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Background: Respiratory disease may cause profound hypoxaemia during flight. Previously derived linear equations poorly predict the need for supplemental oxygen during air travel. The current gold standard assessment is the hypoxic challenge test (HCT). Recent guidelines recommend HCT is performed for those patients with SpO2 < 95% at sea level. The HCT protocol is a costly and time consuming investigation. Methods: Retrospective clinical and HCT data from 138 patients were applied to previous linear equations to assess predictive value. Novel non-linear predictive models (NLMs) were constructed from these data. The linear equations and the NLMs were then applied prospectively to 44 patients undergoing HCT. Results: Overall, 39% of historic patients had a positive HCT (PaO2N2 <50 mmHg). Existing linear equations varied in sensitivity (52-87%) and specificity (40-74%) at predicting positive HCT results. Seven novel NLMs (NLM1 to NLM7) were developed from the historic dataset. All NLMs predicted PaO2N2 more accurately than the original linear equations when tested prospectively. The best fit was observed using NLM2 which uses PaO2RA and PaCO2RA as input terms. The NLMs are applicable to a broad range of conditions. Conclusions: The novel NLMs represent a low cost option for the prediction of significant hypoxia during flight and perform better than SpO2 in identifying those patients who require more formal assessment with HCT. [ABSTRACT FROM AUTHOR]

The COVID-19 pandemic has resulted in a widespread move towards virtual consultations within secondary care due to infection concerns. This service evaluation undertaken in a Welsh Interstitial Lung Disease (ILD) specialist center looks at the experiences of unselected ILD patients and their careers with telephone consultations and aims to identify the role of telephone consultations in future practice for this patient group. A mixed methods approach was used with an initial postal questionnaire followed by a virtual focus group. From the 84 patient and 26 carer questionnaire responses, there was broad support for telephone consultations from both groups. The main perceived benefit was reduced infection risk, although face-to-face consultations were seen as holding more value. Themes identified in the virtual focus group of 6 participants recognized the need to balance quality of care against practical considerations. In conclusion, there was support for future telephone consultations if patients and clinicians have flexibility in determining when this occurs, instead of a face-to-face consultation., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2022.)

Despite proven efficacy of alemtuzumab in multiple sclerosis (MS), approximately 50% of individuals will develop a new autoimmune disease following treatment. To date, these have largely been antibody mediated and organ specific (primarily affecting the thyroid gland). In a retrospective case series of 187 patients from two UK specialist centres (Cardiff and Cambridge) followed up for a median of 10 years, we report three (1.6%) cases of sarcoidosis following alemtuzumab treatment of MS. This report increases the spectrum of auto-inflammatory disease following alemtuzumab and should be considered by clinicians when using this therapeutic agent for MS.

In idiopathic pulmonary fibrosis (IPF) breathing pattern changes with disease progress. This study aims to determine if unsupervised hierarchal cluster analysis (HCA) can be used to define airflow profile differences in people with and without IPF. This was tested using 31 patients with IPF and 17 matched healthy controls, all of whom had their lung function assessed using spirometry and carbon monoxide CO transfer. A resting tidal breathing (RTB) trace of two minutes duration was collected at the same time. A Euclidian distance technique was used to perform HCA on the airflow data. Four distinct clusters were found, with the majority (18 of 21, 86%) of the severest IPF participants (Stage 2 and 3) being in two clusters. The participants in these clusters exhibited a distinct minute ventilation ( p < 0.05), compared to the other two clusters. The respiratory drive was greatest in Cluster 1, which contained many of the IPF participants. Unstructured HCA was successful in recognising different airflow profiles, clustering according to differences in flow rather than time. HCA showed that there is an overlap in tidal airflow profiles between healthy RTB and those with IPF. The further application of HCA in recognising other respiratory disease is discussed.

