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Context: Prader-Willi syndrome (PWS) is a rare, genetic, multisymptom, neurodevelopmental disease due to lack of the expression of the paternal genes in the q11 to q13 region of chromosome 15. The main characteristics of PWS are muscular hypotonia, hyperphagia, obesity, behavioral problems, cognitive disabilities, and endocrine deficiencies, including growth hormone (GH) deficiency. Sleep apnea and abnormal sleep patterns are common in PWS. GH treatment might theoretically have a negative impact on respiration., Objective: Here we present the effect of GH treatment on polysomnographic measurements., Methods: Thirty-seven adults, 15 men and 22 women, with confirmed PWS were randomly assigned to 1 year of GH treatment (n = 19) or placebo (n = 18) followed by 2 years of GH treatment to all. Polysomnographic measurements were performed every 6 months. A mixed-effect regression model was used for comparison over time in the subgroup that received GH for 3 years., Results: At baseline median age was 29.5 years, body mass index 27.1, insulin-like growth factor 115 µg/L, apnea-hypopnea index (AHI) 1.4 (range, 0.0-13.9), and sleep efficiency (SE) 89.0% (range, 41.0%-99.0%). No differences in sleep or respiratory parameters were seen between GH- and placebo-treated patients. SE continuously improved throughout the study, also after adjustment for BMI, and the length of the longest apnea increased. AHI inconsistently increased within normal range., Conclusion: SE improved during GH treatment and no clinical, significantly negative impact on respiration was seen. The etiology of breathing disorders is multifactorial and awareness of them should always be present in adults with PWS with or without GH treatment., (© The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society.)

Background: Users of home mechanical ventilation encounter major psychological and physiologic challenges. To ensure well-functioning home mechanical ventilation, users' experiences of care and treatment are important knowledge to supplement clinical perspectives. This systematic review aimed to summarize current qualitative evidence regarding experiences of home mechanical ventilation users., Methods: By following Preferred Reporting Items for Systematic Review and Meta-Analyses guidelines, 9 databases were systematically searched. Seven studies met the inclusion criteria after title and/or abstract screening and full-text assessment. These were appraised by using the Relevance, Appropriateness, Transparency, Soundness checklist. Thematic analysis guided data extraction and identification of the findings. The Confidence in the Evidence for Reviews of Qualitative Research tool was applied to assess the confidence of the findings., Results: The review showed high confidence in 4 findings: an increase in quality of life, feeling forced to accept home mechanical ventilation, collaboration between home-care assistants and users of home mechanical ventilation is challenging, and information about the technology from a user's perspective. The review showed moderate confidence in 2 findings: living at home is pivotal for a normalized everyday life, and home mechanical ventilation causes a life with continued worries and uncertainty., Conclusions: According to the users, treatment by home mechanical ventilation resulted in increased well-being and facilitated a community- and home-based lifestyle compared with institutional-based treatment. However, the users also expressed difficulties in coming to terms with the necessary extensive surveillance, which gave rise to a sense of undermined autonomy and self-determinism as well as continued worries and uncertainty. The users called this situation dependent independency. As a result of the review we call for an increased focus on a patient-centered treatment and care., Competing Interests: The authors have disclosed no conflicts of interest., (Copyright © 2019 by Daedalus Enterprises.)

There is emerging evidence that feedback techniques based on contingent electrical stimulation (CES) have an inhibitory effect on the electromyogram (EMG) activity of jaw-closing muscles and therefore could be useful in the management of sleep bruxism. This polysomnographic (PSG) study was designed to investigate the effect of CES on PSG parameters in subjects with self-reported bruxism. Fourteen subjects underwent a full PSG investigation in the laboratory for three consecutive nights - one night of adaptation, one night without CES, and one night with CES - in a randomized order. During all sessions the EMG activity was recorded by a portable feedback device from the temporalis muscle. An electrical pulse, which was adjusted to a moderate, but non-painful, intensity, was applied to subjects during the session with CES, if jaw-muscle activity was detected. The total sleep time, the number of micro-arousals per hour of sleep, the time spent in sleep stages 3 and 4 and in rapid eye movement (REM) sleep, and the number of periodic limb movements, were not influenced by CES. The number of EMG episodes per hour of sleep during the nights with and without CES was not significantly different. The present study suggests that CES at non-painful intensities does not cause major arousal responses in any of the sleep parameters assessed in this study., (© 2011 Eur J Oral Sci.)

