Search

Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, Pseudomonas aeruginosa (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung function. There are many possibilities to treat PA-infection.This is a S3-clinical guideline which implements a definition for chronic PA-infection and demonstrates evidence-based diagnostic methods and medical treatment in order to give guidance for individual treatment options., Competing Interests: Eine Übersicht der Interessenkonflikt findet sich im Internet unter http://awmf.org; AWMF-Registernummer 026-022, (Thieme. All rights reserved.)

Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

Chronic inflammation induces proinflammatory cytokine cascades. In addition to systemic inflammation, hypoxemia, hypercapnia, a catabolic metabolism, gonadal or thyroid dysfunction, musculoskeletal dysfunction and inactivity as well as vitamin D deficiency contribute to an increased risk of fragility fractures. Iatrogenic causes of osteoporosis are long-term use of inhaled or systemic glucocorticoids (GC). Inhalative GC application in asthma is often indicated in childhood and adolescence, but interstitial lung diseases such as chronic organizing pneumonia, COPD, sarcoid or rheumatic diseases with lung involvement are also treated with inhalative or oral GC. In patients with cystic fibrosis, malabsorption in the context of pancreatic insufficiency, hypogonadism and chronic inflammation with increased bone resorption lead to a decrease in bone structure. After lung transplantation, immunosuppression with GC is a risk factor.The underlying pneumological diseases lead to a change in the trabecular and cortical bone microarchitecture and to a reduction in osteological formation and resorption markers. Hypercapnia, acidosis and vitamin D deficiency can accelerate this process and thus increase the individual risk of osteoporotic fragility fractures.A bone mineral density measurement with a T‑Score < -2.5 is a threshold value for the diagnosis of osteoporosis; in contrast the vast majority of all osteoporotic fractures occur with a T‑Score > -2.5. A history of low-trauma fracture indicates osteological therapy.All antiresorptive or anabolic drugs approved in Austria for the treatment of osteoporosis are also indicated for pneumological patients with an increased fragility fracture risk of bone fractures in accordance with the national reimbursement criteria.

Patients with asthma should be vaccinated against COVID-19. This includes patients with severe asthma. Treatment with a biological for asthma is no contra-indication for vaccination against COVID-19., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Scientific Members of the Austrian Society of Pneumology describe the expected development in respiratory health and provide guidance towards patient-oriented and cost-efficient respiratory care in Austria.Methods: In November 2017, respiratory care providers (physicians, nurses, physiotherapists) together with patient's advocacy groups and experts in health development, collaborated in workshops on: respiratory health and the environment, bronchial asthma and allergy, COPD, pediatric respiratory disease, respiratory infections, sleep disorders, interventional pneumology, thoracic oncology and orphan diseases.Results: Respiratory disease is extremely prevalent and driven by ill-health behavior, i.e. cigarette smoking, over-eating and physical inactivity. For the majority of respiratory diseases increased prevalence, but decreased hospitalizations are expected.The following measures should be implemented to deal with future challenges:1. Screening and case-finding should be implemented for lung cancer and COPD.2. E-health solutions (telemedicine, personal apps) should be used to facilitate patient management.3. Regional differences in respiratory care should be reduced through E‑health and harmonization of health insurance benefits across Austria.4. Patient education and awareness, to reduce respiratory health illiteracy should be increased, which is essential for sleep disorders but relevant also for other respiratory diseases.5. Respiratory care should be inter-professional, provided via disease-specific boards beyond lung cancer (for ILDs, sleep, allergy)6. Programs for outpatient's pulmonary rehabilitation can have a major impact on respiratory health.7. Increased understanding of molecular pathways will drive personalized medicine, targeted therapy (for asthma, lung cancer) and subsequently health care costs.

Background: There is an increasing need by general practitioners (GPs) for consultations regarding skin diseases or skin lesions. Teledermatological and teledermoscopic diagnoses are possible with good results., Objectives: This descriptive study analyzed whether the increasing need for dermatological consultations could be improved by regional teledermatological and teledermoscopic triage between GPs and dermatologists., Materials and Methods: Via an internet platform, six GPs sent images of unclear skin diseases or skin lesions to a dermatology practice (2 dermatologists) over a period of 9 months. Possible triage answers were (1) stay at the GP, (2) urgent consultation at the dermatologist (days), or (3) later (week/months) consultation at the dermatologist, respectively, with the diagnosis and therapeutic suggestions. A feedback (grade 1-6) were given by the dermatologists about the image quality and from the GPs about the appraisal. The dermatologists' appraisals were done independently and were audited by a third dermatologist., Results: The patients' acceptance was 100% and a very good to good image quality was achieved in 94%; 66.3% of patients could stay at their GP and 20.7% of patients should come urgently to the dermatologist. The teletriage decisions of an urgent consultation at the dermatologist were confirmed in 41.5%. The GPs' feedback about the teletriage appraisal received an overall average grade of 1.1., Conclusion: Successful regional teledermatological and teledermoscopic triage between GPs and dermatologists is possible. Specifications on data privacy laws must be followed and an appropriate remuneration is preferable.

