A5060547883- Cystic Fibrosis Research Advances
- Child Nutrition and Feeding Issues
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Neonatal Respiratory Health Research
- Pediatric health and respiratory diseases
- Inhalation and Respiratory Drug Delivery
- Tracheal and airway disorders
- Obstructive Sleep Apnea Research
- Immunodeficiency and Autoimmune Disorders
- Asthma and respiratory diseases
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Dysphagia Assessment and Management
- Neuroscience of respiration and sleep
- Advanced X-ray and CT Imaging
- Medical Imaging and Pathology Studies
- Children's Physical and Motor Development
- Infant Development and Preterm Care
- Pulmonary Hypertension Research and Treatments
- Tuberculosis Research and Epidemiology
- Sleep and related disorders
- Radiation Dose and Imaging
- Respiratory and Cough-Related Research
- Pneumocystis jirovecii pneumonia detection and treatment
- Behavioral Health and Interventions
- Neonatal Health and Biochemistry
Ruhrlandklinik
2019-2025
Essen University Hospital
2021-2025
University of Duisburg-Essen
2019-2024
St. Josef-Hospital
2023
Ruhr University Bochum
2023
Agaplesion Bethesda Krankenhaus
2017
Cardiovascular Center Bethanien
2005
Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical outcomes in people cystic fibrosis (pwCF) at least one F508del allele. This study evaluated the real-world impact of ETI on lung function, nutritional status, pulmonary exacerbation frequency, and sweat chloride concentrations a large group pwCF.This observational cohort used data from German CF Registry for pwCF who received therapy were followed up period 12 months.The included 2645 67 centres Germany (mean...
Introduction: Recently, cystic fibrosis transmembrane regulator modulator therapy with elexacaftor/tezacaftor/ivacaftor has become available for children (CF) carrying at least one F508del mutation. Objective: To assess the intermediate term effects of in a real-world setting. Methods: We performed retrospective analysis records fibrosis, who started between 8/2020 and 10/2022. Pulmonary function tests, nutritional status, sweat chloride laboratory data were assessed before, 3 6 months after...
Abstract A poor nutritional status is associated with worse pulmonary function and survival in people cystic fibrosis (pwCF). CF transmembrane conductance regulator modulators can improve body weight, but more data needed to evaluate its effects on composition. In this retrospective study, a pre-trained deep-learning network was used perform fully automated composition analysis chest CTs from 66 adult pwCF before after receiving elexacaftor/tezacaftor/ivacaftor (ETI) therapy. Muscle adipose...
Left atrial thrombi are an important cause for embolism-related morbidity and mortality. Transesophageal echocardiography (TEE), the clinical reference, is semiinvasive; thus, we aimed to assess value of contrast-enhanced cardiovascular MRI detection thrombus in left appendage.The image quality was good both 2D perfusion (grade 4 +/- 1) 3D turbo fast low-angle shot (FLASH) 1, n.s.). Compared with TEE, perfusion, turboFLASH, combination techniques yielded sensitivities 47/35/44%,...
Introduction Cystic fibrosis (CF), especially CF lung disease, is characterized by chronic infection, immune dysfunction including impairment of regulatory T cells (Tregs) and an exaggerated inflammatory response. transmembrane conductance regulator (CFTR) modulators have shown to improve clinical outcomes in people with (PwCF) a wide range CFTR mutations. However, it remains unclear whether modulator therapy also affects CF-associated inflammation. We aimed examine the effect...
Background Habitual physical activity (PA) and exercise training are accepted as important aspects of care for people with cystic fibrosis (pwCF) to improve health-related measures fitness, which in turn have a positive impact on quality life prognosis. In the last decade, effective CFTR modulator therapies become promising treatment pwCF by targeting underlying cause CF. This highly therapy improves clinical outcomes specific mutations. Little is known about longitudinal pattern PA or...