The use of noninvasive ventilation (NIV)/ventilatory assistance has in its modern form experienced a resurgence during the last two decades, primarily in the adult population. During the last few years, NIV, predominantly in the form of positive-pressure ventilation/ventilatory assistance, has also become an option in the paediatric population. Although the technique is increasingly being applied, data in this often very heterogeneous group of patients are still very limited, and are usually derived from case series and not randomised controlled studies. Available experience indicates that the technique is useful for children with a wide spectrum of hypercapnic and/or hypoxaemic respiratory disorders, in the acute as well as in the chronic setting. Results have shown improvements in survival and arterial blood gases, and have indicated better quality of life. Adverse effects are generally minor, although the impact on facial bony structures should be monitored closely during long-term ventilation. Considering the paucity of data, the area badly needs research, which preferentially should include: 1) short- and long-term effects and adverse effects in various conditions and age groups; 2) techniques, in particular interfaces and triggering mechanisms; 3) comprehensive plans for training of attendants, discharge and follow-up; and 4) quality of life measurements. As many of the conditions suited for noninvasive ventilation are very rare, much of the research would probably benefit from multicentre studies, or even studies on a European level.

Introduction: Positive expiratory airway pressure seems to dilate narrowed or collapsed airways, but this may be accompanied by a maintained and harmful increase in resting lung volume in obstructive pulmonary disease., Purpose: To evaluate the influence of inhaled terbutaline and positive expiratory pressure (PEP) on airway resistance (Raw) and functional residual capacity (FRC) in bronchial asthma., Design: Randomized crossover design, single blind with regard to inhaled medication, open with regard to PEP (PEP can be felt)., Material and Methods: Ten patients with bronchial asthma inhaled placebo and terbutaline in doses of 0.125 mg, 0.5 mg, and 1.5 mg by cone spacer combined with a facemask giving 0, 10, or 15 cm H2O PEP on separate days. FRC and Raw were measured by body plethysmography before and after inhalations. Data were analyzed by analysis of variance with terbutaline dose and PEP as factor levels., Results: The effect of terbutaline: Raw decreased significantly (p < 0.0001) after 0.125 mg and 1.5 mg. The FRC did not change significantly. The effect of PEP: Raw decreased, but significantly only when the dose of 1.5 mg terbutaline was excluded from the analysis. Raw decreased with PEP 10 and 15 cm H2O, mean 0.6 (95 percent CI: -1.1, -0.2) and 0.9 (95 percent CI: -1.3, -0.4) cm H2O/L/s. The FRC did not change significantly with the PEP level., Conclusion: PEP only had influence on Raw when insufficient doses of terbutaline were inhaled, whereas once an efficient dose of terbutaline was administered, significant bronchodilation was achieved with or without PEP. Positive expiratory pressure did not increase FRC.

The purpose of this study was to evaluate the in- and expiratory pressures achieved by the use of different continuous positive airway pressure (CPAP) delivery systems. The study was performed on ten healthy adults breathing spontaneously with: 1) "simple CPAP", consisting of a fresh gas flow around 40 l/min and reservoir balloons of 2, 6 and 10 l and expiration against a water column, 2) a high-flow system CR60 CPAP, designed for home treatment and 3) another high-flow system Down's CPAP. Both high-flow systems used a venturi device (Downs Flow Generator, Vital Signs) giving flows around 90 and 170 l/min in our set-up, and the same PEEP valves (Vital signs) were used. All systems were tested with expiratory pressures set at 5, 10 and 20 cm H2O. Individual responses were found, but in general the simple CPAP with a 10 l latex balloon reservoir gave optimal CPAP. The high-flow systems were not found to provide better CPAP, however, CR60 CPAP was found also to be optimal with 5 and 10 cm H2O. Down's CPAP provided 3-5 cm higher expiratory pressure than the intended CPAP level. We recommend measurement of the pressure during the CPAP treatment to ensure that the intended CPAP is achieved.

