Marijke Proesmans
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View article: Fibrosing interstitial lung disease in childhood: prevalence and disease trajectories
Fibrosing interstitial lung disease in childhood: prevalence and disease trajectories Open
Background Pulmonary fibrosis is of critical importance in childhood interstitial lung disease (chILD), yet fibrosis prevalence, impact on the clinical progression, and survival have not been systematically evaluated. Methods Data were ext…
View article: The Promising Role of Intestinal Organoids in the Diagnostic Work-Up of Cystic Fibrosis Screen Positive Inconclusive Diagnosis/CFTR-Related Metabolic Syndrome (CFSPID/CRMS)
The Promising Role of Intestinal Organoids in the Diagnostic Work-Up of Cystic Fibrosis Screen Positive Inconclusive Diagnosis/CFTR-Related Metabolic Syndrome (CFSPID/CRMS) Open
Cystic Fibrosis Screen Positive Inconclusive Diagnosis/CFTR-related Metabolic Syndrome (CFSPID/CRMS) presents a significant clinical challenge due to its variable diagnostic outcomes and uncertain disease progression. Current diagnostic st…
View article: Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy
Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy Open
Diagnosis of PTI/NEHI relies on clinical symptoms and HRCT imaging results, with lung biopsies less commonly performed. Differences exist among countries regarding the number and type of investigations. A need exists for guidelines that wi…
View article: Leukocyte telomere length and attrition in association with disease severity in cystic fibrosis patients
Leukocyte telomere length and attrition in association with disease severity in cystic fibrosis patients Open
Cystic fibrosis (CF) is characterized by chronic airway inflammation and premature aging. The link with leukocyte telomere length (LTL) as a marker of biological aging is unclear. We studied disease severity and LTL in 168 CF patients of w…
View article: Systemic Adverse Events Associated with Locally Administered Corticosteroids
Systemic Adverse Events Associated with Locally Administered Corticosteroids Open
Topical corticosteroids are a mainstay in the treatment of many pediatric disorders. While they have proven beneficial therapeutic effects and are generally considered safe, systemic adverse events may occur. This study presents four cases…
View article: Single‐cell <scp>RNA</scp> sequencing of cystic fibrosis liver disease explants reveals endothelial complement activation
Single‐cell <span>RNA</span> sequencing of cystic fibrosis liver disease explants reveals endothelial complement activation Open
Background & Aims Cystic fibrosis (CF) is considered a multisystemic disorder in which CF‐associated liver disease (CFLD) is the third most common cause of mortality. Currently, no effective treatment is available for CFLD because its path…
View article: Optimizing care for children with difficult-to-treat and severe asthma through specialist paediatric asthma centres: expert practical experience and advice
Optimizing care for children with difficult-to-treat and severe asthma through specialist paediatric asthma centres: expert practical experience and advice Open
View article: Respiratory morbidity in patients with spinal muscular atrophy—a changing world in the light of disease-modifying therapies
Respiratory morbidity in patients with spinal muscular atrophy—a changing world in the light of disease-modifying therapies Open
Respiratory complications are common in spinal muscular atrophy (SMA) and significantly contribute to morbidity and mortality in these patients. Generalized respiratory and bulbar muscle weakness translates into diverse and complex clinica…
View article: Baseline predictors of hospital length of stay in children with respiratory syncytial virus infection: A retrospective analysis of three prospective observational studies
Baseline predictors of hospital length of stay in children with respiratory syncytial virus infection: A retrospective analysis of three prospective observational studies Open
Background: Respiratory syncytial virus (RSV) is a common cause of hospitalisation in infants and children. This study aimed to identify common patient characteristics and baseline predictors of hospital length of stay (LOS) among infants …
View article: A core outcome set for bronchiectasis in children and adolescents for use in clinical research: an international consensus study
A core outcome set for bronchiectasis in children and adolescents for use in clinical research: an international consensus study Open
View article: ERS International Congress 2023: highlights from the Paediatrics Assembly
ERS International Congress 2023: highlights from the Paediatrics Assembly Open
Respiratory health in children is essential for general wellbeing and healthy development in the short and long term. It is well known that many respiratory diseases in adulthood have their origins in early life, and therefore research on …
View article: Cystic Fibrosis Cases Missed by Newborn Bloodspot Screening—Towards a Consistent Definition and Data Acquisition