The COVID-19 pandemic is currently a challenge worldwide. In Austria, a crisis within the health care system has so far been avoided. The treatment of patients with community-acquired pneumonia (CAP), including SARS-CoV‑2 infections, should continue to be based on evidence-based CAP guidelines during the pandemic. However, COVID-19-specific adjustments are useful. The treatment of patients with chronic lung diseases must be adapted during the pandemic, but must still be guaranteed., (© The Author(s) 2020.)

Acute viral bronchiolitis and wheezy bronchitis are very common disorders in infants and preschool children. They are caused by viruses, particularly by respiratory syncytial virus and rhinoviruses. Risk factors for severe disease include premature birth, tobacco smoke exposure and immune dysfunctions. Patient history and a physical examination are sufficient to establish a diagnosis; chest X-ray and laboratory investigations are not routinely necessary. For acute bronchiolitis, many treatment options have been suggested but only supportive measures, such as minimal handling, securing adequate oxygenation and hydration are generally recommended. Antibiotics, bronchodilators, corticosteroids und leukotriene receptor antagonists are not routinely recommended. Short-acting beta-2 agonists are the first choice for the treatment of wheezy bronchitis. Inhaled corticosteroids may be tried to control symptoms in children with frequently recurring and/or severe episodes of wheezy bronchitis. The development of bronchial asthma, however, cannot be prevented by any pharmacologic tretament., (© The Author(s) 2020.)

The current consensus report was compiled under the patronage of the Austrian Society of Pneumology (Österreichischen Gesellschaft für Pneumologie, ÖGP) with the intention of providing practical guidelines for out-of-hospital ventilation that are in accordance with specific Austrian framework parameters and legal foundations. The guidelines are oriented toward a 2004 consensus ÖGP recommendation concerning the setup of long-term ventilated patients and the 2010 German Respiratory Society S2 guidelines on noninvasive and invasive ventilation of chronic respiratory insufficiency, adapted to national experiences and updated according to recent literature. In 11 chapters, the initiation, adjustment, and monitoring of out-of-hospital ventilation is described, as is the technical equipment and airway access. Additionally, the different indications-such as chronic obstructive pulmonary diseases, thoracic restrictive and neuromuscular diseases, obesity hypoventilation syndrome, and pediatric diseases-are discussed. Furthermore, the respiratory physiotherapy of adults and children on invasive and noninvasive long-term ventilation is addressed in detail.

Infectious diseases of the upper airway can lead to emergency situations with partial up to complete obstruction and respiratory insufficiency, especially in infants and toddlers. This necessitates a cool-headed and calm approach; however, at times a prompt intervention is required by the primary care physician. Important questions concerning patient history include the onset of symptoms, severity and duration of fever and for orientation possible previously known respiratory problems since birth or in the first weeks afterwards (e.g. congenital malformations of the larynx or trachea). The examination should begin by careful observation of the child's position of comfort and by noting all vital signs. A child with inspiratory or biphasic stridor at rest already has some degree of airway obstruction that can progress to complete obstruction over time. Systemic steroids and inhalation of nebulized epinephrine (adrenaline) are the best therapeutic options for viral laryngotracheobronchitis, which is the most common cause of acute stridor in childhood. Rare differential diagnoses, such as bacterial tracheitis, epiglottitis (supraglottitis), retropharyngeal and parapharyngeal infections necessitate disease-specific management., (© Springer-Verlag Wien 2015.)

Background: Currently no consensus guidelines on the evaluation and therapy of chronic cough in children have been published in German. Aim of this study was therefore, after a literature search on all relevant publications and guidelines concerning chronic cough in children, to publish a Statement of the Austrian Society of Pediatrics and The Austrian Society of Pneumology on the evaluation and therapy of chronic childhood cough and to publish a practice based approach. Aim was also to differentiate cough clearly from obstructive airway diseases and to summarize all relevant differential diagnosis using relevant patient history and clinical characteristics and give an overview of therapeutical approaches. The subjective component of cough and the difficulty to assess cough in an objective manner is discussed., Methods: A Cochrane and Medline Search were performed on all relevant literature until Mai 2013., Results: Chronic cough in children is defined as daily cough for more than 4 weeks. Duration of 3 to 8 weeks is sometimes called prolonged or sub-acute cough. Chronic cough may have specific cause and may be a symptom of an underling disease, which needs to be found and treated adequately. Unspecific cough may only be diagnosed after exclusion of all other causes. Any therapeutical approach needs to be re- evaluated and efficacy needs to be reviewed. Parental understanding of the etiology of cough in general and the influence of environmental exposures is essential for diagnosis., Conclusion: Chronic cough needs a detailed history and various differential diagnoses need to be taken in consideration. Children with persistent complaints without a proper diagnosis need to be seen in a center specialized in Pediatric Respiratory Medicine.