The influence of positive expiratory pressure (PEP) applied during inhalation of a beta 2-agonist in treatment of bronchial asthma was investigated in a randomized crossover study with two-week treatment periods. In one period, two puffs (0.5 mg) of terbutaline was given from a metered dose inhaler and inhaled through a device consisting of a conespacer connected to a facemask giving PEP (10 to 15 cm H2O). In a second period, terbutaline 0.5 mg was inhaled similarly but without PEP, and in a third period placebo spray was inhaled with PEP. Treatments were given three times daily. Peak expiratory flow (PEF) was measured before and after each inhalation and symptom scores for dyspnea, cough, and mucus production were noted in a diary. All treatments increased PEF significantly (p less than 0.0001). The mean increase was 32 L/min during treatment with terbutaline and PEP. This was greater than the increase of 25 L/min during terbutaline treatment (p = 0.005). The increase in PEF during terbutaline treatment was significantly higher than the achieved 18 L/min during PEP (p = 0.026). The study showed improved bronchodilation when PEP was combined with inhalation of beta 2-agonist compared with beta 2-agonist alone.

The effect of positive expiratory pressure (PEP) and inhaled terbutaline was evaluated in ten patients with chronic obstructive pulmonary disease in a randomized cross-over study with three 2 weeks periods. In one period 5 mg terbutaline was inhaled 3 times daily from a nebulizer combined with PEP. In a second period 5 mg terbutaline was inhaled similarly but without PEP and in a third period placebo inhalations were combined with PEP. Symptom score and peak expiratory flow (PEF) measured before and after each inhalation was noted in a diary. The treatment with nebulized terbutaline combined with PEP gave the best relief in symptoms. All treatments increased PEF significantly. PEP alone gave the least increase in PEF (25 1/min), and there was no difference between the increase in PEF after terbutaline inhaled with PEP (28 1/min) compared to terbutaline alone (29 1/min).

Immunotherapy (IT) in venom-allergic patients has been demonstrated to provide a highly efficient protection from severe reactions to a re-sting. It is not known whether this protection will persist after IT is stopped in patients with remaining venom sensitivity. In 25 adult patients with a previous severe systemic reaction to a Hymenoptera insect sting, 28 in-hospital sting challenges after stopping IT resulted in no systemic reactions. The mean duration of venom IT was 42.8 months (range, 36 to 83 months), and the mean time interval from the time IT was stopped until sting challenge was, in mean, 25.2 months (range, 12 to 36 months). The mean venom-specific IgE after stopping IT was 2.4 PRU/ml (range, 0 to 16.9 PRU/ml), at the day of sting challenge, 2.7 PRU/ml (range, 0 to 22 PRU/ml), and 2 weeks later, 2.4 PRU/ml (range, 0.02 to 27.8 PRU/ml). These changes in IgE were not significant. Venom-specific IgG concentration when IT was stopped was 64 PU/ml (range, 20 to 144 PU/ml), decreasing significantly to the day of sting challenge to 41.1 PU/ml (range, 13 to 84 PU/ml), and 2 weeks later, a significant increase to a mean of 53.5 PU/ml (range, 12 to 117 PU/ml) was found. Our results suggest that venom IT may be stopped after 3 years, regardless of the level of specific antibodies. To confirm this finding, a larger number of patients should be studied.

In a prospective study of traumatic lesions of the volar fibrocartilage of the PIP joint, 56 patients were randomized to treatment by immobilization for 3 weeks and 56 patients, to purely analgetic treatment without immobilization. Seventy-eight patients were reexamined 6 months later and 77 patients, in a follow-up study 2-3 years after the injury. No difference was found between the two groups with respect to subjective complaints or objective signs.