[Update of the AWMF S2k Guideline "Diagnostics and Assessment of Occupational Asbestos-Related Diseases" - What's New?] / Update der AWMF-S2k-Leitlinie "Diagnostik und Begutachtung asbestbedingter Erkrankungen" ­ Was gibt es Neues?

The S2k guideline "Diagnostics and assessment of occupational asbestos-related diseases" was updated in November 2020. This article summarizes the most important changes. There is a new reference to the risk of potentially high exposures to asbestos fibers when renovating plaster, fillers and adhesives containing asbestos.Biomarkers such as mesothelin and calretinin should currently only be used in the context of research. The "asbestos airways disease", which can only be diagnosed histologically, is included in the guideline as an early form of asbestosis. Since the UIP pattern is not characteristic of asbestosis, computed tomography cases with UIP patterns alone cannot be assigned reliably to asbestosis without the simultaneous detection of pleural plaques. With regard to the evaluation of the functional damage, attention is drawn to the importance of flow volume curve, whole-body plethysmography, diffusion capacity and exercise testing. If available, the reference values ​​according to GLI are the basis of the assessment. The guideline contains specific recommendations on prevention, medical treatment and, for the first time, on the importance of outpatient rehabilitation and training therapy. There are also references to the assessment of the new occupational disease ovarian cancer after occupational exposure to asbestos.

Association of time under immunosuppression and different immunosuppressive medication on periodontal parameters and selected bacteria of patients after solid organ transplantation.

BACKGROUND: Aim of this study was to investigate the association of the time under immunosuppression and different immunosuppressive medication on periodontal parameters and selected periodontal pathogenic bacteria of immunosuppressed patients after solid organ transplantation (SOT). MATERIAL AND METHODS: 169 Patients after SOT (lung, liver or kidney) were included and divided into subgroups according their time under (0-1, 1-3, 3-6, 6-10 and >10 years) and form of immunosuppression (Tacrolimus, Cyclosporine, Mycophenolate, Glucocorticoids, Sirolimus and monotherapy vs. combination). Periodontal probing depth (PPD) and clinical attachment loss (CAL) were assessed. Periodontal disease severity was classified as healthy/mild, moderate or severe periodontitis. Subgingival biofilm samples were investigated for eleven selected potentially periodontal pathogenic bacteria using polymerasechainreaction. RESULTS: The mean PPD and CAL as well as prevalence of Treponema denticola and Capnocytophaga species was shown to be different but heterogeneous depending on time under immunosuppression (p<0.05). Furthermore, only the medication with Cyclosporine was found to show worse periodontal condition compared to patients without Cyclosporine (p<0.05). Prevalence of Porphyromonas gingivalis, Tannerella forsythia and Fusobacterium nucleatum was reduced and prevalence of Parvimonas micra and Capnocytophaga species was increased in patients under immunosuppression with Glucocorticoids, Mycophenolate as well as combination therapy. CONCLUSION: Time under and form of immunosuppression might have an impact on the clinical periodontal and microbiological parameters of patients after SOT. Patients under Cyclosporine medication should receive increased attention. Differences in subgingival biofilm, but not in clinical parameters were found for Glucocorticoids, Mycophenolate and combination therapy, making the clinical relevance of this finding unclear.

[Guideline (S2k, AWMF) of the Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin and the Deutsche Gesellschaft für Arbeitsmedizin und Umweltmedizin "Diagnostics and Expert Opinion in the Occupational Disease No. 4101 Silicosis (Including Coal Worker's Pneumoconiosis)"]. / S2k-Leitlinie nach AWMF-Schema der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin und der Deutschen Gesellschaft für Arbeitsmedizin und Umweltmedizin "Diagnostik und Begutachtung der Berufskrankheit Nr. 4101 Quarzstaublungenerkrankung (Silikose) der Berufskrankheitenverordnung".

