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BACKGROUND AND PURPOSE: Invasive and non-invasive mechanical ventilation are therapeutic options in patients with amyotrophic lateral sclerosis (ALS). Related to known national ALS incidence figures, the study aims to examine gender aspects versus physiological data in patients with ALS commencing mechanical ventilation. METHODS: ata from two nationwide registers involving all patients with ALS in Norway and Sweden who started invasive and non-invasive mechanical ventilation during 2002-2007. RESULTS: The total ALS population on invasive and non-invasive mechanical ventilation comprised n = 308 subjects [Norway n = 96 (72% men), Sweden n = 212 (69% men)]. Compared to Swedish ALS incidence figures, our finding of a male/female ratio of 2.3/1 in patients with ALS on invasive and non-invasive mechanical ventilation shows a statistically significant male predominance in the use of mechanical ventilation (P-value 0.0084 Chi square). Only 6.7% of men and 3.8% of women had invasive (via tracheotomy) ventilation (P = 0.344). Initiation of mechanical ventilation was acute (not planned) in 18% of patients (no gender difference). Age distribution (mean age 62), pulmonary function tests (FVC%pred, FEV(1) %pred), daytime blood gas analyses (PaO(2), PaCO(2)) and survival revealed no statistically significant gender differences. CONCLUSION: In Norwegian and Swedish patients with ALS on invasive and non-invasive mechanical ventilation, two-thirds were men. Associated with known national ALS male/female incidence figures, our finding shows that statistically significantly more men than women with ALS are using mechanical ventilation. Physiological data and survival were equal in both genders. This may indicate the need for a more aggressive approach to stimulate mechanical ventilation in female patients with ALS.

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Measurements of health-related quality of life (HRQL) have not been reported in patients with chronic alveolar hypoventilation (CAH) before starting home mechanical ventilation. The purpose of this study was to investigate quality of life in a population of such patients. Forty-four consecutive patients with CAH due to previous polio, scoliosis, healed pulmonary tuberculosis or neuromuscular disease answered a battery of condition specific and generic (Sickness Impact Profile, Hospital Anxiety and Depression scale, Mood Adjective Check List) self-report questionnaires. Spirometry, arterial blood gases and overnight oxygen saturation were measured. Patients with untreated CAH had significantly impaired HRQL compared to historical data from a healthy reference population. Sleep-related problems were frequent. Age, underlying disease, and standard bicarbonate correlated significantly with HRQL measures, albeit with modest levels of explained variance (8-37%). Patients with chronic alveolar hypoventilation due to neuromuscular or restrictive chest wall disorders had severely impaired health-related quality of life. Age, the underlying disease and severity of hypoventilation are each related to the health-related quality of life decrements. Health-related quality of life measurements add important information to traditional clinical observations.

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The Swedish Society of Chest Medicine has started a national register of patients on home mechanical ventilation, to establish reliable national prevalence data and to accurately document patient and treatment characteristics to enable a scientific evaluation of this treatment. In this first collection of retrospective register data, covering patients on home mechanical ventilation at the register start on 1 January 1996, we found 541 patients, corresponding to 6.1/100000 inhabitants, using home mechanical ventilation. Non-invasive ventilation, night-time ventilation and volume controlled ventilation dominated. We found four diagnosis categories of approximately equal size, namely post-polio, chest wall deformities, neuromuscular diseases and 'other diseases'. The age distribution was bimodal, with one small peak in the 20-29 year group and a large peak in the 60-69 year group. A survey of Danish patients on home mechanical ventilation showed that they were considerably younger and that almost half of them suffered from neuromuscular diseases. Further work will be done to follow the situation in Sweden and in Denmark to elucidate the obvious differences in the selection of patients for home mechanical ventilation.

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The Swedish prevalence of home mechanical ventilation is 8.2 per 100.000 with 10% annual increase. There is a large span (20 vs 2 per 100.000) between "top level" and "low level" counties, in spite of Sweden's homogeneous publicly financed system for provision of health care. The largest prevalence difference was found in patients with obstructive sleep apnoea syndrome (Pickwickian type), but their blood gas and lung function data were identical in top-level vs low-level counties. These data refute the hypothesis of overprescription in top-level counties. We conclude that the most probable explanation is under-recognition of patients in low-level counties.

