The incidence of interstitial lung disease 1995–2005: a Danish nationwide population-based study

<p>Abstract</p> <p>Background</p> <p>Current data on incidence of interstitial lung diseases (ILDs) are sparse and concerns about an increasing trend have been raised. We examined incidence rates (IRs) of ILDs and changes in IRs between 1995 and 2005.</p> <p>Methods</p> <p>All persons with a first-time hospital discharge or outpatient diagnosis of ILD were identified through the Danish National Registry of Patients, which covers all Danish hospitals. Crude and age-standardised IRs were computed for ILD overall, as well as stratified by ILD subcategories.</p> <p>Results</p> <p>A total of 21,765 patients with ILD were identified. Between 1995 and 1998 the overall standardised IR of ILD decreased from 27.14 (95% CI 25.82–28.46) per 100,000 person-years to 19.36 (95% CI 18.26–20.46) per 100,000 person-years. After 1998 the IR increased considerably, peaking at 34.34 (95% CI 32.84–35.85) per 100,000 person-years in 2002. Subsequently there was a slight decrease. The highest IR was observed in the non-specific category "Respiratory disorders in diseases classified elsewhere". By ILD subcategory, the greatest average increase during the study period was observed in "Respiratory disorders in diseases classified elsewhere".</p> <p>Conclusion</p> <p>The incidence rate of ILD in Denmark increased during the study period, most pronounced for ILDs associated with systemic diseases.</p

Survival in severe alpha-1-antitrypsin deficiency (PiZZ)

<p>Abstract</p> <p>Background</p> <p>Previous studies of the natural history of alpha-1-antitrypsin (AAT) deficiency are mostly based on highly selected patients. The aim of this study was to analyse the mortality of PiZZ individuals.</p> <p>Methods</p> <p>Data from 1339 adult PiZZ individuals from the Swedish National AAT Deficiency Registry, followed from 1991 to 2008, were analysed. Forty-three percent of these individuals were identified by respiratory symptoms (respiratory cases), 32% by liver diseases and other diseases (non-respiratory cases) and 25% by screening (screened cases). Smoking status was divided into two groups: smokers 737 (55%) and 602 (45%) never-smokers.</p> <p>Results</p> <p>During the follow-up 315 individuals (24%) died. The standardised mortality rate (SMR) for respiratory cases was 4.70 (95% Confidence Interval (CI) 4.10-5.40), 3.0 (95%CI 2.35-3.70) for the non-respiratory cases and 2.30 (95% CI 1.46-3.46) for the screened cases. The smokers had a higher mortality risk than never-smokers, with a SMR of 4.80 (95%CI 4.20-5.50) for the smokers and 2.80(95%CI 2.30-3.40) for the never-smokers. The Rate Ratio (RR) was 1.70 (95% CI 1.35-2.20). Also among the screened cases, the mortality risk for the smokers was significantly higher than in the general Swedish population (SMR 3.40 (95% CI 1.98-5.40).</p> <p>Conclusion</p> <p>Smokers with severe AAT deficiency, irrespective of mode of identification, have a significantly higher mortality risk than the general Swedish population.</p

Are exhaled nitric oxide measurements using the portable NIOX MINO repeatable?

<p>Abstract</p> <p>Background</p> <p>Exhaled nitric oxide is a non-invasive marker of airway inflammation and a portable analyser, the NIOX MINO (Aerocrine AB, Solna, Sweden), is now available. This study aimed to assess the reproducibility of the NIOX MINO measurements across age, sex and lung function for both absolute and categorical exhaled nitric oxide values in two distinct groups of children and teenagers.</p> <p>Methods</p> <p>Paired exhaled nitric oxide readings were obtained from 494 teenagers, aged 16-18 years, enrolled in an unselected birth cohort and 65 young people, aged 6-17 years, with asthma enrolled in an interventional asthma management study.</p> <p>Results</p> <p>The birth cohort participants showed a high degree of variability between first and second exhaled nitric oxide readings (mean intra-participant difference 1.37 ppb, 95% limits of agreement -7.61 to 10.34 ppb), although there was very close agreement when values were categorised as low, normal, intermediate or high (kappa = 0.907, p < 0.001). Similar findings were seen in subgroup analyses by sex, lung function and asthma status. Similar findings were seen in the interventional study participants.</p> <p>Conclusions</p> <p>The reproducibility of exhaled nitric oxide is poor for absolute values but acceptable when values are categorised as low, normal, intermediate or high in children and teenagers. One measurement is therefore sufficient when using categorical exhaled nitric oxide values to direct asthma management but a mean of at least two measurements is required for absolute values.</p

Effect of allergen-specific immunotherapy with purified Alt a1 on AMP responsiveness, exhaled nitric oxide and exhaled breath condensate pH: a randomized double blind study