During the last 1.5 years an update of the guideline on silicosis was made by an interdisciplinary working group. New medical and scientific knowledge and the experience in expert opinion practice were taken into account.By preparing the initial guideline in 2010 standardization of diagnostics and adaption of the "Moers convention" which was not based on medical knowledge was in the focus, whereas the current update deals with fine emendation and extension, especially of the compensation rate (adaption with the Reichenhall recommendation).The diagnosis of silicosis (including mixed dust pneumoconiosis) is based on a detailed occupational history, and predominantly on the typical radiological findings. However, at initial diagnosis the standardized LD-HRCT takes an important role because of its high sensitivity and specificity. Exceptional cases are those with characteristic findings in chest X-ray follow-up. Correspondingly, it is mentioned in the guideline: "The standardized appraisal of the Low-Dose-Volume HRCT requires application of the CT classification (ICOERD, International Classification of Occupational and Environmental Respiratory diseases). In order to diagnose silicosis in CT scan opacities with sharp borders in both central upper lung fields and their circumferencies have to be documented. By comparing with ILO standard radiographs at least profusion category 1 in the right and left upper lung fields has to be reached (total profusion category 2)."The pathologic minimal requirement for the diagnosis of silicosis which has undergone controversial discussion has now also been defined. Corresponding to Hnizdo et al. 2000 it is now mentioned: "Finding of less than 5 silicotic granuloma per lung lobe by palpation is regarded as insignificant." This is a convention and not a threshold based on detailed medical scientific and statistical studies; it is based on extended experience in the South African gold mines.This guideline also deals with silicotic hilar (and sometimes mediastinial) lymph nodes; according to the guideline working group they do not closely correlate with the degree of pulmonary involvement. Extended conglomerating and enduring lymph-node processes may lead to dislocation of the hili with impairment of large bronchi and vessels. Shell-like calcifications dominating in the periphery of lymph nodes produce so-called egg-shell hili.The paragraph on exercise testing is now extended: if neither ergometry nor spiroergometry can be performed a 6 minute walking test by measuring oxygen saturation should be done.Furthermore, in individual expert opinion examinations right heart catheterization (the patient is not obliged to give informed consent) may be recommended, if echo cardiography gives evidence for pulmonary hypertension or if it is difficult to differentiate between right and left heart failure. The presence of pulmonary hypertension which is of prognostic relevance has to be considered when grading reduction in earning capacity.For interpretation of spirometry values the new GLI reference values has to be applied. Grading of impairment is due to the recommendation of the DGP.According to current medical scientific knowledge it is unclear, whether certain disorders of the rheumatic group such is scleroderma or Caplan syndrome which are sometimes associated with silicosis (or coal workers' pneumoconiosis) belong in toto to the occupational disease number 4101 (silicosis). Within this context, additional studies are needed to clarify the role of occupational quartz exposure and other risk factors.The guideline working group hopes that this update will help to optimize diagnostics and expert opinion of silicotic patients.

[Cardiac surgery in underlying chronic pulmonary disease. Prognostic implications and efficient preoperative evaluation]. / Herzchirurgie bei pulmonalen Grunderkrankungen. Prognostische Bedeutung und rationelle präoperative Diagnostik.

Cardiac surgery in patients with chronic pulmonary diseases carries a high risk of postoperative pulmonary complications (ppc) because both are known to cause ppc. Autopsy studies have revealed ppc as the main cause of mortality in approximately 5-8% of patients after cardiac surgery. Not all pulmonary diseases are high risk comorbidities in cardiac surgery: whereas chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea significantly increase the risk of ppc, a well controlled asthma does not carry an additional risk of ppc. A thorough preoperative risk stratification is crucial for risk estimation and some validated risk calculators, such as the Canet risk score exist. Surprisingly the additional value of pulmonary function testing beyond a thorough patient history and physical examination is low. No validated thresholds exist in pulmonary function testing below which cardiac surgery should be denied if clearly indicated. Perioperative strategies for risk reduction should be applied to all patients whenever possible.

[Lung transplantation]. / Lungentransplantation.

Lung transplantation is a therapeutic option for patients with end-stage lung diseases. Selection of candidates requires careful consideration of the disease-specific indications and contraindications for transplantation. Advances have been made in candidate selection via the ability to prognosticate outcomes of various lung diseases and through the implementation of the lung allocation score (LAS) with specific consideration of the degree of urgency and good postoperative survival rate, after neglecting the waiting time. This system has resulted in decreased mortality on the waiting list for lung transplantation. The availability of donor organs can possibly be increased by implementation of ex vivo lung perfusion as an alternative to conventional organ preservation. Risk factors for poor outcomes post-lung transplantation have been identified and understanding of the physiological, cellular and molecular mechanisms responsible for lung and airway damage has been extensively expanded. Primary graft dysfunction, infectious diseases, acute rejection, antibody-mediated rejection, lymphocytic bronchiolitis, obliterative bronchiolitis, restrictive allograft syndrome, and chronic lung allograft dysfunction are well defined complications and continue to be common causes of morbidity and mortality. This article provides a comprehensive update on these topics for the non-transplantation clinician.

