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View article: Progression of Structural Lung Disease in Different Aspergillus fumigatus Disease Phenotypes in Children with CF
Progression of Structural Lung Disease in Different Aspergillus fumigatus Disease Phenotypes in Children with CF Open
Aspergillus fumigatus (Asp) is frequently cultured from airways of children with Cystic Fibrosis (CwCF), but the impact on structural lung disease (SLD) remains unknown. In this retrospective study of 125 CwCF with a positive Asp airway cu…
View article: The impact of dornase alfa on imaging features of bronchiectasis
The impact of dornase alfa on imaging features of bronchiectasis Open
Background Nebulized dornase alfa is an approved therapy for persons with cystic fibrosis, but not for other causes of bronchiectasis. Nonetheless, dornase works by breaking down extracellular DNA in airway phlegm and should be effective i…
View article: Finding the optimal regimen for <i>Mycobacteroides abscessus</i> treatment (FOR<i>Ma</i>T) in people with <i>Mycobacteroides abscessus</i> pulmonary disease: a multicentre, randomised, multi-arm, adaptive platform trial
Finding the optimal regimen for <i>Mycobacteroides abscessus</i> treatment (FOR<i>Ma</i>T) in people with <i>Mycobacteroides abscessus</i> pulmonary disease: a multicentre, randomised, multi-arm, adaptive platform trial Open
Introduction Mycobacteroides abscessus (MABS) is within the non-tuberculous mycobacteria family. It inhabits soil and water, exhibits multi-antibiotic resistance and causes opportunistic lung infections, which may progress to symptomatic M…
View article: Validation of an artificial intelligence-based automated PRAGMA and mucus plugging algorithm in pediatric cystic fibrosis
Validation of an artificial intelligence-based automated PRAGMA and mucus plugging algorithm in pediatric cystic fibrosis Open
Our results show that AI-derived outcomes are not identical to visual PRAGMA-CF scores in size, but strongly correlated on measures of bronchiectasis, bronchial-disease and mucus plugging. They could therefore be a promising alternative fo…
View article: Pulmonary endpoints in clinical trials for children with cystic fibrosis under two years of age
Pulmonary endpoints in clinical trials for children with cystic fibrosis under two years of age Open
Cystic fibrosis is a lifelong progressive disease in which lung disease is the main prognostic factor, where starting early treatment is crucial for improving long-term outcomes. Therefore, new treatment should be available as early as pos…
View article: Early life elevations of methionine oxidation and ornithine track and predict cystic fibrosis structural lung disease
Early life elevations of methionine oxidation and ornithine track and predict cystic fibrosis structural lung disease Open
Introduction Early cystic fibrosis (CF) lung disease monitoring is crucial for understanding responses to therapy and preventing progressive pulmonary decline. Structural lung disease (SLD) is a major cause of pulmonary decline in CF. We a…
View article: Silence of the lungs: comparing measures of slow and noncommunicating lung units from pulmonary function tests with computed tomography
Silence of the lungs: comparing measures of slow and noncommunicating lung units from pulmonary function tests with computed tomography Open
We consider the term “trapped air” either in the use of imaging or pulmonary function testing, something of a misnomer that can lead to an inaccurate assessment of an important physiological feature. Instead, we propose the term underventi…
View article: Key inflammatory markers in bronchoalveolar lavage predict bronchiectasis progression in young children with CF
Key inflammatory markers in bronchoalveolar lavage predict bronchiectasis progression in young children with CF Open
High levels of IL-8, neutrophil%, ICOSGL and HGF in BALF may be good predictors for progression of bronchiectasis in young children with CF.
View article: Unlocking the potential of photon counting detector CT for paediatric imaging: a pictorial essay
Unlocking the potential of photon counting detector CT for paediatric imaging: a pictorial essay Open
Recent advancements in CT technology have introduced a revolutionary innovation to practice known as the Photon-Counting detector (PCD) CT imaging. The pivotal hardware enhancement of the PCD-CT scanner lies in its detectors, which consist…
View article: Automatic analysis of bronchus-artery dimensions to diagnose and monitor airways disease in cystic fibrosis
Automatic analysis of bronchus-artery dimensions to diagnose and monitor airways disease in cystic fibrosis Open
Background Cystic fibrosis (CF) lung disease is characterised by progressive airway wall thickening and widening. We aimed to validate an artificial intelligence-based algorithm to assess dimensions of all visible bronchus-artery (BA) pair…
View article: <i>Aspergillus</i>-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?
<i>Aspergillus</i>-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease? Open
In people with cystic fibrosis (PwCF), viscous sputum and dysfunction of the mucociliary escalator leads to early and chronic infections. The prevalence of Aspergillus fumigatus in sputum is high in PwCF and the contribution of A. fumigatu…
View article: A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis
A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis Open
Background The marked heterogeneity in cystic fibrosis (CF) disease complicates the selection of those most likely to benefit from existing or emergent treatments. Objective We aimed to predict the progression of bronchiectasis in preschoo…
View article: Sleep‐disordered breathing in Australian children with Prader‐Willi syndrome following initiation of growth hormone therapy
Sleep‐disordered breathing in Australian children with Prader‐Willi syndrome following initiation of growth hormone therapy Open
Aim In children with Prader‐Willi syndrome (PWS), growth hormone (GH) improves height and body composition; however, may be associated with worsening sleep‐disordered breathing (SDB). Some studies have reported less SDB after GH initiation…
View article: A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis
A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis Open
Background: The marked heterogeneity in CF disease complicates selection of those most likely to benefit from existing or emergent treatments. Objective: We aimed to predict progression of bronchiectasis in preschool children with CF. Meth…
View article: European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia
European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia Open
The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no "go…
View article: Structural determinants of long-term functional outcomes in young children with cystic fibrosis
Structural determinants of long-term functional outcomes in young children with cystic fibrosis Open
Background Accelerated lung function decline in individuals with cystic fibrosis (CF) starts in adolescence with respiratory complications being the most common cause of death in later life. Factors contributing to lung function decline ar…
View article: Requirements for improving health and well‐being of children with Prader‐Willi syndrome and their families
Requirements for improving health and well‐being of children with Prader‐Willi syndrome and their families Open
Prader‐Willi syndrome (PWS) is a rare genetic condition with multi‐system involvement. The literature was reviewed to describe neurodevelopment and the behavioural phenotype, endocrine and metabolic disorders and respiratory and sleep func…
View article: Changing Prevalence of Lower Airway Infections in Young Children with Cystic Fibrosis
Changing Prevalence of Lower Airway Infections in Young Children with Cystic Fibrosis Open
Rationale: Historical studies suggest that airway infection in cystic fibrosis initiates with Staphylococcus aureus and Haemophilus influenzae, with later emergence of Pseudomonas aeruginosa. Aspergillus species are regarded as relatively …