Type Scientific Poster Presentation Topic Pediatric RadiologyPurpose Cystic fibrosis (CF) is characterized by chronic respiratory infections and functional impairment of the lung. Lung function tests such as nitrogen multiple breath washout (N2-MBW), are sensitive in detecting ventilation inhomogeneity, but cannot determine its exact origin. Novel magnetic resonance imaging (MRI) methods such as matrix pencil decomposition MRI can visualize functional changes in the lung without the administration of contrast agents and the need for breathing maneuvers.Objectives: To examine the correlation between novel functional MRI and lung function tests in patients with CF. Methods and MaterialsMethods: Forty patients with CF (mean age 11.7 years, range 6-18) underwent MRI and lung function tests on the same day. Functional MRI provided semiquantitative measures of the perfusion (RQ) and ventilation (RFV) impairment as percentages of the affected lung volume. Morphological MRI was evaluated using a CF-specific score. N2-MBW provided information about global (lung clearance index, LCI) ventilation inhomogeneity. ResultsResults: MRI detected functional impairment in all patients with CF: RFV ranged from 19% to 38% and RQ ranged from 16% to 35%. RFV and RQ were strongly correlated with LCI (r=0.76, p<0.001; r=0.85, p<0.001, respectively), as well as total morphology scores and sub-scores. ConclusionConclusions: Non-invasive functional MRI is a promising method to detect and visualize perfusion and ventilation impairment in CF without the need of contrast agents or breath holding maneuvers. AffirmationsAuthors agreement: I confirm that all authors indicated within the author block of this abstract have been informed about this abstract submission and agreed to the same. (I confirm) Mandatory presenter registration: I understand that if the presenting author has not paid his registration fee by April 24, 2017, he will not be permitted to display the poster or hold the presentation and the abstract will not be published. (I agree)Copyright: I affirm that if my poster contains any material that has been previously published, I was entitled to use this material by applicable law or have obtained a transferable license from the copyright holder. (I confirm) Seite

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A dysregulated immune response with hyperinflammation is observed in patients with severe coronavirus disease 2019 (COVID-19). The aim of the present study was to assess the safety and potential benefits of human recombinant C1 esterase inhibitor (conestat alfa), a complement, contact activation and kallikrein-kinin system regulator, in severe COVID-19. Patients with evidence of progressive disease after 24 h including an oxygen saturation <93% at rest in ambient air were included at the University Hospital Basel, Switzerland in April 2020. Conestat alfa was administered by intravenous injections of 8400 IU followed by 3 additional doses of 4200 IU in 12-h intervals. Five patients (age range, 53-85 years; one woman) with severe COVID-19 pneumonia (11-39% lung involvement on computed tomography scan of the chest) were treated a median of 1 day (range 1-7 days) after admission. Treatment was well-tolerated. Immediate defervescence occurred, and inflammatory markers and oxygen supplementation decreased or stabilized in 4 patients (e.g., median C-reactive protein 203 (range 31-235) mg/L before vs. 32 (12-72) mg/L on day 5). Only one patient required mechanical ventilation. All patients recovered. C1INH concentrations were elevated before conestat alfa treatment. Levels of complement activation products declined after treatment. Viral loads in nasopharyngeal swabs declined in 4 patients. In this uncontrolled case series, targeting multiple inflammatory cascades by conestat alfa was safe and associated with clinical improvements in the majority of severe COVID-19 patients. Controlled clinical trials are needed to assess its safety and efficacy in preventing disease progression.

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Both functional and structural changes of the subchondral bone in terms of scintigraphic osseous activity and the presence and degree of BMEP were significantly associated with cartilage lesions in patients with OA of the knee. This association was pronounced with full thickness lesions, indicating a possible protective effect of the cartilage layer for the subjacent bone.

Background The clinical characteristics of human metapneumovirus (hMPV)-associated lower respiratory tract infection (LRTI) after allogeneic hematopoietic stem cell transplantation (HSCT) is not well described. We describe the clinical course in eight HSCT recipients suffering from hMPV infection. Methods We prospectively included all patients with hMPV-associated LRTI after allogeneic HSCT during a period of 1 year. hMPV was diagnosed by multiplex polymerase chain reaction (PCR) from bronchoalveolar lavage (BAL). Results Eight patients with hMPV-associated LRTI were identified from 93 BAL samples. Three of the eight patients had co-infections with other pathogens. The median age of the patients was 45 years [interquartile range (IQR) 36.8-53.5], the median time posttransplant was 473 days (IQR 251-1,165), 5/8 patients had chronic graft-versus-host disease (cGvHD), and 6/8 patients received immunosuppression. Chest computed tomography (CT) scanning showed a ground-glass pattern in 7/8 patients. Seven of eight patients required hospitalization due to severe symptoms and hypoxemia. All were treated with intravenous immunoglobulin (IVIG), which was combined with oral ribavirin in six patients. The mortality rate was 12.5 % (1/8).Conclusions hMPV-associated LRTI in allogeneic HSCT recipients are not uncommon and present with unspecific respiratory symptoms, ground-glass pattern in CT scanning, and co-infection.

Rationale: Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by heterogeneous airway disease. Traditional lung function techniques (e.g. spirometry) may underestimate severity and complexity of PCD. Objectives: We assessed lung impairment in individuals with PCD using structural and functional magnetic resonance imaging (MRI) and different lung function techniques. Methods: Thirty study participants with PCD (median 13.4 years, range 5-28) underwent structural and functional MRI, spirometry, and multiple breath washout (MBW) on the same day. Primary endpoints included structural MRI morphology scores, relative ventilation and perfusion impairment from functional MRI, FEV 1 from spirometry, and lung clearance index (LCI) from MBW. Results: Severity and complexity of PCD lung disease varied significantly between individuals. Structural lung disease was detected in all subjects with a median (IQR) extent score of 10.3 (7 to 19; maximum score = 60). Functional MRI ventilation impairment was present in 52% of subjects affecting 24.2% (21.1 to 25.2%) of the lung. Relative perfusion impairment was detected in 78% of individuals affecting 21.1% (19.4 to 25.9%) of the lung. LCI was abnormal in 83% (median 8.3 (2.6 to 13.2) z-scores) and FEV 1 was abnormal in 27% (-0.5 (-1.6 to 0.3) zscores) of individuals. Concordance between spirometry and imaging outcomes was poor, with 52% of patients showing both abnormal MRI and LCI values, but normal FEV 1. Conclusions: Discordance between lung function and imaging outcomes in patients with PCD supports the use of both imaging and lung function, such as MBW, for surveillance of this heterogeneous disease.

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