Over more than 50 years, the nocuous effects of smoking in pregnancy on the fetus are well known. In the first years of science the focus was primarily on restricted fetal growth while in more recent years over 10.000 studies investigated the incomparably big sum of detrimental effects for the unborn's health. In this statement we want to present the recent scientific findings on this topic. The statement is aimed to show all doctors who treat pregnant women the present situation and evidence. In the beginning we give a short overview about the epidemiological situation in Europe. Then we present step by step the health effects with regards to pathophysiology and clinics. Furthermore the reader will learn about possibilities for smoking cessation in pregnancy. The problem of passive-smoking in pregnancy will be dealt with in a separate chapter. At present there is strong evidence that pregnant smoking has a detrimental effect on birth-weight, placenta-associated disease, stillbirth, sudden infant death syndrome (SIDS), childhood overweight, clefts, lung function, asthma, cardiovascular diseases and mental developmental disorders. These factors can be summarized by the term Fetal Tobacco Syndrome. There is supply for more studies for less investigated health effects. Pregnancy is a chance to stop smoking as most women show a high motivation in this period. Hence doctors of all disciplines should inform pregnant women about the detrimental effects of smoking on their unborn child and show them possibilities for smoking cessation.

Recent publications suggest that long-acting beta-2 agonists (LABAs) increase the risk for death in asthma. The American Food and Drug Administration (FDA) published a relevant alert in 2005. In the currently valid Austrian consensus guidelines for drug therapy of bronchial asthma in children and adolescents, LABAs are only recommended as add-on therapy in those patients whose asthma is not sufficiently controlled by inhaled corticosteroids (ICS) alone. LABAs have no established role in earlier steps of the therapeutic algorithm; consequently, the prescription of ICS-LABA combinations for initial treatment of paediatric asthma is not supported by these consensus treatment guidelines.

Bronchopulmonary dysplasia (BPD) is a chronic lung disease that develops in preterm infants treated with oxygen and positive-pressure ventilation for respiratory distress syndrome. Despite the introduction of new treatment modalities (surfactant therapy, high-frequency oscillation) and improvements in the outcome of critically ill preterm infants, BPD has become an extremely important complication of neonatal intensive care and the most common form of chronic lung disease in infants. Specific pathogenesis, treatment modalities, prognosis, and multidisciplinary approaches to the prevention of BPD are described in detail. Extrinsic allergic alveolitis ("hypersensitivity pneumonitis") is a rare pulmonary disease in childhood due to inhaled organic dust, containing fungal antigens, thermophilic actinomycetes, or avian proteins. Diagnosis is often difficult, but it should be considered in every child with persistent and otherwise unexplained respiratory symptoms.

The spectrum of chronic interstitial lung disease in children includes a large and heterogeneous group of rare disorders. This paper reviews these disorders by focussing on basic pathophysiological mechanisms, and by discussing the difficulties in the classification of these diseases. Diagnostic and therapeutic approaches are also listed. The overall prognosis is dubious and mortality remains high.

With special emphasis on the paediatric aspects, the importance of peak expiratory flow rate measurements in asthma patients is reviewed. Portable peak flow meters allow for regular home monitoring of lung function. Repeated peak expiratory flow recordings by patients at home are of great value in the diagnosis and the therapeutic management of bronchial asthma. Monitoring of peak expiratory flow rates may improve the perception of compromised airway function and thereby enhance compliance in asthma patients. As a relatively simple form of pulmonary function testing, the measurement of peak expiratory flow rates, however, cannot substitute for pulmonary function testing by spirometry, flow-volume-curves or bodyplethysmography.

Over a 4-year period we performed 420 endoscopic investigations in 262 patients (aged 1 day-26 yrs) under sedation and local anesthesia, using several different flexible instruments; repeat procedures were performed in 58 children. The most common indications for diagnostic endoscopy were tracheostomy evaluation (27.1%), non-specific chest x-ray changes (13.8%), stridor (12.4%), atelectasis (7.9%), and the sampling of airway secretions or cells by bronchoalveolar lavage (5.5%); indications for 17 therapeutic endoscopies (4%) included the removal of airway secretions and mucus plugs, and aid in difficult intubations. Bronchomalacia (46X), tracheomalacia (41X), obstructing granulation tissue (36X), and laryngomalacia (35X) were the most common diagnoses; in addition, subglottic stenosis was found in 13, a subglottic hemangioma in 5, vocal cord paralysis in 5 and an endobronchial foreign body in 5 children. Findings were normal in 39 (9.3%) cases. A relevant diagnosis was established on 73.7% of all investigations. The diagnostic yield was highest in patients with stridor, persistent wheezing, atelectasis, non-specific chest x-ray changes, and in patients with a tracheostomy. The success rate of therapeutic procedures was 70.6%. Minor complications occurred in altogether 4.3% of cases; all were completely and rapidly reversible. Flexible fiberoptic bronchoscopy is a safe and valuable diagnostic and therapeutic tool for the management of infants and children with respiratory problems.