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The object of this investigation was to prospectively study the annual decline in lung function in a cohort of postpolio patients, and to determine the usefulness of initial lung function tests in the prediction of a subsequent poor outcome. Cross-sectional data were analysed in 55 patients from the total cohort of 350 survivors of poliomyelitis in our admission area of 550,000 inhabitants. Longitudinal data (> 5 yrs, average 8.9 yrs) were available for 31 patients. Seventeen of the patients had a poor outcome (13 were started on domiciliary artificial ventilation and five died from respiratory failure; with one overlap). At the time of entry to the study (on average 4.3 years before the poor outcome), these patients had a lower vital capacity (VC) (43 vs 65% of predicted; p < 0.01) and arterial oxygen tension (Pa,O2) (9.9 vs 11 kPa; p < 0.05) and a higher arterial carbon dioxide tension (Pa,CO2) (6.0 vs 5.0 kPa; p < 0.01). They also had a more rapid increase in Pa,CO2 (0.3 vs 0.03 kPa.yr-1; p < 0.01), but the difference in decline in VC (40 vs 30 mL.yr-1) was not significant. Initial VC < 50% of predicted and/or Pa,CO2 > 6 kPa was associated with a poor prognosis. In conclusion, annual decline in vital capacity was not abnormally rapid but annual increase in arterial carbon dioxide tension was higher in patients with a poor outcome. Initial determination of vital capacity and initial and repeated blood gas analysis appear to be useful in identifying high-risk postpolio patients.

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Long-term domiciliary ventilation is an effective treatment for chronic hypoventilation due to neuromuscular disease or thoracic deformity. As the majority of patients require assisted ventilation only at night, it is a simple means of improving quality of life, and even sometimes of prolonging life. According to a nationwide enquiry organised by the Swedish Thoracic Society in 1993, 460 patients (5.5 per 100,000 of the population) were using domiciliary ventilation (almost a doubling of the figure of 250 patients reported from a similar survey in 1990). The most prevalent indications were poliomyelitis sequelae (30%), followed by myopathy (21%), idiopathic scoliosis (13%) and tuberculosis sequelae (13%). Eighty per cent of the patients used assisted ventilation only at night, and 60 per cent used non-invasive devices such as a nasal mask.

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Domiciliary ventilation has been used for 40 years, and is an effective treatment for chronic hypoventilation and thoracic deformity. Most patients only require assisted respiration at night, and are able to live quite normal lives during the day. The demand for domiciliary ventilation is expected to increase in all the Nordic countries. The articles in this special feature issue show that there are manifest national differences in the extent of domiciliary ventilation services, not only due to organisational differences but also due to differences in attitudes to the treatment as used in chronic progressive neurological diseases and chronic lung diseases. Current figures for the prevalence per 100,000 of the population are 3.5 for Denmark, 2.4 for Finland, 6.7 for Iceland, 2.3 for Norway and 5.5 for Sweden. The service is centralised in Denmark and Iceland, but less centralised in the remaining Nordic countries. Traditionally, it has been anaesthesiologists and specialists in infectious diseases who have shown interest in the service, and more recently interest among specialists in respiratory diseases has increased along with the increase in our knowledge of sleep-related breathing disorders. The use of nasal masks is becoming increasingly common. In Denmark many children and adolescents with muscular dystrophy, myopathy, etc, are referred to the two respiratory support units run in conjunction with the respective intensive care services at Copenhagen and Arhus, for assessment as candidates for domiciliary ventilation. These centres also train personal aides and relatives so that patients will be able to manage more or less on their own.(ABSTRACT TRUNCATED AT 250 WORDS)

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BACKGROUND: The hypothesis that adrenaline can augment and/or prolong the antitussive effect of nebulised lignocaine was examined. METHODS: The effect of inhaled lignocaine alone (20 mg) and in combination with adrenaline (400 micrograms) was studied on capsaicin-induced cough in 10 healthy subjects. RESULTS: Cough was significantly reduced between five and 25 minutes by lignocaine. Adrenaline alone had no inhibitory effect and it neither augmented nor prolonged the antitussive effect of lignocaine. The subjective anaesthesia by lignocaine was short lasting (less than 15 minutes) and not altered by adrenaline, suggesting different sensory mechanisms for anaesthesia and cough suppression. Plasma concentrations of lignocaine were low (< 30 ng/ml), not altered by adrenaline, and did not correlate with the local anaesthetic or the antitussive effect. CONCLUSIONS: Lignocaine acts locally in the oropharynx and airways and adrenaline does not alter the effect or absorption of nebulised lignocaine on the human respiratory mucosa.

