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Objective: Granulomatosis with polyangiitis (GPA) is a potentially fatal condition which often manifests in the head and neck. Currently, diagnosis relies on antineutrophil cytoplasmic autoantibody (c-ANCA) serology and mucosal or renal biopsy. However, a significant proportion of patients with GPA limited to the head and neck are seronegative and biopsy negative. This study evaluates the role of clinical diagnosis of GPA in the absence of positive laboratory findings. Study Design: Case series with chart review. Setting: Academic Tertiary Medical Center. Methods: This was a retrospective review of 143 patients treated in an outpatient otolaryngology clinic at a tertiary care hospital for known or suspected GPA from 1998 to 2021. Presenting symptoms, C-ANCA status at initial presentation, biopsy results, long-term serology results, and time to initiation of treatment were analyzed. Results: Twenty-six of 143 (18.2%) patients were seronegative; only 3 of these patients (12%) had positive biopsies. Seventeen (73.9%) of these patients presented with nasal and sinus disease and 12 (52.2%) presented with airway involvement. Only 4 (17.4%) patients had renal involvement. Delay in treatment of patients with negative laboratory workup ranged from 0 months to 11 years. All patients who were seronegative and/or biopsy negative at presentation responded clinically to immunosuppressive therapy. Conclusion: GPA cases are often limited to the upper respiratory tract, making diagnosis difficult, particularly in seronegative patients. These results suggest that, when GPA is suspected, despite negative serology, the diagnosis of GPA should be made on clinical grounds, and empiric therapy encouraged to prevent delay in treatment.

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BACKGROUND: In case of distorted airway anatomy, awake intubation with a flexible bronchoscope can be extremely difficult or even impossible. To facilitate this demanding procedure, an infrared flashing light source can be placed on the patient's neck superficial to the cricothyroid membrane. The light travels through the skin and tissue to the trachea, from where it can be registered by the advancing bronchoscope in the pharynx and seen as flashing white light on the monitor. We hypothesised that the application of this technique would allow more proximal and easier identification of the correct pathway to the trachea in patients with severe airway pathology. METHODS: As part of awake intubation, patients underwent insertion of a flexible video bronchoscope via the mouth into the trachea. The procedure was performed twice, in random order in each patient, with and without the aid of the transcutaneous flashing infrared light. All insertions were video recorded to determine at which anatomical landmark within the airway the correct pathway was identified. The videos are accessible via this link: https://airwaymanagement.dk/infrared_comparative. The predefined landmarks were in successive order: oral cavity, oro-pharynx, tip of epiglottis, arytenoid cartilages, false cords, vocal cords and trachea, as well as the spaces between them. RESULTS: Twenty-two patients had a total of 44 awake insertions with the flexible bronchoscope. The median anatomical level, at which correct identification of the trachea was obtained on the monitor, was, past the epiglottis, with the conventional technique, and at the level of the oropharynx, when using the infrared flashing light (p = .005). The time until the flashing light or the vocal cords were seen was 21 (22) S, mean (SD), and 48 (62) S, during the insertion with and without infrared flashing light activated, respectively (p = .005). Endoscopists rated it easier (p = .001) to recognise the entrance to the trachea in the infrared-group. CONCLUSION: During awake intubation of patients with airway pathology, the application of trans-cricothyroid infrared flashing light to guide the insertion of a flexible bronchoscope significantly facilitated the recognition of the pathway into the trachea and the correct advancement of the flexible endoscope. REGISTRATION OF CLINICAL TRIAL: NCT03930550.

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Congenital or acquired disorders of the pediatric airway can affect the upper, lower, or entire airway. There are fundamental differences between the anatomy and physiology of the neonate, pediatric, and adult airways. Infants are not merely small adults in this respect and size, surface area, proportion, resistance, and compliance vary greatly between the age groups. A clear understanding of these significant differences and how they affect patients dependent on age is key to appropriate management.

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BACKGROUND: Bronchoscopy involves exploration of a three-dimensional (3D) bronchial tree environment using just two-dimensional (2D) images, visual cues and haptic feedback. Sound knowledge and understanding of tracheobronchial anatomy as well as ample training experience is mandatory for technical mastery. Although simulated modalities facilitate safe training for inexperienced operators, current commercial training models are expensive or deficient in anatomical accuracy, clinical fidelity and patient representation. The advent of Three-dimensional (3D) printing technology may resolve the current limitations with commercial simulators. The purpose of this report is to develop and test the novel multi-material three-dimensional (3D) printed airway models for bronchoscopy simulation. METHODS: Using material jetting 3D printing and polymer amalgamation, human airway models were created from anonymized human thoracic computed tomography images from three patients: one normal, a second with a tumour obstructing the right main bronchus and third with a goitre causing external tracheal compression. We validated their efficacy as airway trainers by expert bronchoscopists. Recruited study participants performed bronchoscopy on the 3D printed airway models and then completed a standardized evaluation questionnaire. RESULTS: The models are flexible, life size, anatomically accurate and patient specific. Five expert respiratory physicians participated in validation of the airway models. All the participants agreed that the models were suitable for training bronchoscopic anatomy and access. Participants suggested further refinement of colour and texture of the internal surface of the airways. Most respondents felt that the models are suitable simulators for tracheal pathology, have a learning value and recommend it to others for use in training. CONCLUSION: Using material jetting 3D printing to create patient-specific anatomical models is a promising modality of simulation training. Our results support further evaluation of the printed airway model as a bronchoscopic trainer, and suggest that pathological airways may be simulated using this technique.

