Publications by authors named "Cordula Koerner-Rettberg"

Article Synopsis
  • * Data were collected from 408 PCD patients across 12 countries, focusing on their lung function and the presence of common respiratory pathogens, with a significant finding that those with certain pathogens had lower lung function scores.
  • * The results indicated that certain pathogens were strongly associated with decreased lung function, particularly in adults, highlighting the need for early eradication strategies and timely treatment of infections in PCD patients.
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  • Maintaining physical fitness is crucial for adults with cystic fibrosis (pwCF), but there is limited data on how new therapies like ETI affect their fitness levels over time.
  • A study tracked 28 adult pwCF for about 5.6 years, measuring various physical fitness components and health indicators like BMI and FEV1, finding significant improvements in BMI and some health-related fitness measures after starting ETI therapy.
  • While ETI therapy showed some positive effects, the overall impact on fitness was small, suggesting that other factors also play a role in achieving high physical fitness in pwCF.
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  • Habitual physical activity and exercise training are crucial for improving health and quality of life in people with cystic fibrosis (pwCF), particularly with the advent of new CFTR modulator therapies like Elexacaftor/Tezacaftor/Ivacaftor (ETI).
  • A study was conducted to evaluate the effects of ETI on habitual physical activity, measuring steps per day and intensity among adult pwCF over time, with a focus on data collected during the post-pandemic period.
  • Results showed that pwCF on ETI increased daily steps by 25%, while those not receiving ETI saw a slight decline; however, changes in lung function were significant for those on
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Purpose: In late 2022, a surge of severe S. pyogenes infections was reported in several European countries. This study assessed hospitalizations and disease severity of community-acquired bacterial infections with S.

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Background: The influence of habitual physical activity and exercise capacity on health-related quality of life (HRQoL) in people with cystic fibrosis (pwCF) is poorly characterized. This study investigated the influence of habitual physical activity, exercise capacity, lung function, and body mass index (BMI) on HRQoL in adolescent and adult pwCF.

Method: Subjects were fitted with an accelerometer to determine habitual physical activity (steps/day), including time spent at different intensities, for up to 4 weeks.

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Article Synopsis
  • The study examines the impact of the Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy on health-related quality of life (HRQoL) in adults with cystic fibrosis (pwCF) over a period of about 5.6 years, comparing those who received ETI to those who did not.
  • It found that ETI significantly improved several HRQoL domains, while pwCF not on ETI saw little improvement in psychosocial factors and decline in physical health over time.
  • The findings suggest ETI therapy enhances both HRQoL and clinical outcomes, but disease progression appears to affect physical health more negatively than psychological aspects; however
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Low nasal nitric oxide (nNO) is a typical feature of Primary Ciliary Dyskinesia (PCD). nNO is part of the PCD diagnostic algorithm due to its discriminative power against other lung diseases, such as cystic fibrosis (CF). However, the underlying pathomechanisms are elusive.

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Background: Asthma is increasingly recognized as heterogeneous, characterized by different endotypes, with obesity not only a distinct phenotype but a risk factor for severe asthma.

Objective: We sought to understand the associations of obesity with relevant parameters of severe asthma, including asthma control, disease burden, and lung function.

Methods: The German Asthma Net registry is a multicenter international real-life registry capturing long-term follow-up data.

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Background: PCD is a genetic disease leading to a decline in pulmonary function. There is only little knowledge of factors determining the long-term pulmonary outcome. Especially adherence has not been addressed yet although being an independent risk factor for an increased loss of lung capacity in other chronic respiratory diseases.