<p>Abstract</p> <p>Background</p> <p>Little information is available on the effect of allergen-specific immunotherapy on airway responsiveness and markers in exhaled air. The aims of this study were to assess the safety of immunotherapy with purified natural Alt a1 and its effect on airway responsiveness to direct and indirect bronchoconstrictor agents and markers in exhaled air.</p> <p>Methods</p> <p>This was a randomized double-blind trial. Subjects with allergic rhinitis with or without mild/moderate asthma sensitized to <it>A alternata </it>and who also had a positive skin prick test to Alt a1 were randomized to treatment with placebo (n = 18) or purified natural Alt a1 (n = 22) subcutaneously for 12 months. Bronchial responsiveness to adenosine 5'-monophosphate (AMP) and methacholine, exhaled nitric oxide (ENO), exhaled breath condensate (EBC) pH, and serum Alt a1-specific IgG₄antibodies were measured at baseline and after 6 and 12 months of treatment. Local and systemic adverse events were also registered.</p> <p>Results</p> <p>The mean (95% CI) allergen-specific IgG₄value for the active treatment group increased from 0.07 μg/mL (0.03-0.11) at baseline to 1.21 μg/mL (0.69-1.73, P < 0.001) at 6 months and to 1.62 μg/mL (1.02-2.22, P < 0.001) at 12 months of treatment. In the placebo group, IgG₄value increased nonsignificantly from 0.09 μg/mL (0.06-0.12) at baseline to 0.13 μg/mL (0.07-0.18) at 6 months and to 0.11 μg/mL (0.07-0.15) at 12 months of treatment. Changes in the active treatment group were significantly higher than in the placebo group both at 6 months (P < 0.001) and at 12 months of treatment (P < 0.0001). However, changes in AMP and methacholine responsiveness, ENO and EBC pH levels were not significantly different between treatment groups. The overall incidence of adverse events was comparable between the treatment groups.</p> <p>Conclusion</p> <p>Although allergen-specific immunotherapy with purified natural Alt a1 is well tolerated and induces an allergen-specific IgG₄response, treatment is not associated with changes in AMP or methacholine responsiveness or with significant improvements in markers of inflammation in exhaled air. These findings suggest dissociation between the immunotherapy-induced increase in IgG₄levels and its effect on airway responsiveness and inflammation.</p

Minimizing the evidence-practice gap – a prospective cohort study incorporating balance training into pulmonary rehabilitation for individuals with chronic obstructive pulmonary disease

Background: We have recently demonstrated the efficacy of balance training in addition to Pulmonary Rehabilitation (PR) at improving measures of balance associated with an increased risk of falls in individuals with Chronic Obstructive Pulmonary Disease (COPD). Few knowledge translation (KT) projects have been conducted in rehabilitation settings. The goal of this study was to translate lessons learnt from efficacy studies of balance training into a sustainable clinical service. Methods: Health care professionals (HCPs) responsible for delivering PR were given an hour of instruction on the principles and practical application of balance training and the researchers offered advice regarding; prescription, progression and practical demonstrations during the first week. Balance training was incorporated three times a week into conventional PR programs. Following the program, HCPs participated in a focus group exploring their experiences of delivering balance training alongside PR. Service users completed satisfaction surveys as well as standardized measures of balance control. At six month follow-up, the sustainability of balance training was explored. Results: HCPs considered the training to be effective at improving balance and the support provided by the researchers was viewed as helpful. HCPs identified a number of strategies to facilitate balance training within PR, including; training twice a week, incorporating an interval training program for everyone enrolled in PR, providing visual aids to training and promoting independence by; providing a set program, considering the environment and initiating a home-based exercise program early. Nineteen service users completed the balance training [ten male mean (SD) age 73 (6) y]. Sixteen patients (84 %) enjoyed balance training and reported that it helped them with everyday activities and 18 (95 %) indicated their wish to continue with it. Scores on balance measures improved following PR that included balance training (all p < 0.05). At six month follow-up balance training is being routinely assessed and delivered as part of standardised PR. Conclusions: Implementing balance training into PR programs, with support and training for HCPs, is feasible, effective and sustainable. Trail registration Clinical Trials ID: NCT02080442 (05/03/2014) Electronic supplementary material The online version of this article (doi:10.1186/s12890-015-0067-2) contains supplementary material, which is available to authorized users

Non-specific interstitial pneumonia in cigarette smokers: a CT study

The goal of this study was to seek indirect evidence that smoking is an aetiological factor in some patients with non-specific interstitial pneumonia (NSIP). Ten current and eight ex-smokers with NSIP were compared to controls including 137 current smokers with no known interstitial lung disease and 11 non-smokers with NSIP. Prevalence and extent of emphysema in 18 smokers with NSIP were compared with subjects meeting GOLD criteria for chronic obstructive pulmonary disease (COPD; group A; n = 34) and healthy smokers (normal FEV1; group B; n = 103), respectively. Emphysema was present in 14/18 (77.8%) smokers with NSIP. Emphysema did not differ in prevalence between NSIP patients and group A controls (25/34, 73.5%), but was strikingly more prevalent in NSIP patients than in group B controls (18/103, 17.5%, P < 0.0005). On multiple logistic regression, the likelihood of emphysema increased when NSIP was present (OR = 18.8; 95% CI = 5.3–66.3; P < 0.0005) and with increasing age (OR = 1.04; 95% CI = 0.99–1.11; P = 0.08). Emphysema is as prevalent in smokers with NSIP as in smokers with COPD, and is strikingly more prevalent in these two groups than in healthy smoking controls. The association between NSIP and emphysema provides indirect support for a smoking pathogenesis hypothesis in some NSIP patients

