[Chronic rhinosinusitis in people with cystic fibrosis-an up-to-date review from the perspective of otorhinolaryngology]. / Chronische Rhinosinusitis bei Menschen mit zystischer Fibrose ­ eine aktuelle Übersicht aus der Perspektive der Hals­, Nasen­, Ohrenheilkunde.

BACKGROUND: Cystic fibrosis (CF) is a complex systemic disease involving numerous organ systems. With improved treatment options and increasing life expectancy of persons with CF (PwCF), extrapulmonary manifestations are coming increasingly into the focus. From birth, almost all PwCF have radiologically detectable pathologies in the upper airways attributable to CF-associated chronic rhinosinusitis (CF-CRS). OBJECTIVE: The aim of this work is to provide an up-to-date overview of CF-CRS from the otorhinolaryngology perspective and to provide the reader with background knowledge and current developments. PATHOPHYSIOLOGY: The cystic fibrosis transmembrane conductance regulator (CFTR) gene defect leads to increased viscosity of sinonasal secretions and reduced mucociliary clearance, causing chronic infection and inflammation in the upper airway segment and, consequently, to CF-CRS. CLINICAL PICTURE AND DIAGNOSTICS: The clinical picture of CF-CRS comprises a wide spectrum from asymptomatic to symptomatic courses. CF-CRS is diagnosed clinically and radiologically. THERAPY: Sinonasal saline irrigation is recommended as a conservative treatment measure. Topical corticosteroids are also commonly used. Surgical therapy is reserved for highly symptomatic treatment-refractory patients without a sufficient response to conservative treatment including CFTR modulator (CFTRm) therapies. Depending on the CFTR mutation, CFTRm therapies are the treatment of choice. They not only improve the pulmonary and gastrointestinal manifestations in PwCF, but also have positive effects on CF-CRS. CONCLUSION: The ENT specialist is part of the interdisciplinary team caring for PwCF. Depending on symptom burden and treatment responsiveness, CF-CRS should be treated conservatively and/or surgically. Modern CFTRm have a positive effect on the clinical course of CF-CRS.

[Microvascular changes in COVID-19]. / Mikrovaskuläre Veränderungen bei COVID-19.

BACKGROUND: Clinically, coronavirus disease 2019 (COVID-19) is associated with a wide range of symptoms, which can range from mild complaints of an upper respiratory infection to life-threatening hypoxic respiratory insufficiency and multiorgan failure. OBJECTIVE: The initially identified pulmonary damage patterns, such as diffuse alveolar damage in acute lung failure, are accompanied by new findings that draw a more complex scenario. These include microvascular involvement and a wide range of associated pathologies of multiple organ systems. A back-scaling of microstructural vascular changes is possible via targeted correlation of pathological autopsy results with radiological imaging. MATERIAL AND METHODS: Radiological and pathological correlation as well as microradiological imaging to investigate microvascular involvement in fatal COVID-19. RESULTS: The cases of two COVID-19 patients are presented. Patient 1 showed a relative hypoperfusion in lung regions that did not have typical COVID-19 infiltrates; the targeted post-mortem correlation also showed subtle signs of microvascular damage even in these lung sections. Patient 2 showed both radiologically and pathologically advanced typical COVID-19 destruction of lung structures and the case illustrates the damage patterns of the blood-air barrier. The perfusion deficit of the intestinal wall shown in computed tomography of patient 2 could not ultimately clearly be microscopically attributed to intestinal microvascular damage. CONCLUSION: In addition to microvascular thrombosis, our results indicate a functional pulmonary vasodysregulation as part of the pathophysiology during the vascular phase of COVID-19. The clinical relevance of autopsies and the integration of radiological imaging findings into histopathological injury patterns must be emphasized for a better understanding of COVID-19.

[Magnetic resonance imaging of the lungs in cystic fibrosis]. / Magnetresonanztomographie der Lunge bei Mukoviszidose.

CLINICAL ISSUE: Disease severity and mortality in patients with cystic fibrosis (CF) is mainly determined by (progressive) pulmonary lung disease. Early diagnosis and therapy are important and of prognostic value to conserve lung function. STANDARD RADIOLOGICAL METHODS: Primary imaging techniques for lung imaging are x­ray and computed tomography (CT) to monitor disease severity and regional distribution. METHODICAL INNOVATIONS: Radiation-free imaging techniques such as magnetic resonance imaging (MRI) have gained interest over the last decade in order to prevent radiation damage. PERFORMANCE: The main findings of CF lung disease are airway wall thickening, bronchiectasis, and mucus plugging, which are found in up to 60% of preschool age children. Pleural abnormalities and consolidations are often associated with pulmonary exacerbation. Young CF patients often show a mosaic pattern as functional changes and also perfusion defects can be seen from birth in 50% of CF patients by contrast-enhanced perfusion imaging, and in up to 90% of adult patients, with varying degrees of severity. Dilated bronchial arteries indicate an increased risk for hemoptysis. ACHIEVEMENTS: Proton MRI is the sole imaging technique that can show structural and functional lung changes in one examination. Structured assessment using a scoring system helps to systematically grade the extent and severity of all CF-associated changes. CONCLUSIONS: Lung MRI for cystic fibrosis has been recently established as a clinical standard examination and is routinely performed at experienced centers. More recently, it has also been used as an endpoint within the framework of clinical studies.

