PiZZ (Glu342Lys) α1-antitrypsin deficiency (AATD) is characterized by intrahepatic AAT polymerization and is a risk factor for liver disease development in children. The majority of PiZZ children are disease free, hence this mutation alone is not sufficient to cause the disease. We investigated Z-AAT polymers and the expression of fibrosis-related genes in liver tissues of PiZZ children with different clinical courses.
View Article and Find Full Text PDFAnaerobic methanotrophic (ANME) archaea obtain energy from the breakdown of methane, yet their extrachromosomal genetic elements are little understood. Here we describe large plasmids associated with ANME archaea of the Methanoperedens genus in enrichment cultures and other natural anoxic environments. By manual curation we show that two of the plasmids are large (155,605 bp and 191,912 bp), circular, and may replicate bidirectionally.
View Article and Find Full Text PDFAmong patients with bronchiectasis, nontuberculous mycobacterial pulmonary disease (NTM-PD) ranged between 1 and 6% and it is suspected that its prevalence is underestimated. Our aim was to evaluate differences in computed tomography (CT) features in patients with bronchiectasis, with and without NTM-PD, in order to facilitate earlier diagnosis in the future. In addition, we evaluated longitudinal changes after successful NTM-PD treatment.
View Article and Find Full Text PDFAnti-infective treatment of pulmonary exacerbations is a major issue in people with cystic fibrosis (CF). Individualized dosing strategies and adaptation of infusion times are important concepts to optimize anti-infective therapy. In this prospective non-randomized controlled open-label trial, we compared pharmacokinetics of meropenem in 12 people with CF experiencing a pulmonary exacerbation, of whom six received parenteral meropenem 2 g tid as short infusion over 30 min and six extended infusion over 120 min.
View Article and Find Full Text PDFObjective: There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF). We aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions.
Methods: We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 interstitial lung disease expert centres in Germany.
Purpose To quantify regional lung ventilation in patients with chronic obstructive pulmonary disease (COPD) by using free-breathing dynamic fluorinated (fluorine 19 [F]) gas magnetic resonance (MR) imaging. Materials and Methods In this institutional review board-approved prospective study, 27 patients with COPD were examined by using breath-hold F gas wash-in MR imaging during inhalation of a normoxic fluorinated gas mixture (perfluoropropane) and by using free-breathing dynamic F gas washout MR imaging after inhalation of the gas mixture was finished for a total of 25-30 L. Regional lung ventilation was quantified by using volume defect percentage (VDP), washout time, number of breaths, and fractional ventilation (FV).
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