CONCLUSION: The members of the DGP have mostly a positive view about the profound changes expected from digital medicine. The more active the role of DGP members and other professional societies, the more digital elements can add value to practice.

Anemia Severely Reduces Health-Related Quality of Life in COPD Patients Receiving Long-Term Home Non-Invasive Ventilation.

PURPOSE: To assess the influence of anemia on health-related quality of life (HRQL) in COPD patients receiving long-term non-invasive ventilation (NIV). PATIENTS AND METHODS: In this prospective single-center cohort study, COPD patients on long-term NIV were analyzed between June 2015 and May 2020. Linear multiple regression analyses were performed using the results of the Severe Respiratory Insufficiency Questionnaire (SRI) along with the following variables: sex, age, body mass index, duration of NIV, exacerbation history (≤1 versus >1 in the previous year), the updated Charlson comorbidity index, hemoglobin levels and anemia (WHO criteria). RESULTS: Anemia was identified in 32.8% (N=128). Anemia (mean difference -8.4, 95% CI -2.0/-14.9 SRI points, P=0.011) and exacerbations (mean difference -9.9, 95% CI -4.3/-15.5 SRI points, P=0.001) each had a negative impact on SRI summary scores. Exacerbations were negatively associated with six out of seven SRI subscale scores, while anemia was negatively associated with four out of seven. SRI summary scores dropped by 1.5 points for every g/dl of hemoglobin (P=0.08). No other variables had an influence on the SRI scores. CONCLUSION: The present study has shown that within a cohort of COPD patients undergoing long-term NIV, one-third were identified as anemic. Furthermore, anemia, like exacerbation history, was found to have a considerable negative impact on HRQL that is specific to patients with chronic respiratory failure. CLINICAL TRIAL REGISTRATION: German Clinical Trials Registry (DRKS00008759).

Living conditions and autonomy levels in COPD patients receiving non-invasive ventilation: impact on health related quality of life.

BACKGROUND: Research on health-related quality of life (HRQL) has become increasingly important in recent decades. However, the impact of both living conditions and the level of autonomy impairments on HRQL in COPD patients receiving non-invasive ventilation (NIV) is still unclear. METHODS: The Severe Respiratory Insufficiency Questionnaire (SRI) was used to measure HRQL in a prospective cohort of COPD patients in whom home NIV was already established. Data on sociodemographics, clinical characteristics and standardized levels of autonomy impairment were evaluated. A multiple linear regression analysis was performed to identify the factors associated with a reduced HRQL. RESULTS: A total of 137 patients (67.0 ± 7.8 years, 45% female) were assessed. The mean SRI Summary Score was 54.1 ± 16.9 (95%CI: 51.1-57.1; N = 127). Regular ambulatory care was provided in 76% of patients, but only 37% underwent pulmonary rehabilitation. Overall, 69% of patients lived with family members, while 31% lived alone (family situation). Autonomy impairment levels were most serious in 3%, serious in 14%, and significant in 29% of patients, while 54% had no impairments at all. Of note, higher levels of autonomy impairment were markedly associated with lower SRI scores (regression coefficient - 6.5 ± 1.1 per level; P < 0.001). In contrast, family situation (0.2 ± 3.0; P = 0.959), ambulatory care by a respiratory specialist (1.7 ± 3.6; P = 0.638), and pulmonary rehabilitation (- 0.8 ± 3.1; P = 0.802) did not appear to influence HRQL. Possible subgroup effects were evident for the factors "impaired autonomy" and "living in a nursing home" (P = 0.016). CONCLUSION: A higher level of autonomy impairment has been identified as the major determinant of reduced HRQL in COPD-patients receiving long-term NIV, particularly in those living in a nursing home. Trial Registration German Clinical Trials Register (DRKS00008759).