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The aim of this study was to measure the short-term effects on mucus clearance after forced expiratory technique (FET) combined with either postural drainage (PD) or positive expiratory pressure breathing (PEP) on two different days. We also wanted to assess the patient's preference to the two methods. We measured mucociliary clearance at rest and during physiotherapy in 14 patients with chronic obstructive pulmonary disease. The subjects inhaled an aerosol containing 99mTc-labelled albumin colloid. Five sets of scintigraphic images were obtained with 22-min intervals. Lung retention of radioactivity was quantified using a gamma camera and the clearance of particles from the lungs calculated for each 22-min period. The first image was obtained directly after inhalation, the second after a period of 22 min rest, the third after physiotherapy and the fourth and fifth images after further periods of rest. Clearance from the whole lung and from central and peripheral regions were much greater after physiotherapy than at rest. Clearance during PD+FET was significantly higher than during PEP+FET in the total lung field (P < 0.05) and in the peripheral region (P < 0.004). The patients found the two methods equally efficient but most of the patients preferred PEP as a treatment.

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We have evaluated the properties of capsaicin as a selective cough-inducing agent in healthy human subjects. Despite frequent coughing, the subjects could inhale repeated breaths of capsaicin aerosol during 60 s without difficulty. Cough started immediately on inhalation and was most intense during the first 30 s. Cough always disappeared promptly when the capsaicin inhalation was terminated. The cough response was well reproducible and concentration-dependent up to 10 microM; at higher concentrations there was a distinct plateau of the cough response. Specific airway conductance was not changed 3 min after 50 microM capsaicin. Capsaicin (> or = 10 microM) had a burning taste, but there were no visual signs of pharyngitis or laryngitis. Citric acid (nebulized solutions 0.125 to 32%) had a choking effect and could be administered only as single breaths. There was no correlation between the cough response to citric acid and to capsaicin. Inhaled lidocaine (20 and 80 mg from nebulized solutions) caused a dose-dependent inhibition of capsaicin-induced cough. Lidocaine suppressed citric acid-induced cough as effectively as capsaicin-induced cough. In conclusion, we have characterized capsaicin-induced cough and demonstrated that it can be a useful tool in the study of cough reactivity and for evaluation of antitussive agents in humans. Capsaicin may be complementary to citric acid and may offer experimental advantages over this traditional tussive stimulus.

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STUDY OBJECTIVE: To determine whether formoterol, a new beta 2 agonist with experimentally documented long duration, is clinically more effective than salbutamol in the maintenance treatment of chronic asthma. DESIGN: Randomized double-blind between-patient comparison between treatment with formoterol and with salbutamol during four weeks. SETTING: Asthma/allergy department in a university hospital. PATIENTS: Thirty-seven patients with chronic stable asthma, who during a two-week run-in period with inhaled salbutamol, 4 x 100 micrograms twice a day, used at least four additional doses (100 micrograms each) daily, were randomly assigned to use either formoterol or salbutamol. Thirty-five patients were evaluated for efficacy. One early withdrawal and one dropout were found in the salbutamol group. The groups were similar with respect to demographic data and baseline lung function. INTERVENTIONS: During the four-week study period, the patients used either formoterol (4 x 6 micrograms twice a day and as necessary, n = 19) or salbutamol (4 x 100 micrograms twice a day and as necessary, n = 16). Inhaled steroids and orally administered theophylline were allowed if doses were kept constant. MEASUREMENTS AND MAIN RESULTS: The median number of additional doses per 24 h (median of two weeks) of the test aerosols was 0 (range, 0 to 6) for formoterol and 4 (range, 0 to 14) for salbutamol (p less than 0.01). Morning and evening PEFRs were 422 (SEM = 31) and 443 (SEM = 30), respectively, for formoterol, and 335 (SEM = 30) and 360 (SEM = 26), respectively, for salbutamol (p = 0.05 for both). Formoterol was superior (p less than 0.05) to salbutamol with respect to control of asthma symptoms, estimated duration of action and patient preference. Side effects did not differ. CONCLUSIONS: Inhaled formoterol administered twice a day and as necessary was clinically more effective than the same regimen of salbutamol.

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There have been few studies examining the relationship between NOD and mortality in patients with COPD and none examining this relationship in those patients with a daytime PaO2 greater than 60 mm Hg. Is NOD related to early death, and if so, should nocturnal supplemental oxygen be considered as therapy for altering survival? We examined survival in 169 COPD subjects. Two definitions were used to classify subjects as NOD and non-NOD, one considering episodic desaturation associated mainly with REM sleep (definition 1) and one considering greater than 30 percent of time in bed spent below an SaO2 of 90 percent (definition 2) to be significant. Survival corrected for age was significantly better in non-NOD subjects. However, when stratified for supplemental oxygen use, survival remained better only in subjects separated by definition 1. There was a trend toward increased survival in 35 oxygen-treated vs 38 non-oxygen-treated NOD subjects (definition 1), but this difference was not statistically significant.

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