Purpose: Maintaining physical fitness plays an important role in the management of people with cystic fibrosis (pwCF). Longitudinal data on course and potential impact introduction highly effective CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) adult pwCF are scarce. Methods: Health-related skill-related components were assessed using incremental cycle test (Wpeak), plus forward bend (FB), prone bent knee hip extension (HE), plank leg raise (PLR), standing long jump (SLJ), one...
Abstract Liver-related side effects are a known complication of treatment with elexacaftor/tezacaftor/ivacaftor (ETI) for cystic fibrosis (CF). Gilbert’s syndrome is caused by genetic mutation that reduces activity the enzyme UDP glucuronosyltransferase 1 polypeptide A1 (UGT1A1), causing elevated levels unconjugated bilirubin in blood and duodenal bile. The presence CF might represent additive risk factors liver-related adverse events during ETI treatment. This case series describes six...
<b><i>Background:</i></b> Sleep-disordered breathing (SDB) and disturbed sleep are common, often underrecognized, comorbidities in people with cystic fibrosis (pwCF). <b><i>Objectives:</i></b> We studied the effect of CFTR triple combination therapy elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on pwCF. <b><i>Method:</i></b> This was a prospective, observational study clinically stable adult All participants underwent...
Cystic fibrosis (CF) is associated with dysregulated immune responses, exaggerated inflammation and chronic infection. CF transmembrane conductance regulator (CFTR) modulator therapies directly target the underlying protein defects resulted in significant clinical benefits for people (pwCF). This study analysed effects of triple CFTR therapy elexacaftor/tezacaftor/ivacaftor (ETI) on CF-associated inflammation, especially systemic chemokines.
In this proof of principle study, we evaluated the diagnostic accuracy novel Nox BodySleep
Abstract Background The present study evaluates personality traits in adult patients with cystic fibrosis (CF) and correlates these results health-related quality of life (HRQoL) other clinical parameters indicative disease severity. Methods Seventy adults completed the Cystic Fibrosis Questionnaire-Revised (CFQ-R 14+), a CF-specific measure HRQoL, self-administered questionnaire about disorders. Mean subscale scores prevalence extreme on `Persönlichkeits-Stil- und Störungs-Inventar (PSSI)´...
Abstract Objectives The aim of this study was to examine motor performance and trainability in youths with cystic fibrosis (CF). Methods Twenty‐two children adolescents (11 f/11 m), age range 6–17 years (11.3 ± 3.3 years), mean FEV1 91.0 21.7% pred.finished the partially monitored 12‐months exercise program. Patients performed Deutsche Motorik Test (DMT) assess flexibility, balance, strength, power totalmotor performance. An incremental ergometer cycle test used maximal capacity (Wpeak). All...
Obstructive sleep apnea (OSA), nocturnal hypoxemia and excessive daytime sleepiness (EDS) are common comorbidities in people with cystic fibrosis (pwCF). Most of the data showing this originates from children adolescents. The aim study was to collect on parameters, EDS pulmonary function a large cohort adult pwCF.Full overnight polysomnography (PSG) performed. determined using Epworth Sleepiness Scale (ESS). Demographic clinical (body mass index [BMI], function, capillary blood gases) were...
Carcinoid heart disease (Hedinger syndrome) is a long-term consequence in hormone-active neuroendocrine tumors with hepatic metastases and carcinoid syndrome. Because of serotonin, excess multiple cardiac pulmonary symptoms evolve, which are further complicated by patent foramen ovale due to right-left shunting. We present 53-year-old man an ileum-neuroendocrine tumor including gross liver stable who subsequently developed Hedinger Initially experiencing progressive dyspnea, he eventually...
Abstract Background The influence of habitual physical activity and exercise capacity on health‐related quality life (HRQoL) in people with cystic fibrosis (pwCF) is poorly characterized. This study investigated the activity, capacity, lung function, body mass index (BMI) HRQoL adolescent adult pwCF. Method Subjects were fitted an accelerometer to determine (steps/day), including time spent at different intensities, for up 4 weeks. Then bicycle ergometry (maximal capacity; Wpeak), function...