Publications by authors named "Svenja Strassburg"
Cystic fibrosis bone disease (CFBD) is a common comorbidity in adult people with cystic fibrosis (pwCF), resulting in an increased risk of bone fractures. This study evaluated the capacity of artificial intelligence (AI)-assisted low-dose chest CT (LDCT) opportunistic screening for detecting low bone mineral density (BMD) in adult pwCF. In this retrospective single-center study, 65 adult pwCF (mean age 30.
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- Poor nutritional status negatively impacts lung function and survival in people with cystic fibrosis (pwCF).
- A study using deep-learning analyzed body composition changes in 66 adults with CF before and after elexacaftor/tezacaftor/ivacaftor (ETI) therapy, finding significant increases in adipose tissue but only minimal improvements in muscle ratio.
- The results indicate that ETI therapy mainly influences fat tissue, particularly in underweight patients, which may inform future nutritional strategies for managing CF.
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- Cystic fibrosis (CF) is characterized by immune dysregulation and chronic inflammation, and this study focused on the effects of the CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) on associated inflammation in patients with CF.
- Researchers analyzed plasma samples from 51 patients before, three months, and six months after starting ETI therapy, measuring various pro-inflammatory chemokines.
- Results showed significant improvements in lung function and decreases in specific inflammatory markers, particularly those linked to neutrophilic inflammation, indicating ETI's anti-inflammatory effects.
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- The study investigates the personality traits of adult patients with cystic fibrosis (CF) and their relationship to health-related quality of life (HRQoL) and clinical severity indicators.
- Seventy adults completed specific questionnaires, revealing that CF patients displayed varying personality traits compared to the norm, with two distinct personality clusters identified based on their scores.
- Results indicate that psychological factors, particularly personality, significantly affect HRQoL in CF patients, suggesting that those with pronounced personality traits could benefit from psychosocial support to improve their quality of life.
Recently, cystic fibrosis transmembrane regulator modulator therapy with elexacaftor/tezacaftor/ivacaftor has become available for children with cystic fibrosis (CF) carrying at least one mutation. To assess the intermediate term effects of elexacaftor/tezacaftor/ivacaftor in children with cystic fibrosis in a real-world setting. We performed a retrospective analysis of records of children with cystic fibrosis, who started elexacaftor/tezacaftor/ivacaftor between 8/2020 and 10/2022.
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- Cystic fibrosis (CF) lung disease features chronic infections and immune dysfunction, and CFTR modulators like elexacaftor/tezacaftor/ivacaftor have been shown to improve clinical outcomes in patients with CF, but their effect on inflammation is unclear.* -
- In a study of 77 patients, 3 months of treatment with elexacaftor/tezacaftor/ivacaftor resulted in significant improvements in lung function (12.5% increase in FEV1) and a marked increase in regulatory T cells (Tregs) by 18.7%, especially in those clearing Pseudomonas aeruginosa.* -
- Additionally, there was a
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- Obstructive sleep apnea (OSA), nocturnal hypoxemia, and excessive daytime sleepiness (EDS) are frequently observed in adults with cystic fibrosis (pwCF), highlighting the need for better understanding of sleep-related issues.* -
- In a study of 52 adult pwCF, 40% exhibited abnormal apnea-hypopnea indices, with 25% experiencing nocturnal hypoxemia, while 15% reported significant EDS as measured by the Epworth Sleepiness Scale.* -
- Despite the prevalence of these sleep disorders, no strong predictive factors were identified; the study recommends routine sleep assessments (polysomnography and Epworth Sleepiness Scale) for adult pwCF regardless of their general health
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- Cough suppression is common in adults with cystic fibrosis (CF) and negatively affects their health-related quality of life (HRQoL).
- A study with 71 adults found that while cough suppression is present, it does not significantly correlate with clinical measures of disease severity like BMI or lung function.
- Mental health factors appear to play a role in cough suppression, indicating a need for further research in this area.
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- People with cystic fibrosis (pwCF) often experience sleep-disordered breathing (SDB) and disturbed sleep, which are frequently overlooked.
- A study was conducted to assess the impact of the CFTR triple combination therapy, elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA), on sleep quality and related parameters in adult pwCF, using polysomnography before and after starting the treatment.
- Results showed significant improvements in lung function, body mass index (BMI), and a decrease in sleep-disordered breathing metrics, while other sleep measures like total sleep time and efficiency remained unchanged.
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- Sphingolipids, particularly ceramides, are implicated in cystic fibrosis (CF) lung disease, with a specific ratio of ceramides linked to inflammation and disease severity in people with CF (PWCF).
- Research analyzed sphingolipid levels in serum from 112 PWCF and 96 healthy individuals, and evaluated changes in levels before and after CFTR modulator treatment (elexacaftor/tezacaftor/ivacaftor, or ELX/TEZ/IVA) using advanced mass spectrometry techniques.
- Results showed PWCF had higher long-chain and very long-chain ceramides compared to healthy controls; treatment with ELX/TEZ/IVA reduced certain ceramide levels
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- - The study investigated how respiratory rate (RR) in patients with cystic fibrosis (pwCF) relates to factors like antibiotic treatment, exacerbation status, lung function (FEV1), and inflammation (CRP) over a one-year period.
- - Researchers enrolled 47 pwCF, measuring RR and heart rate during hospital stays, noting that patients with exacerbations had higher RR compared to those without and that RR decreased for both groups by discharge.
- - The results indicated a correlation between RR and FEV1 (lung function) as well as a positive correlation with CRP (marker of inflammation), suggesting that monitoring RR could provide valuable insights into the clinical status of pwCF during treatment.
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- * Researchers developed a specific interferon-γ release assay to detect immune responses to MABC by using overexpressed proteins to stimulate immune cells, with results indicating stronger responses in infected subjects compared to controls.
- * The findings suggest that the protein MAB_0405c significantly correlates with MABC infection, with promising diagnostic potential reflected in high sensitivity and specificity rates from testing, especially in CF patients.
Background: Chronic infection and an exaggerated inflammatory response are key drivers of the pathogenesis of cystic fibrosis (CF), especially CF lung disease. An imbalance of pro- and anti-inflammatory mediators, including dysregulated Th2/Th17 cells and impairment of regulatory T cells (Tregs), maintain CF inflammation. CF transmembrane conductance regulator (CFTR) modulator therapy might influence these immune cell abnormalities.
View Article and Find Full Text PDFPurpose: In our study we aimed to analyze sleep variability and activity in patients with cystic fibrosis (CF) during their hospital stay.
Methods: Forty-three CF patients were recruited and have been divided into two subgroups: exacerbated (n = 18) and non-exacerbated (n = 25). During the course of their hospital stay we used VitaLog, a minimal-impact biomotion device, in order to determine total sleep time (TST), time in bed (TIB), sleep efficiency (SE) and intra patient standard deviation (IPSD) of TST.
Background: CFTR modulator therapy with ivacaftor is a treatment option for Cystic Fibrosis (CF) patients with at least one copy of a R117H-7T mutation in the CFTR gene. Desirable effects of this therapy are improvement of lung function, decrease in exacerbation rate, normalization or reduction of sweat chloride and weight gain. Monogenetic CF-twins carry identical genetic information, so therapy response and side effects are expected to be nearly identical under this specific therapy.
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