Starting HMV at home: a reasonable option for many patients?

BACKGROUND AND OBJECTIVE: In the current study, we undertook a more detailed exploration of the reasons why patients undergoing HMV were screened out of a recently published study in order to better understand how applicable home initiation of HMV is under real life conditions. METHODS: All referred patients who had an indication for starting HMV were screened to participate in the Homerun study. In this trial 512 patients were screened out of the study. Those patients not enrolled in the trial were divided into the following 3 groups: (1) those not fulfilling the inclusion criteria; 2) those meeting the exclusion criteria and 3) those excluded on the basis of medical or organisation reasons. Each group was then further divided into those who would likely have been suitable for initiation of HMV at home in real world practice and those who were unsuitable. RESULTS: Based on inclusion criteria (group 1) 116 patients could not start HMV in real life, while this was 245 patients in the study. Based on the exclusion criteria (group 2) 11 patients could not start in real life while this was 79 in the study. One hundred and eighty-eight could not be enrolled in the study due to medical and organisational reasons ( group 3), while in real life this was only 95. CONCLUSION: This study indicates that more than 55% of patients who did not participate in the Homerun study could have started HMV at home in real life.

Home mechanical ventilation: the Dutch approach.

In the Netherlands we have an unique organisation of only 4 centres being responsible for all patients who need Home Mechanical ventilation(HMV). Nationwide criteria for referral and initiation of HMV are stated in our national guideline and recently a unique national learning management system (LMS) for all caregivers and professionals was developed. A nationwide multi-centric research program is running and every centre is participating. In this paper we provide information about the evolution of HMV in the Netherlands during the last 30 years, including details about the number of patients, different diagnose groups, residence and the type of ventilators.

Recent increase of ulcerative lesions caused by Anisakis spp. in cetaceans from the north-east Atlantic.

Species of Anisakis typically infect the stomach of cetaceans worldwide, often causing ulcerative lesions that may compromise the host's health. These nematodes also cause anisakiasis or allergic reactions in humans. To assess the risks of this emerging zoonosis, data on long-term changes in Anisakis infections in cetaceans are necessary. Here, we compare the prevalence and severity of ulcerative lesions caused by Anisakis spp. in five cetacean species stranded along the north-west Spanish coast in 2017-2018 with published data from 1991-1996. Open ulcers were found in 32/43 short-beaked common dolphins, Delphinus delphis; 3/5 striped dolphins, Stenella coeruleoalba; 1/7 bottlenose dolphins, Tursiops truncatus; and 1/3 harbour porpoises, Phocoena phocoena meridionalis; a single individual of long-finned pilot whale, Globicephala melas, was found uninfected. In common dolphins, the mean abundance of open ulcers per host was 1.1 (95% confidence interval: 0.8-1.3), with a maximum diameter (mean ± standard deviation) of 25.4 ± 16.9 mm. Stomachs with scars or extensive fibrosis putatively associated with Anisakis were detected in 14 and five animals, respectively. A molecular analysis based on the mitochondrial cytochrome c oxidase II gene using 18 worms from three cetacean species revealed single or mixed infections of Anisakis simplex sensu stricto and Anisakis pegreffii. Compared with the period 1991-1996, we found a strong increase of prevalence, abundance and extension of ulcerative lesions in most cetacean species. Anisakis populations could have increased in the study area over the last decades, although we cannot rule out that a higher environmental stress has also boosted the pathological effects of these parasites.

Is chronic ventilatory support really effective in patients with amyotrophic lateral sclerosis?

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.

Facioscapulohumeral muscular dystrophy and respiratory failure; what about the diaphragm?

INTRODUCTION: We present a case of facioscapulohumeral muscular dystrophy (FSHD) with a diaphragm paralysis as the primary cause of ventilatory failure. FSHD is an autosomal dominant inherited disorder with a restricted pattern of weakness. Although respiratory weakness is a relatively unknown in FSHD, it is not uncommon. METHODS: We report on the clinical findings of a 68-year old male who presented with severe dyspnea while supine. RESULTS: Supplementing our clinical findings with laboratory, electrophysiological and radiological performances led to the diagnosis of diaphragm paralysis. Arterial blood gas in sitting position without supplemental oxygen showed a mild hypercapnia. His sleep improved after starting non-invasive ventilation and his daytime sleepiness disappeared. DISCUSSION: We conclude that in patients with FSHD who have symptoms of nocturnal hypoventilation, an adequate assessment of the diaphragm is recommended. This is of great importance as we know that nocturnal hypoventilation can be treated effectively by non-invasive ventilation.

Initiation of home mechanical ventilation at home: a randomised controlled trial of efficacy, feasibility and costs.

INTRODUCTION: Home mechanical ventilation (HMV) in the Netherlands is normally initiated in hospital, but this is expensive and often a burden for the patient. In this randomised controlled study we investigated whether initiation of HMV at home in patients with chronic respiratory failure is non-inferior to an in hospital based setting. METHODS: Seventy-seven patients were included, of which 38 patients started HMV at home. All patients were diagnosed with chronic respiratory failure due to a neuromuscular or thoracic cage disease. Primary outcome was the arterial carbon dioxide (PaCO2) while quality of life and costs were secondary outcomes. Telemonitoring was used in the home group to provide therapeutic information, for example; transcutaneous carbon dioxide, oxygen saturation and ventilator information, to the caregivers. Follow-up was six months. RESULTS: PaCO2, improved by 0.72 (SE ± 0.16) kPa in the hospital group and by 0.91 (±0.20) in the home group, both improvements being significant and the latter clearly not inferior. There were also significant improvements in quality of life in both groups, again not being inferior with home treatment. CONCLUSION: 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.

[Measurement of quality of life following stapes surgery]. / Lebensqualitätbewertung nach stapesoperationen.