The open-circuit nitrogen washout technique, as developed by Gerhardt et al., seems to be ideally suited for assessing functional residual capacity (FRC) in infants. By performing this measurement in over 250 infants throughout the last three years, we gathered considerable practical experience of our own, but also encountered several methodological problems, which, so far, have received only little attention by the relevant literature, or have remained unresolved altogether. Our data illustrate the importance of reproducing the infant's own breathing rate and tidal volume when calibrating the system. The choice of the O2-background-flow should be based on the individual peak tidal inspiratory flow, as derived from the tidal flow-volume loop. The importance of maintaining this O2-background-flow unchanged between calibration and measurement is also demonstrated. The question, at which N2-concentration the measurement should be terminated, has remained unresolved. Diffusion of N2 from blood and tissue into the alveolar space is responsible for considerable noise. Our own measurements resulted in widely differing FRC-values for different N2-target-concentrations; these differences seem to increase with more severe bronchial obstruction. Finally, there remains the question, how long the minimal interval between two subsequent measurements should be. In conclusion, these unresolved questions have to be answered in relevant prospective studies, before recommending this technique for routine clinical application.

Up to now, 16.338 IRT-measurements have been carried out on dried blood spot specimens; 15.505 of them were taken in the first week of life. Related to a provisionally chosen cut-off point of 750 ng/ml, 134 newborns (= 0.86%) showed an elevated IRT-value and subsequently were recalled between the fourth and sixth week of life for a second IRT-determination. Twenty-five out of 116 reinvestigated children again showed an elevated value, as based on likewise provisional, age-dependent reference values. Four of these children subsequently were identified as CF patients by sweat testing. So far, we did not encounter any false-negative IRT values. We also commenced to establish a profile of reference values for the first twelve weeks of life; as yet, there are not enough data for definitely defining these limits of normality. In conclusion, IRT-screening appears to be a reliable method for identifying CF patients in the newborn period. Our preliminary results indicate an incidence of CF of 1 to 3880 in the southeast of Austria.

Up to now 49,116 immunoreactive trypsin (IRT) measurements have been carried out in Austrian newborns in the first week of life. Related to provisionally chosen cut-off points, 301 newborns (0.61%) showed an elevated IRT value; 253 of them were successfully recalled. According to a direct strategy, sweat tests were done without a second IRT measurement in 101 infants; eleven of them were identified as cystic fibrosis (CF) patients. In accordance with a 2-step strategy, 152 infants were reinvestigated by a second IRT determination. Twenty-eight of them again showed an elevated IRT value, as based on provisional, age-dependent reference values; seven were subsequently identified as CF patients by sweat testing. So far two false-negative findings were obtained on IRT screening: one child was later identified as having CF on the basis of typical clinical symptoms and a positive sweat test, the other patient presenting with meconium ileus showed a normal IRT value after surgery, but was subjected to a sweat test in view of the underlying condition. These preliminary results suggest a CF incidence of 1 to 2460 newborns in Austria. Hence, IRT screening appears to be a reliable method for identifying CF patients in the newborn period, thereby facilitating early treatment and genetic counselling.

Thirteen centres studied the efficacy and safety of nebulized cromolyn solution in 53 asthmatic children (36 m., 17 f.; mean age 4.8 +/- 1.7 years). During a fiveweek treatment period a significant reduction of asthmatic symptoms was observed by investigators daily symptom scores, as assessed by the parents, also showed a significant improvement during treatment period. In addition, there was also a marked decrease in the use of other anti-asthmatic medication (beta 2-sympathomimetics, theophylline and antihistamines). There were no side effects of nebulized cromolyn solution treatment.

To evaluate the reliability and accuracy of a rapidly performed enzyme immunochromatography method for measuring the serum concentration of theophylline, we compared it with a conventional technique (fluorescence-polarisation immunoassay). We investigated 103 venous blood samples from children and adolescents with bronchial asthma, medicated with sustained-release theophylline preparations. There was a highly significant correlation of the measured values; with a few exceptions, the individual values scattered only narrowly around the regression line. Based on this close correlation, the investigated enzyme immunochromatography test can be recommended for rapid theophylline serum level determinations in clinical practice.