[Medical therapy of heart and lung diseases. Effects on the respective other organ]. / Medikamentöse Therapie von Herz- und Lungenerkrankungen. Effekte auf das jeweils andere Organ.

Many patients suffer from both heart and lung diseases. The choice of medical drugs should not only be driven by the clinical and prognostic effects on the target organ but should also be selected based on the effects on the respective other organ. Beta blockers and statins have both beneficial and harmful effects on the respiratory system. Angiotensin-converting enzyme (ACE) inhibitors and amiodarone can cause severe lung damage. Low-dose thiazides and calcium antagonists are first-line medications in hypertensive asthma patients but beta blockers should be avoided. Theophyline should be used with caution in patients with known cardiac disease. Glucocorticosteroids can cause cardiovascular symptoms while the phosphodiesterase inhibitor roflumilast appears to have no relevant cardiovascular side effects. Anticholinergic drugs have both favorable and unfavorable cardiovascular (side) effects. Short-acting beta-2 sympathomimetic drugs (SABA) and macrolides in particular can trigger arrhythmia and some SABAs are associated with a higher incidence of myocardial infarction. Detailed knowledge of the effects of drugs used for the treatment of lung and heart diseases on the respective other organ and the associated complications and long-term effects are essential in providing optimal medical care to the many patients who present with both respiratory and cardiovascular diseases.

[Expert meeting chronic obstructive airway disease--cardiovascular aspects of COPD]. / Expertentreffen obstruktive Atemwegserkrankungen: Kardiovaskuläre Aspekte der COPD.

This overview presents data that take advantage of a new step of insight into COPD. Large population-based retrospective studies and intensively investigated prospective cohorts are two important sources of knowledge that have been recently developed. One of the contributions introduces the German COSYCONET which is on its way shortly after the American ECLIPSE cohort. The vast amount of new data has also contributed to some corrections of the recommendations of the international GOLD committee. Clinically important are the waiver of the reversibility test for the diagnosis of COPD, the inclusion of sympotom scores to evaluate quality of life and the estimation of exacerbations. The COPD types I through IV were originally the result of expert opinion, but their impact on prognosis has recently been evaluated empirically.The top issues of the expert meeting were cardiovascular aspects of COPD. Besides the comorbidity of two significant chronic diseases, it became clear that cardiovascular events have an outstanding significance for COPD patients. Inversely, advanced COPD is an important risk factor in cardiac and vascular diseases. The mutual influence of both disease entities does not only affect the long term progression but also the outcome of acute events like myocardial infarction and COPD exacerbation. The following contributions investigate the topic with regard to epidemiology, the biology of vessels, and especially with regard to acute COPD exacerbations and pharmakotherapy. Recent evidence enables a fresh view on the cardiovascular toxicity of COPD medication and on possible protective effects of cardiovascular drugs (i.e. statins and ß-receptor antagonists) for patients with COPD.

Prevalence and prediction of exercise-induced oxygen desaturation in patients with chronic obstructive pulmonary disease.

BACKGROUND: Previous studies with small sample sizes reported contradicting findings as to whether pulmonary function tests can predict exercise-induced oxygen desaturation (EID). OBJECTIVE: To evaluate whether forced expiratory volume in one second (FEV(1)), resting oxygen saturation (SpO(2)) and diffusion capacity for carbon monoxide (DLCO) are predictors of EID in chronic obstructive pulmonary disease (COPD). METHODS: We measured FEV(1), DLCO, SpO(2) at rest and during a 6-min walking test as well as physical activity by an accelerometer. A drop in SpO(2) of >4 to <90% was defined as EID. To evaluate associations between measures of lung function and EID univariate and multivariate analyses were used and positive/negative predictive values were calculated. Receiver operating characteristic curve analysis was performed to determine the most useful threshold in order to predict/exclude EID. RESULTS: We included 154 patients with COPD (87 females). The mean FEV(1) was 43.0% (19.2) predicted and the prevalence of EID was 61.7%. The only independent predictor of EID was FEV(1) and the optimal cutoff value of FEV(1) was at 50% predicted (area under ROC curve, 0.85; p < 0.001). The positive predictive value of a threshold of FEV(1) <50% was 0.83 with a likelihood ratio of 3.03 and the negative predicting value of a threshold of FEV(1) ≥80% was 1.0. The severity of EID was correlated with daily physical activity (r = -0.31, p = 0.008). CONCLUSIONS: EID is highly prevalent among patients with COPD and can be predicted by FEV(1). EID seems to be associated with impaired daily physical activity which supports its clinical importance.

