IntroductionChronic non-invasive ventilation (NIV) has become evidence-based care for stable hypercapnic COPD patients. While the number of patients increases, home initiation of NIV would greatly alleviate the healthcare burden. We hypothesise that home initiation of NIV with the use of telemedicine in stable hypercapnic COPD is non-inferior to in-hospital NIV initiation.MethodsSixty-seven stable hypercapnic COPD patients were randomised to initiation of NIV in the hospital or at home using telemedicine. Primary outcome was daytime arterial carbon dioxide pressure (PaCO2) reduction after 6 months NIV, with a non-inferiority margin of 0.4 kPa. Secondary outcomes were health-related quality of life (HRQoL) and costs.ResultsHome NIV initiation was non-inferior to in-hospital initiation (adjusted mean difference in PaCO2 change home vs in-hospital: 0.04 kPa (95% CI −0.31 to 0.38 kPa), with both groups showing a PaCO2 reduction at 6 months compared with baseline (home: from 7.3±0.9 to 6.4±0.8 kPa (p<0.001) and in-hospital: from 7.4±1.0 to 6.4±0.6 kPa (p<0.001)). In both groups, HRQoL improved without a difference in change between groups (Clinical COPD Questionnaire total score-adjusted mean difference 0.0 (95% CI −0.4 to 0.5)). Furthermore, home NIV initiation was significantly cheaper (home: median €3768 (IQR €3546–€4163) vs in-hospital: median €8537 (IQR €7540–€9175); p<0.001).DiscussionThis is the first study showing that home initiation of chronic NIV in stable hypercapnic COPD patients, with the use of telemedicine, is non-inferior to in-hospital initiation, safe and reduces costs by over 50%.Trial registration numberNCT02652559.

This study is the first to show that initiation of HMV at home in a selective group of patients with chronic respiratory failure is as effective for gas exchange and quality of life as hospital initiation. In addition we found that it is safe, technically feasible and that more than € 3000 per patient can be saved compared to our standard care.

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To study effectiveness of surgery and watchful waiting in localized laryngeal amyloidosis, retrospective case series. This retrospective study comprises all consecutive patients with localized laryngeal amyloidosis surgically treated in a tertiary hospital between 1994 and February 2016. Recurrence rate, revision surgery, progression to systemic amyloidosis, and changes in voice were monitored yearly. Eighteen patients were included. Seven women and eleven men had a median age 50 years (range 21–77 years) and median follow-up 6.4 years (2.4–17 years). Amyloid was located in subglottis (5), glottis (8), false vocal folds (8) and other supraglottic areas (5), in more than one laryngeal region (13) and bilaterally (12). Cold steel excision was used at the glottis; CO2 laser excision, sometimes assisted by microdebrider, at other laryngeal areas. Eleven patients needed revision surgery, ten within the first 4 years after surgical treatment. One patient needed his first revision surgery after 11 years. Five patients needed a second revision within 6 years after initial diagnosis. Two patients needed a third revision. Indications for first revision surgery were progression (8) with dysphonia (7), dyspnea (2), dysphagia (1), exclusion of malignancy (1), and aphonia (1). No patient developed systemic amyloidosis during follow-up. Although local progression of amyloid necessitates revision surgery once or twice in the first 4–6 years, progression slows down thereafter. Late progression, however, remains possible.

Most patients with amyotrophic lateral sclerosis (ALS) develop respiratory insufficiency in the advanced stage of their disease. Non-invasive ventilation (NIV) is commonly regarded to be a treatment that is effective in reducing these complaints. To assess whether the effect of NIV on gas exchange and quality of life (QOL) is different in patients with ALS versus without ALS. A post hoc analysis was done with data from a previously published trial, in which all patients were instituted on NIV. Arterial blood gasses were assessed next to QOL by generic as well as disease-specific questionnaires. 77 patients started NIV: 30 with ALS and 47 without. Both groups showed significant improvements in blood gasses after 2 and 6 months. Compared to the non-ALS group, the ALS group had significantly worse scores after 6 months in MRF-28, SRI, HADS and SF-36 than the non-ALS group. This study shows that NIV improves gas exchange, both in patients with and without ALS. QOL improves markedly more in patients without ALS than in those with ALS, in whom only some domains improve. Our observation of little or no effect in ALS patients warrants a large study limited to ALS patients only.

Laryngeal symptoms may be the presenting feature of hereditary systemic AApoAI amyloidosis, and comprehensive investigations including apoA-I genotyping are warranted in patients who present with apparently localized laryngeal amyloidosis. The distinctive appearance of the amyloidotic vocal folds described here may further signal the possibility of hereditary AApoAI type.

Background: Home mechanical ventilation is usually started in hospital as arterial blood gas sampling is deemed necessary to monitor CO₂ and O₂ adequately during institution of ventilatory support. A non-invasive device to reliably measure CO₂ transcutaneously would alleviate the need for high care settings for measurement and open the possibility for home registration. Objectives: In this study we investigated whether the TOSCA® transcutaneous CO₂ (PtcCO₂) measurements, performed continuously during the night, reliably reflect arterial CO₂ (PaCO₂) measurements in adults with chronic respiratory failure. Methods: Paired measurements were taken in 15 patients hospitalised to evaluate their blood gas exchange. Outcomes were compared 30 min, 2, 4, 6 and 8 h after attaching the sensor to the earlobe. A maximum difference of 1.0 kPa and 95% limits of agreement (LOA) of 1 kPa between CO₂ pressure measurements, following the analysis by Bland and Altman, were determined as acceptable. Results: Mean PtcCO₂ was 0.4 kPa higher (LOA –0.48 to 1.27 kPa) than mean PaCO₂ after 30 min. These figures were 0.6 kPa higher (LOA –0.60 to 1.80 kPa) after 4 h, with a maximum of 0.72 kPa (LOA 0.35 to 1.79 kPa) after 8 h. The corresponding values for changes in PtcCO₂ versus PaCO₂ were not significant (ANOVA). Conclusions: PtcCO₂ measurement, using TOSCA, is a valid method showing an acceptable agreement with PaCO₂ during 8 h of continuous measurement. Therefore, this device can be used to monitor CO₂ adequately during chronic ventilatory support.