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  • Physical activity (PA) is a key part of cystic fibrosis (CF) treatment, but barriers to participation need to be identified and managed, prompting this study to assess those obstacles.
  • The study involved 88 individuals with CF, who completed a questionnaire about their physical and psychosocial barriers to exercise before and during a 12-month exercise program, revealing that physical barriers were more significant than psychosocial ones.
  • Supervision during the exercise program did not significantly improve barrier management, highlighting the need for personalized discussions about individual barriers and strategies to encourage more PA in daily life.
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  • The study aimed to explore socio-familial and behavioral traits in patients with functional respiratory disorders compared to controls using psychological questionnaires, while also looking at symptom outcomes 6 months post-intervention.
  • A total of 164 participants (106 patients and 58 controls) were involved, revealing higher scores for anxious and schizoid behaviors in patients according to the child behavior checklist.
  • After 6 months, a significant percentage of patients showed symptom improvement, and parents reported reduced burden and impairment, yet psychological traits remained stable with no significant changes noted across the questionnaires.
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Background: No results of controlled trials are available for any of the few treatments offered to children with interstitial lung diseases (chILD). We evaluated hydroxychloroquine (HCQ) in a phase 2, prospective, multicentre, 1:1-randomized, double-blind, placebo-controlled, parallel-group/crossover trial. HCQ (START arm) or placebo were given for 4 weeks.

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  • The study aimed to evaluate a monitored exercise program's impact on aerobic fitness in children with cystic fibrosis (CF), involving six participants aged 6 to 14.
  • After 12 months, aerobic fitness measures (VO2peak and Wpeak) showed slight improvements during the monitoring phase but remained stable post-monitoring.
  • The findings suggest that continuous exercise monitoring may enhance physical fitness and encourage ongoing physical activity in CF children, highlighting the importance of support from exercise therapists.
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Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in children, adolescents and young adults with PCD. We analysed data from 486 patients with repeated lung function measurements obtained between the age of 6 and 24 years from the International PCD Cohort and calculated z-scores for forced expiratory volume in 1 s (FEV), forced vital capacity (FVC) and FEV/FVC ratio using the Global Lung Function Initiative 2012 references.

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Introduction: L-Arginine (Arg) is a semi-essential amino acid. Constitutive and inducible nitric oxide synthase (NOS) isoforms convert Arg to nitric oxide (NO), a potent vaso- and bronchodilator with multiple biological functions. Atopic dermatitis (AD) and bronchial asthma (BA) are atopic diseases affecting many children globally.

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  • A study assessed the motor performance of youth with cystic fibrosis (CF) across different age groups and compared their results to healthy peers, revealing that CF patients had significantly lower fitness levels.
  • Children with CF showed steady improvement in motor performance and physical activity until age 14, after which their progress plateaued or declined.
  • The findings suggest the need for tailored exercise programs that include aerobic, strength, and neuromuscular training to help improve the fitness and motivation of children and adolescents with CF, especially in older age groups.
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Background: Regular physical activity plays an important role in the treatment of patients with cystic fibrosis (CF). This study is aimed at investigating the effects of a 12-month partially supervised exercise program on attributes of health-related and motor performance fitness, lung function (ppFEV1), BMI, and habitual physical activity (HPA, steps/day) in adults with CF.

Methods: Attributes of health-related and motor performance fitness were examined at the beginning (T0), after 6 (T1), and 12 months (T2) on the basis of five test items: forward bend (FB), bent knee hip extension (HE), plank leg raise (PLR), standing long jump (SLJ), and standing on one leg (OLS).

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Background: Lung clearance index (LCI) is a promising lung function outcome in individuals with primary ciliary dyskinesia (PCD). The impact of events clinically important for individuals with PCD, such as pulmonary exacerbations, on LCI is unknown.

Methods: We conducted an international, multicentre, observational cohort study to assess the association of LCI and risk of pulmonary exacerbation, specific changes in LCI during pulmonary exacerbation and global variability of LCI across four visits every 4 months.

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Article Synopsis
  • * Twenty-two children aged 6-17 participated in a 12-month exercise program where they were tested on their flexibility, balance, strength, and overall motor performance at three points in time (before, halfway through, and after the program).
  • * Results showed improvement in motor skills during the monitored phase, but no further gains were made afterward, highlighting the importance of consistent supervision in maintaining physical activity motivation for better results in CF patients.
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