[Rare diseases in pulmonary medicine and its challenges]. / Seltene Erkrankungen in der Pneumologie und damit verbundene Herausforderungen.

The importance of rare disease is appreciated by all parties and tremendous effort is made to increase the knowledge about the individual disorders and improve the care of affected patients. Political initiatives on a European level aim to improve the structure of medical care for patients with rare diseases in each member state. The provided incentives for the development of medicines for orphan diseases have led to increased research activities and numbers of licensed Orphan Drugs. Patients are organized nationally and internationally in various patient organizations and umbrella organizations. They are involved in health care policy, support the detection and research of rare diseases and offer support to affected patients and families with educational meetings and materials as well as options for discussions. Many experts are engaged in national and international networks and registries that generate and publish high quality research data on rare diseases. A well developed infrastructure is in place to support the search for qualified partners that can be of assistance with specific questions in a rare lung disease.

[Meeting of experts on obstructive airway diseases: targets and methods]. / Expertentreffen Obstruktive Atemwegserkrankungen: Targets und Methoden.

New insights into the pathogenesis and clinical course of chronic obstructive pulmonary disease (COPD) and asthma have become available. Systematic analyses of well-defined and intensively monitored patient cohorts are being published, particularly from the ECLIPSE cohort in the U.S.A. and from the network COSYCONet in Germany. Important articles from 2011 on COPD and asthma put former concepts into question. There is a new understanding of the relationship between parenchymal destruction and bronchial obstruction in COPD as well as on the impact of cardiovascular comorbidity. Computed tomography allows high-resolution imaging of lung structures, and MRI delivers supplementary functional information. Researchers have also investigated the value of patient-reported outcomes, such as quality of life, dyspnoea, or the COPD assessment test (CAT). Members of the GOLD committee are trying to establish a feasible classification of the multiple facets of COPD. With respect to treatment, novel data on beta-adrenergic antagonists in COPD and on muscarinic antagonists in asthma have been published. These aspects were discussed during an expert meeting and are now summarised in the present review article.

Screening for sleep-disordered breathing in neuromuscular disease using a questionnaire for symptoms associated with diaphragm paralysis.

Patients with neuromuscular disease (NMD) are at risk of developing sleep-disordered breathing (SDB) following respiratory muscle involvement. We hypothesised that a questionnaire based on clinical symptoms and signs of diaphragm weakness can be used to screen for SDB in such patients. We developed a self-administered multiple choice questionnaire containing five questions (Sleep-Disordered Breathing in Neuromuscular Disease Questionnaire (SiNQ)-5), scoring 0-10 points. 125 patients were enrolled: 32 with respiratory muscle weakness, 35 subjects with normal respiratory muscle strength and 58 patients with obstructive sleep apnoea (OSA). All subjects underwent full polysomnography. NMD patients with involvement of the respiratory muscles scored mean ± sd 6.8 ± 2.3 out of 10 points, significantly higher than both OSA patients 2.5 ± 2.3 and normal subjects 1.0 ± 2.0 (p < 0.001). A score of five or more points in the SiNQ-5 had a sensitivity of 86.2%, specificity of 88.5%, positive predictive value of 69.4% and a negative predictive value of 95.5% to identify NMD with combined SDB. A short self-administered questionnaire, the SiNQ-5, based on clinical symptoms can reliably screen for SDB in patients with diaphragm weakness. However, comorbidities, such as heart failure, that have symptoms influenced by posture could alter diagnostic accuracy.