BACKGROUND: The objectives of this study were to compare a German version of the Glasgow Benefit Inventory (GBI) with the original English version and to describe quality of life following stapes surgery. PATIENTS AND METHODS: Audiometry and a questionnaire on handicap in various listening situations were used to assess 36 patients with otosclerosis before and 6 months after stapes surgery. The GBI was used to estimate the change in quality of life following surgery. RESULTS: Postoperative air-bone gap closure was <10 dB in 71% of patients. The postoperative air conduction threshold was <30 dB in 48% of patients. Using the GBI, the mean benefit score was 28 and the general benefit score 42. The social support and physical health scores were both zero. Where the postoperative improvement in air conduction was >15 dB, a significant reduction in handicap in everyday life, watching TV, listening to the radio and making telephone calls was seen. CONCLUSIONS: The German version of the GBI showed an improvement in quality of life in various everyday situations following stapes surgery.

Validation of a transcutaneous CO(2) monitor in adult patients with chronic respiratory failure.

BACKGROUND: Home mechanical ventilation is usually started in hospital as arterial blood gas sampling is deemed necessary to monitor CO(2) and O(2) adequately during institution of ventilatory support. A non-invasive device to reliably measure CO(2) 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 CO(2) (PtcCO(2)) measurements, performed continuously during the night, reliably reflect arterial CO(2) (PaCO(2)) 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 CO(2) pressure measurements, following the analysis by Bland and Altman, were determined as acceptable. RESULTS: Mean PtcCO(2) was 0.4 kPa higher (LOA -0.48 to 1.27 kPa) than mean PaCO(2) 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 PtcCO(2) versus PaCO(2) were not significant (ANOVA). CONCLUSIONS: PtcCO(2) measurement, using TOSCA, is a valid method showing an acceptable agreement with PaCO(2) during 8 h of continuous measurement. Therefore, this device can be used to monitor CO(2) adequately during chronic ventilatory support.

Recurrent neck abscess due to a bronchogenic cyst in an adult.

BACKGROUND: Neck abscesses can originate from congenital cervical cysts. Cervical cysts of bronchogenic origin are rare and often asymptomatic. Common symptoms of bronchogenic cysts are stridor, dyspnoea and dysphagia. The reported patient represents the second published case of a bronchogenic cyst causing a neck abscess in an adult. CASE REPORT: We report a case of a cervical bronchogenic cyst presenting as a recurrent supraclavicular abscess in a middle-aged woman. During extirpation, a fistula was demonstrated to the right upper lobe of the lung, suspected because the cyst inflated synchronously with respiration. DISCUSSION: The symptoms of bronchogenic cysts are due to the effects of compression or fistulas. In the majority of these cysts, a thorough investigation involving history, examination and radiological imaging does not clearly demonstrate a fistula. Therefore, extirpation is both diagnostic and therapeutic. CONCLUSION: A bronchogenic cyst is a very rare cause of a recurrent deep neck abscess. Total extirpation is the treatment of choice.

Mechanisms involved in symptomatic myocardial bridging: Value of sequential testing for endothelial function, flow reserve measurements and dobutamine stress angiography.

BACKGROUND: In patients with extensive myocardial bridging, evaluation of its clinical significance remains a challenge. HYPOTHESIS: Sequential invasive testing is feasible and gives more insight into the pathophysiological mechanism of bridging-related angina. METHODS: Twelve patients with chest pain, proven ischaemia and extensive myocardial bridging were assessed. Myocardial bridging was evaluated at rest, during intracoronary acetylcholine infusion, through coronary flow velocity and flow reserve measurements, and during dobutamine stress. RESULTS: The mean length of the bridging segment was 24.9 mm (QCA; range 8.4-48.0 mm). Acetylcholine infusion caused severe vasospasm in two patients. In these two patients anginal symptoms were related to vasospasm and sequential testing was discontinued. In the remaining ten patients sequential testing was continued. Coronary flow reserve was normal in all patients: 3.3+/-0.6. In six patients reliable quantitative measurements could be performed during dobutamine stress. The mean systolic diameter of the bridging segment was 1.6+/-0.4 at baseline and 1.3+/-0.3 during dobutamine stress (mean of differences 0.38 (95% CI 0.1-0.7)). The difference between the diastolic and systolic diameter in the bridging segment increased from 0.3+/-0.2 mm at baseline to 1.0+/-0.5 mm during dobutamine infusion (mean of differences 0.6 (95% CI 0.3 to 0.9)). CONCLUSION: Sequential testing for bridging is feasible and may disclose endothelial dysfunction or spasm as an underlying mechanism in a minority of patients. Coronary flow reserve was preserved. Dobutamine stress unmasked further lumen reduction and may give further insight into the clinical significance of myocardial bridging in individual patients. (Neth Heart J 2008;16:10-5.).

[Diagnostic image (271). A woman with blue sclerae, brittle bones and hearing loss]. / Diagnose in beeld (271). Een vrouw met blauwe sclerae, broos beenstelsel en gehoorklachten.

A 51-year-old woman presented with blue sclerae, brittleness of the bones and hearing loss due to osteogenesis imperfecta with ear involvement: Van der Hoeve-De Kleyn syndrome.

Walking with continuous positive airway pressure.

A ventilator-dependent child had been in the paediatric intensive care unit (PICU) ever since birth. As a result, she had fallen behind considerably in her development. After 18 months, continuous positive airway pressure was successfully administered via a tracheostomy tube with a novel lightweight device. This enabled her to walk in the PICU. With this device, the child was discharged home where she could walk with an action range of 10 m. Subsequently, her psychomotor development improved remarkably. To the authors' knowledge, this is the first case report of a patient, adult or paediatric, who could actually walk with a sufficient radius of action while receiving long-term respiratory support.