Background: TKM-130803, a small interfering RNA lipid nanoparticle product, has been developed for the treatment of Ebola virus disease (EVD), but its efficacy and safety in humans has not been evaluated., Methods and Findings: In this single-arm phase 2 trial, adults with laboratory-confirmed EVD received 0.3 mg/kg of TKM-130803 by intravenous infusion once daily for up to 7 d. On days when trial enrolment capacity was reached, patients were enrolled into a concurrent observational cohort. The primary outcome was survival to day 14 after admission, excluding patients who died within 48 h of admission. After 14 adults with EVD had received TKM-130803, the pre-specified futility boundary was reached, indicating a probability of survival to day 14 of ≤0.55, and enrolment was stopped. Pre-treatment geometric mean Ebola virus load in the 14 TKM-130803 recipients was 2.24 × 109 RNA copies/ml plasma (95% CI 7.52 × 108, 6.66 × 109). Two of the TKM-130803 recipients died within 48 h of admission and were therefore excluded from the primary outcome analysis. Of the remaining 12 TKM-130803 recipients, nine died and three survived. The probability that a TKM-130803 recipient who survived for 48 h will subsequently survive to day 14 was estimated to be 0.27 (95% CI 0.06, 0.58). TKM-130803 infusions were well tolerated, with 56 doses administered and only one possible infusion-related reaction observed. Three patients were enrolled in the observational cohort, of whom two died., Conclusions: Administration of TKM-130803 at a dose of 0.3 mg/kg/d by intravenous infusion to adult patients with severe EVD was not shown to improve survival when compared to historic controls., Trial Registration: Pan African Clinical Trials Registry PACTR201501000997429.

Background and Objective: Idiopathic pulmonary fibrosis (IPF) is characterized by progressive decline in lung function and increasing dyspnoea. The aim of this study was to investigate the relationship among IPF, pulmonary function, resting tidal breathing patterns and level of breathlessness., Methods: Thirty-one participants with IPF and 17 matched healthy controls underwent lung function testing, followed by a 2-min period of resting tidal breathing. The IPF cohort was stratified according to disease severity, based on their forced vital capacity and diffusion capacity for carbon monoxide., Results: In comparison to the healthy controls, the IPF cohort showed a higher tidal volume, VT , of 0.22 L (P = 0.026) and a raised minute ventilation in the severest IPF group, while no differences in the timing of inspiration or expiration were observed. In the IPF cohort, the ratio of VT to forced vital capacity was around 15% higher. These changes corresponded with an increase in the self-reported sensation of breathlessness., Conclusion: Those with IPF increased their depth of breathing with worsening disease severity, with IPF-induced changes in pulmonary function and breathlessness associated with an altered tidal breathing pattern., (© 2015 Asian Pacific Society of Respirology.)

Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening illness of unknown aetiology, with no proven pharmacological treatments. There is a limited evidence base indicating that the disease negatively affects quality of life, leading to increased dependence, restrictions on daily activities and fatigue. However, there is a paucity of in-depth information on disease impact across its trajectory, particularly in relation to unmet needs, outcomes of importance to patients and the experiences of carers. Furthermore, little is known about the support and information needs of individuals and their carers, or at what point individual need should trigger a referral to palliative care services., Methods and Analysis: A mixed-methods study is proposed recruiting individuals with IPF at different stages of the disease and their carers from three respiratory centres in England and Wales. In-depth interviews will be undertaken with participants, adopting an Interpretative Phenomenological Analysis approach. The study will also use validated questionnaires to explore quality of life (EQ-5D), depression (Hospital Anxiety and Depression Scale), breathlessness (Borg dyspnoea scale) and cough (Leicester Cough Questionnaire, Cough Symptom Score)., Ethics and Dissemination: Ethical approvals were gained in April 2012. Palliative care research is a developing field, but there has been limited focus on IPF. We anticipate that the results of the study will enable healthcare professionals to provide appropriate palliative care across the trajectory for individuals with the disease, and their carers, and we therefore aim to disseminate via relevant respiratory and palliative care journals and conferences. We will also support the lay representative involved in the project to disseminate the findings to patient groups.