[Cystic fibrosis and computed tomography of the lungs]. / Computertomographie der Lunge bei Mukoviszidose.

With its high detail of morphological changes in lung parenchyma and airways as well as the possibilities for three-dimensional reconstruction, computed tomography (CT) represents a solid tool for the diagnosis and follow-up in patients suffering from cystic fibrosis (CF). Guidelines for standardized CT image acquisition in CF patients are still missing. In the mostly younger CF patients, an important issue is the well-considered use of radiation in CT imaging. The use of intravenous contrast agent is mainly restricted to acute emergency diagnostics. Typical morphological findings in CF lung disease are bronchiectasis, mucus plugging, or signs of decreased ventilation (air trapping) which can be detected with CT even in early stages. Various scoring systems that have become established over time are used to grade disease severity and for structured follow-up, e.g., in clinical research studies. With the technical development of CT, a number of postprocessing software tools were developed to help clinical reporting and overcome interreader differences for a standardized quantification. As an imaging modality free of ionizing radiation, magnetic resonance imaging (MRI) is becoming increasingly important in the diagnosis and follow-up of CF patients and is already frequently a substitute for CT for long-term follow-up at numerous specialized centers.

[CF Lung Disease - a German S3 Guideline: Module 2: Diagnostics and Treatment in Chronic Infection with Pseudomonas aeruginosa]. / S3-Leitlinie: Lungenerkrankung bei Mukoviszidose ­ Modul 2: Diagnostik und Therapie bei der chronischen Infektion mit Pseudomonas aeruginosa.

Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. 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 epithelial lining fluid which leads to chronic inflammation of the airways. Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90 % of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. Pseudomonas aeruginosa (Pa) is the most relevant pathogen in the course of CF lung disease. Colonization and chronic infection are leading to additional loss of pulmonary 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 for Pa-infection in order to give guidance for individual treatment options.

Dephasing and diffusion on the alveolar surface.

We propose a surface model of spin dephasing in lung tissue that includes both susceptibility and diffusion effects to provide a closed-form solution of the Bloch-Torrey equation on the alveolar surface. The nonlocal susceptibility effects of the model are validated against numerical simulations of spin dephasing in a realistic lung tissue geometry acquired from synchotron-based µCT data sets of mouse lung tissue, and against simulations in the well-known Wigner-Seitz model geometry. The free induction decay is obtained in dependence on microscopic tissue parameters and agrees very well with in vivo lung measurements at 1.5 Tesla to allow a quantification of the local mean alveolar radius. Our results are therefore potentially relevant for the clinical diagnosis and therapy of pulmonary diseases.

[Incidental findings in chest X­rays]. / Zufallsbefunde im Röntgenthorax.

Conventional projection radiography (chest x­ray) is one of the most frequently requested procedures in radiology. Even though chest x­ray imaging is frequently performed in asymptomatic patients for preoperative assessment, clinically relevant incidental findings are relatively scarce. This is due to the relatively low sensitivity of chest x­rays where few clinically relevant incidental findings are to be expected, as any detectable pathologies will as a rule already be clinically symptomatic. Recommendations from relevant societies for the management of incidental findings, apart from the clarification of incidental nodules, do not exist. This review article therefore describes the most frequent and typical incidental findings of lung parenchyma (apart from pulmonary nodules), mediastinal structures including the hilum of the lungs, pleura, chest wall and major vessels. Also described are those findings which can be diagnosed with sufficient certainty from chest x­rays so that further clarification is not necessary and those which must be further clarified by multislice imaging procedures or other techniques.

Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation.

UNLABELLED: Progressive lung disease in cystic fibrosis (CF) is the life-limiting factor of this autosomal recessive genetic disorder. Increasing implementation of CF newborn screening allows for a diagnosis even in pre-symptomatic stages. Improvements in therapy have led to a significant improvement in survival, the majority now being of adult age. Imaging provides detailed information on the regional distribution of CF lung disease, hence longitudinal imaging is recommended for disease monitoring in the clinical routine. Chest X-ray (CXR), computed tomography (CT) and magnetic resonance imaging (MRI) are now available as routine modalities, each with individual strengths and drawbacks, which need to be considered when choosing the optimal modality adapted to the clinical situation of the patient. CT stands out with the highest morphological detail and has often been a substitute for CXR for regular severity monitoring at specialized centers. Multidetector CT data can be post-processed with dedicated software for a detailed measurement of airway dimensions and bronchiectasis and potentially a more objective and precise grading of disease severity. However, changing to CT was inseparably accompanied by an increase in radiation exposure of CF patients, a young population with high sensitivity to ionizing radiation and lifetime accumulation of dose. MRI as a cross-sectional imaging modality free of ionizing radiation can depict morphological hallmarks of CF lung disease at lower spatial resolution but excels with comprehensive functional lung imaging, with time-resolved perfusion imaging currently being most valuable. KEY POINTS: • Hallmarks are bronchiectasis, mucus plugging, air trapping, perfusion abnormalities, and emphysema.• Imaging is more sensitive to disease progression than lung function testing.• CT provides the highest morphological detail but is associated with radiation exposure.• MRI shows comparable sensitivity for morphology but excels with additional functional information.• MRI sensitively depicts reversible abnormalities such as mucus plugging and perfusion abnormalities. Citation Format: • Wielpütz MO, Eichinger M, Biederer J et al. Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation. Fortschr Röntgenstr 2016; 188: 834 - 845.