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BACKGROUND: Congenital cardiovascular malformations (CCVM) may cause infrequently airway pathologies (AP) in children and are of prognostic and therapeutic relevance. While computed tomography (CT) is considered first-line imaging modality in many centres, we started using magnetic resonance imaging (MRI) more and more in the last years to detect CCVM and AP to avoid radiation in this patient group. OBJECTIVE: The aim of this retrospective study was to determine and to compare the diagnostic accuracy of CT and MRI when used to detect CCVM and/or AP. METHODS: All patients suspected to have CCVM and/or AP and examined either by CT or MRI between 2000 and 2013 in our hospital were included. Extension and type of CCVM, as well as their relationship to esophagus, trachea or bronchi were assessed and related to findings of tracheobronchoscopy, cardiac catheterization or surgery if available. RESULTS: One hundred six patients (median [range] 4 years [2 days to 66 years]) were examined by CT (n = 27) or MRI (n = 79). In 78 patients (74%), CCVM and/or AP were found with either of the imaging methods. CCVM were found in 63 subjects. Forty-six of 63 subjects had both, CCVM and AP. The presence of CCVM was always detected correctly by CT or MRI, although both techniques had a weakness detecting atretic segments directly. AP (n = 61) were correctly diagnosed in all patients not intubated for artificial ventilation by CT (n = 17) and in all but 2 patients by MRI (39 out of 41). CONCLUSIONS: MRI is sensitive to detect CCVM associated with AP equally to CT without any radiation exposure.

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The seasonal variability of certain non-allergic respiratory diseases is not clearly understood. Analysis of the breath condensate, the liquid that can be collected by breathing into a cold tube, has been proposed to bring closer to the understanding of airway pathologies. It has been assumed, that (1) airway lining fluid was a stable body liquid and (2) the breath condensate samples were representative of the airway lining fluid. Research was focussed on the identification of biomarkers indicative of respiratory pathologies. Despite 30 years of extended investigations breath condensate analysis has not gained any clinical implementation so far. The pH of the condensate is the characteristic that can be determined with the highest reproducibility. The present paper shows, that contrary to the initial assumptions, breath condensate is not a representative of the airway lining fluid, and the airway lining fluid is not a stable body liquid. Condensate pH shows baseline variability and it is influenced by drinking and by the ambient temperature. The changes in condensate pH are linked to changes in airway lining fluid pH. The variability of airway lining fluid pH may explain seasonal incidence of certain non-allergic respiratory diseases such as the catching of a common cold and the increased incidence of COPD exacerbations and exercise-induced bronchoconstriction in cold periods.

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Airway remodeling is generally quite broadly defined as any change in composition, distribution, thickness, mass or volume and/or number of structural components observed in the airway wall of patients relative to healthy individuals. However, two types of airway remodeling should be distinguished more clearly: (1) physiological airway remodeling, which encompasses structural changes that occur regularly during normal lung development and growth leading to a normal mature airway wall or as an acute and transient response to injury and/or inflammation, which ultimately results in restoration of a normal airway structures; and (2) pathological airway remodeling, which comprises those structural alterations that occur as a result of either disturbed lung development or as a response to chronic injury and/or inflammation leading to persistently altered airway wall structures and function. This review will address a few major aspects: (1) what are reliable quantitative approaches to assess airway remodeling? (2) Are there any indications supporting the notion that airway remodeling can occur as a primary event, i.e., before any inflammatory process was initiated? (3) What is known about airway remodeling being a secondary event to inflammation? And (4), what can we learn from the different animal models ranging from invertebrate to primate models in the study of airway remodeling? Future studies are required addressing particularly pheno-/endotype-specific aspects of airway remodeling using both endotype-specific animal models and "endotyped" human asthmatics. Hopefully, novel in vivo imaging techniques will be further advanced to allow monitoring development, growth and inflammation of the airways already at a very early stage in life.

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Pertussis toxin (PT) is a multisubunit protein toxin secreted by Bordetella pertussis, the bacterial agent of the disease pertussis or whooping cough. PT in detoxified form is a component of all licensed acellular pertussis vaccines, since it is considered to be an important virulence factor for this pathogen. PT inhibits G protein-coupled receptor signaling through Gi proteins in mammalian cells, an activity that has led to its widespread use as a cell biology tool. But how does this activity of PT contribute to pertussis, including the severe respiratory symptoms of this disease? In this minireview, the contribution of PT to the pathogenesis of pertussis disease will be considered based on evidence from both human infections and animal model studies. Although definitive proof of the role of PT in humans is lacking, substantial evidence supports the idea that PT is a major contributor to pertussis pathology, including the severe respiratory symptoms associated with this disease.

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This review is the summary of a workshop on small airways disease, which took place in Porquerolles, France in November 2011. The purpose of this workshop was to review the evidence on small airways (bronchiolar) involvement under various pathophysiological circumstances, excluding asthma and chronic obstructive pulmonary disease. Histopathological patterns associated with small airways disease were reviewed, including cellular and obliterative bronchiolitis. Many pathophysiological conditions have been associated with small airways disease including airway infections, connective tissue diseases and inflammatory bowel diseases, bone marrow and lung transplantation, common variable immunodeficiency disorders, diffuse panbronchiolitis, and diseases related to environmental exposures to pollutants, allergens and drugs. Pathogenesis, clinical presentation, a computed tomography scan and pulmonary function test findings are reviewed, and therapeutic options are described with the objective of providing an integrative approach to these disorders.

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