[COPD and Clinical Trials. Results of an expert meeting "Castles in the Air" 2010]. / COPD und Studien. Ergebnisse des Expertentreffens Luftschlösser 2010, Mainz, 3.­4. Dezember 2010 (Sponsor: Boehringer Ingelheim Pharma GmbH & Co KG).

Clinical trials in COPD patients aim at achieving progress in diagnosis and treatment. Study results should be applicable to a large number of patients. However, an analysis of the methods and design of current and previous trials reveals considerable room for improvement. COPD is a complex disease with different clinical phenotypes. Genetic factors need to be evaluated systematically to allow appropriate stratification of patients. Frequently used endpoints such as the FEV1 that had previously been considered reliable have shown limitations in recent trials. Thus, researchers now aim to identify new surrogate parameters that are related to the prognosis of the disease, e. g., composite endpoints and biomarkers. Physical activity and capacity are becoming increasingly important for the evaluation of disease progression. The focus of pharmaceutical development is long acting bronchodilators and new anti-inflammatory drugs. The value of non-drug interventions will also be evaluated.

[Sleep-related breathing disorders - historical development, current status, future prospects]. / Schlafassoziierte Ventilationsstörungen - historische Entwicklung, Status quo und Ausblick.

Sleep-related breathing disorders are common adult illnesses in Western countries and classified as either dominant obstructive sleep apnoea or dominant central sleep apnoea. Cheyne-Stokes Respiration is part of the spectrum of CSA. The earliest descriptions of patients who presumably suffered from sleep apnoea were made in the 19th century. The term ''Pickwickian'' in connection with sleepy patients was introduced in 1889. The first electrophysiological sleep recordings of Pickwickian patients and the understanding of the syndrome as disordered breathing in sleep, were made during the late 1950s and 1960s at the universities of Heidelberg and Freiburg in Germany. The term sleep apnoea syndrome was introduced by Guilleminault from Stanford. The introduction of continuous positive airway pressure (CPAP) therapy by C. E. Sullivan and co-workers gave an enormous impetus to the field of sleep-disordered breathing. Its recognition as a public health problem was facilitated by the Wisconsin study, investigating the prevalence of sleep apnoea in the middle-aged general population. Nowadays obstructive sleep apnoea (OSA) is recognised as an independent risk factor for a wide range of clinical conditions, such as atherosclerosis, hypertension, heart failure, arrhythmias, stroke, diabetes, and depression. This article focuses on issues related to OSA and CSA/CSR, their pathogenesis, interaction with other comorbidities including cardiovascular diseases. Future research will focus on treatment effects on cardiovascular and metabolic outcomes in sleep apnoea and on the pathophysiological mechanisms responsible for the inflammatory state and cardiovascular morbidity in the syndrome. Other potential areas of research include biochemical markers, new diagnostic and therapeutic modalities.

Serum KL-6 as a novel disease marker in adolescent and adult cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a chronic progressive disease leading to obstructive pulmonary impairment, fibrosis and shortened life expectancy. Serum levels of KL-6, high molecular weight human MUC1 mucin, are increased in the majority of patients with various interstitial lung disorders. Whether they are also elevated in CF has not been investigated before. OBJECTIVE: To evaluate whether serum KL-6 levels are elevated and correlate with pulmonary function variables in CF. DESIGN: Serum KL-6, lactate dehydrogenase (LDH) and C-reactive protein (CRP) levels were measured in 72 consecutive CF and 80 age- and sex-matched healthy control subjects. The relationship between serum KL-6 levels and pulmonary function variables was analyzed. RESULTS: Serum KL-6 levels in CF patients were significantly increased compared to healthy subjects. Receiver operating characteristic curve analysis revealed that the diagnostic accuracy of KL-6 was better than that of LDH and CRP. Serum KL-6 levels showed an inverse relationship with vital capacity (VC) % predicted and forced expiratory volume in one second (FEV1) % predicted. CONCLUSIONS: Serum KL-6 levels are elevated and appear to be correlated with pulmonary function variables in CF. These results suggest that KL-6 may be a useful noninvasive marker to monitor disease severity.

[Assessment and outcome parameters in COPD]. / Beurteilungs- und Prognosekriterien bei COPD1.