[MRI of interstitial lung diseases: what is possible?]. / MRT bei interstitiellen Lungenerkrankungen : Was ist möglich?

BACKGROUND: Magnetic resonance imaging (MRI) of the lungs is becoming increasingly appreciated as a third diagnostic imaging modality besides chest x-ray and computed tomography (CT). Its value is well acknowledged for pediatric patients or for scientific use particularly when radiation exposure should be strictly avoided. However, the diagnosis of interstitial lung disease is the biggest challenge of all indications. The objective of this article is a summary of the current state of the art for diagnostic MRI of interstitial lung diseases. MATERIAL AND METHODS: This article reflects the results of a current search of the literature and discusses them against the background of the authors own experience with lung MRI. RESULTS: Due to its lower spatial resolution and a higher susceptibility to artefacts MRI does not achieve the sensitivity of CT for the detection of small details for pattern recognition (e.g. fine reticulation and micronodules) but larger details (e.g. coarse fibrosis and honeycombing) can be clearly visualized. Moreover, it could be shown that MRI has the capability to add clinically valuable information on regional lung function (e.g. ventilation, perfusion and mechanical properties) and inflammation with native signal and contrast dynamics. DISCUSSION: In its present state MRI can be used for comprehensive cardiopulmonary imaging in patients with sarcoidosis or for follow-up of lung fibrosis after initial correlation with CT. Far more indications are expected when the capabilities of MRI for the assessment of regional lung function and activity of inflammation can be transferred into robust protocols for clinical use.

In vivo monitoring of cystic fibrosis-like lung disease in mice by volumetric computed tomography.

The onset and spontaneous development of cystic fibrosis (CF) lung disease remain poorly understood. In the present study, we used volumetric computed tomography (VCT) as a new method for longitudinal in vivo monitoring of early lesions and disease progression in CF-like lung disease in ß-epithelial Na(+) channel (ENaC)-transgenic (TG) mice. Using a VCT scanner prototype (80 kV, 50 mA·s, scan time 19 s and spatial resolution 200 µm), ßENaC-TG mice and wild-type (WT) littermates were examined longitudinally at 10 time-points from neonatal to adult ages, and VCT images were assessed by qualitative and quantitative morphological parameters. We demonstrate that VCT detected early-onset airway mucus obstruction, diffuse infiltrates, atelectasis and air trapping as characteristic abnormalities in ßENaC-TG mice. Furthermore, we show that early tracheal mucus obstruction predicted mortality in ßENaC-TG mice and that the density of lung parenchyma was significantly reduced at all time-points in ßENaC-TG compared with WT mice (median ± sem -558 ± 8 HU in WT versus -686 ± 16 HU in ßENaC-TG at 6 weeks of age; p < 0.005). Our study demonstrates that VCT is a sensitive, noninvasive technique for early detection and longitudinal monitoring of morphological abnormalities of CF-like lung disease in mice, and may thus provide a useful tool for pre-clinical in vivo evaluation of novel treatment strategies for CF.

Percutaneous treatment of biliary cast syndrome after orthotopic liver transplantation: comparison of mechanical versus hydraulic rheolytic cast extraction.

PURPOSE: Biliary cast syndrome (BCS) is the presence of casts within the intrahepatic or extrahepatic biliary system after orthotopic liver transplantation. Our work compares two percutaneous methods for BCS treatment: the mechanical cast-extraction technique (MCE) versus the hydraulic cast-extraction (HCE) technique using a rheolytic system. MATERIALS AND METHODS: A total of 24 patients were included in the study. Six patients were referred for HCE, and 18 patients were treated with MCE. A statistically significant larger number of sessions was required in the MCE group (21.0, range 11 to 72 sessions) (p = 0.033). RESULTS: Median therapy duration was shorter in the HCE group at 2.4 months (range 2 to 5) compared with 6.7 months (range 3 to 39) in the MCE group (p < 0.001). Both patient acceptance was better and costs for total therapy were 40% less in the HCE group. No significant differences where found concerning clinical and biochemical improvement or graft and patient survival. CONCLUSION: The use of the hydraulic rheolytic system decreased total therapy time, thereby decreasing the induced inflammation time of the biliary tree. A significant benefit of HCE has been observed in our patients when we compare our results with those of MCE.