Vital capacity evolution in patients treated with the CMCR brace: statistical analysis of 90 scoliotic patients treated with the CMCR brace

<p>Summary</p> <p>Objective</p> <p>To study the evolution of pulmonary capacity during orthopaedic treatment of scoliosis with the CMCR brace.</p> <p>Background</p> <p>Investigating the impact of moderate scoliosis on respiratory capacity and its evolution during CMCR brace treatment with mobile pads.</p> <p>Context</p> <p>Several studies demonstrate the impact of scoliosis on respiratory capacity but few of them focus on the impact of bracing treatment. We studied the evolution of the pulmonary capacity of a cohort of 90 scoliotic patients.</p> <p>Methods</p> <p>This retrospective study included 90 scoliotic patients treated since 1999 with a brace with mobile pads called CMCR (n = 90; mean age: 13 years; 10-16). These patients were diagnosed with an idiopathic scoliosis (mean angulation 20.6°). All patients underwent a radiographic and respiratory evaluation at the beginning, the middle and the end of treatment.</p> <p>Results</p> <p><it>Mean age at treatment start was </it>13. Before treatment, our patients did not have a normal pulmonary capacity: Forced Vital Capacity (FVC) was only 75% of the theoretical value. All curvature types (thoracic, thoraco-lumbar and combined scoliosis) involved this reduced pulmonary capacity, with moderate-angulated scoliosis having a negative impact. At the beginning of brace treatment, the loss of real vital capacity with brace (0.3 litres) was 10% lower than without brace.</p> <p>At CMCR removal, the FVC had increased by 0.4 litre (21% +/- 4.2% compared to the initial value). The theoretical value had increased by 3%. This positive evolution was most important in girls at a low Risser stage (0,1,2), and before 11 years of age.</p> <p>Conclusion</p> <p>These results supported our approach of orthesis conception for adolescent idiopathic scoliosis which uses braces with mobile pads to preserve thorax and spine mobility.</p

Hereditary alpha-1-antitrypsin deficiency and its clinical consequences

Alpha-1-antitrypsin deficiency (AATD) is a genetic disorder that manifests as pulmonary emphysema, liver cirrhosis and, rarely, as the skin disease panniculitis, and is characterized by low serum levels of AAT, the main protease inhibitor (PI) in human serum. The prevalence in Western Europe and in the USA is estimated at approximately 1 in 2,500 and 1 : 5,000 newborns, and is highly dependent on the Scandinavian descent within the population. The most common deficiency alleles in North Europe are PI Z and PI S, and the majority of individuals with severe AATD are PI type ZZ. The clinical manifestations may widely vary between patients, ranging from asymptomatic in some to fatal liver or lung disease in others. Type ZZ and SZ AATD are risk factors for the development of respiratory symptoms (dyspnoea, coughing), early onset emphysema, and airflow obstruction early in adult life. Environmental factors such as cigarette smoking, and dust exposure are additional risk factors and have been linked to an accelerated progression of this condition. Type ZZ AATD may also lead to the development of acute or chronic liver disease in childhood or adulthood: prolonged jaundice after birth with conjugated hyperbilirubinemia and abnormal liver enzymes are characteristic clinical signs. Cirrhotic liver failure may occur around age 50. In very rare cases, necrotizing panniculitis and secondary vasculitis may occur. AATD is caused by mutations in the SERPINA1 gene encoding AAT, and is inherited as an autosomal recessive trait. The diagnosis can be established by detection of low serum levels of AAT and isoelectric focusing. Differential diagnoses should exclude bleeding disorders or jaundice, viral infection, hemochromatosis, Wilson's disease and autoimmune hepatitis. For treatment of lung disease, intravenous alpha-1-antitrypsin augmentation therapy, annual flu vaccination and a pneumococcal vaccine every 5 years are recommended. Relief of breathlessness may be obtained with long-acting bronchodilators and inhaled corticosteroids. The end-stage liver and lung disease can be treated by organ transplantation. In AATD patients with cirrhosis, prognosis is generally grave

Psycho-educational interventions for adults with severe or difficult asthma: a systematic review.

types: Journal Article; Research Support, Non-U.S. Gov't; ReviewThis is the author's version of the work that was accepted for publication in the Journal of Asthma. The final version can be accessed via the DOI in this record.Research highlights psychosocial factors associated with adverse asthma events. This systematic review therefore examined whether psycho-educational interventions improve health and self-management outcomes in adults with severe or difficult asthma. Seventeen controlled studies were included. Characteristics and content of interventions varied even within broad types. Study quality was generally poor and several studies were small. Any positive effects observed from qualitative and quantitative syntheses were mainly short term and, in planned subgroup analyses (involving < 5 trials), effects on hospitalizations, quality of life, and psychological morbidity in patients with severe asthma did not extend to those in whom multiple factors complicate management.UK NHS Health Technology Assessment Programm