BACKGROUND: A standard outcome parameter for pharmacological trials in COPD has not yet been defined. Therefore, it is the aim of this review to evaluate frequently used parameters for their eligibility as assessment and outcome parameters in COPD. METHODS: A review of the actual scientific literature was performed. RESULTS: It is recommended to continue to rely primarily on the FEV (1), which has been used as a primary variable in the vast majority of trials. In addition, further parameters, such as FVC and IC/TLC should be determined. If available, additional information is provided by RV/TLC, K (co), PaO (2) and PaCO (2). FEV (1) is not a surrogate parameter for dyspnoea, quality of life, and exercise tolerance, which should therefore be assessed separately. Frequency and severity of exacerbations and mortality are important outcome parameters in long-term trials. Complex indices, such as the BODE index, may be superior to single variables. CONCLUSIONS: No single additional parameter has been evaluated sufficiently in order to substitute FEV (1) as the standard parameter for the assessment and outcome in COPD.

[Health effects of particulate matter exposure: current scientific knowledge]. / Gesundheitliche Effekte der Feinstaubbelastung - aktueller wissenschaftlicher Kenntnisstand.

INTRODUCTION: Air quality is not only important for respiratory health but it also influences the homeostasis of the whole human organism. In the past years numerous violations of European Union particulate matter thresholds have been recorded. METHODS: The present study is a selective literature analysis encompassing the epidemiology and pathophysiological effects of particulate matter. RESULTS: Epidemiological studies point to an association between chronic particulate matter exposure and mortality. The most prominent effects on the human body are present in subjects with cardiovascular or respiratory conditions. However, the effects of air pollutants need to be examined critically and the plausibility of thresholds should be evaluated in detail. DISCUSSION: The negative influences of chronic particulate matter exposure have been proven by a multitude of epidemiological and experimental studies. From the viewpoint of primary prevention, air quality plays a crucial role. This encompasses both the outdoor compartment with particulate matter and other pollutants and the indoor compartment with tobacco smoke.

[Care of patients under mechanical ventilation at home and in nursing home conditions. Position paper of the German Medical Associations of Pneumology and Ventilatory Support ("Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V." and "Arbeitsgemeinschaft für Heimbeatmung und Respiratorentwöhnung e. V.")]. / Betreuung von Patienten mit maschineller Beatmung unter häuslichen und heimpflegerischen Bedingungen. Stellungnahme der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP) und der Arbeitsgemeinschaft für Heimbeatmung und Respiratorentwöhnung e. V.

The advances in intensive and critical care medicine have not only improved the prognosis of patients with acute respiratory failure but have also increased the number of ventilator-dependent patients. The continuously increasing number of patients, the differentiation of care-giving institutions and the technical progress make it necessary to re-evaluate the quality of health care in weaning centres and outpatient care of patients on long-term ventilation. Therefore, the German medical associations of pneumology and ventilatory support, "Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V." and the "Arbeitsgemeinschaft für Heimbeatmung und Respiratorentwöhnung e. V.", wish to present this actual position paper. However, scientific guidelines are in preparation.

Predictors of severe chest infections in pediatric neuromuscular disorders.

Chest infections are serious complications in neuromuscular disorders. The predictive values of lung and respiratory muscle function including peak cough flow still remain unclear. We performed retrospective analysis of 46 children and adolescents (12.7+/-3.7 years) in whom lung function, respiratory muscle function and peak cough flows had been obtained. Data were related to: (1). number of chest infections and days of antibiotic treatment the year prior to the study and (2). history of severe chest infection requiring hospital admission. The number of chest infections and the number of days treated with antibiotics correlated with Inspiratory Vital Capacity IVC, peak cough flow PCF and Peak Expiratory Pressure PEP. Twenty-two patients were hospitalized at least once due to severe chest infection. IVC (0.65 vs. 1.44 l; P<0.0001) and PCF (116 vs. 211 l/min; P<0.0005) in these patients were significantly lower than in the non-hospitalized group. IVC<1.1l and PCF<160 l/min were specific and sensitive thresholds to discriminate between patients who had already suffered severe chest infections and those who had not. Therefore, spirometry and peak cough flow are reliable tests to identify patients at high risk for severe chest infections. Patients with IVC below 1.1l and/or PCF below 160 l/min should be well monitored and introduced to assisted coughing techniques.