Cystic Fibrosis Cases Missed by Newborn Bloodspot Screening—Towards a Consistent Definition and Data Acquisition Open
Repeated European surveys of newborn bloodspot screening (NBS) have shown varied strategies for collecting missed cases, and information on data collection differs among countries/regions, hampering data comparison. The ECFS Neonatal Scree…
View article: WS09.02 Theratyping molecular defects of CFTR rare variants in patient derived rectal organoids
WS09.02 Theratyping molecular defects of CFTR rare variants in patient derived rectal organoids Open
View article: WS04.06 Validation of rectal organoid morphology analysis (ROMA) as a novel physiological CFTR assay for diagnosis of cystic fibrosis
WS04.06 Validation of rectal organoid morphology analysis (ROMA) as a novel physiological CFTR assay for diagnosis of cystic fibrosis Open
View article: P411 Thinking out of the box: twinning in the era of the COVID-19 pandemic
P411 Thinking out of the box: twinning in the era of the COVID-19 pandemic Open
View article: P071 Evaluation of CFTR modulator efficacy by rectal organoid morphology analysis (ROMA) indexes
P071 Evaluation of CFTR modulator efficacy by rectal organoid morphology analysis (ROMA) indexes Open
View article: ERS International Congress 2022: highlights from the Paediatrics Assembly
ERS International Congress 2022: highlights from the Paediatrics Assembly Open
This review has been prepared by the Early Career Members and Chairs of the European Respiratory Society (ERS) Assembly 7: Paediatrics. We here summarise the highlights of the advances in paediatric respiratory research presented at the ER…
View article: Diffuse alveolar haemorrhage in children: an international multicentre study
Diffuse alveolar haemorrhage in children: an international multicentre study Open
Background Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. Methods A retrospective, descriptive multicentre follow-up study initiated f…
View article: ABCA3-related interstitial lung disease beyond infancy
ABCA3-related interstitial lung disease beyond infancy Open
Background The majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first year of lif…
View article: The burden and surveillance of RSV disease in young children in Belgium—expert opinion
The burden and surveillance of RSV disease in young children in Belgium—expert opinion Open
View article: European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance
European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance Open
View article: Congenital cystic adenomatoid malformations of the lung: a retrospective study of diagnosis, treatment strategy and postoperative morbidity in surgically treated patients
Congenital cystic adenomatoid malformations of the lung: a retrospective study of diagnosis, treatment strategy and postoperative morbidity in surgically treated patients Open
OBJECTIVES The purpose of this study was to evaluate the diagnosis of, clinical signs of and strategy for congenital cystic adenomatoid malformations (CCAM). METHODS In this retrospective study, patients who had thoracic surgery for CCAM l…
View article: Quality standards for managing children and adolescents with bronchiectasis: an international consensus
Quality standards for managing children and adolescents with bronchiectasis: an international consensus Open
The global burden of bronchiectasis in children and adolescents is being recognised increasingly. However, marked inequity exists between, and within, settings and countries for resources and standards of care afforded to children and adol…
View article: The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey
The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey Open
Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome …
View article: European Respiratory Society statement for defining respiratory exacerbations in children and adolescents with bronchiectasis for clinical trials
European Respiratory Society statement for defining respiratory exacerbations in children and adolescents with bronchiectasis for clinical trials Open
Bronchiectasis is being diagnosed increasingly in children and adolescents. Recurrent respiratory exacerbations are common in children and adolescents with this chronic pulmonary disorder. Respiratory exacerbations are associated with an i…
View article: Risk factors for disease severity and increased medical resource utilization in respiratory syncytial virus (+) hospitalized children: A descriptive study conducted in four Belgian hospitals
Risk factors for disease severity and increased medical resource utilization in respiratory syncytial virus (+) hospitalized children: A descriptive study conducted in four Belgian hospitals Open
Background We aimed to provide regional data on clinical symptoms, medical resource utilization (MRU), and risk factors for increased MRU in hospitalized respiratory syncytial virus (RSV)-infected Belgian pediatric population. Methods This…
View article: International consensus statement on quality standards for managing children/adolescents with bronchiectasis from the ERS CRC Child-BEAR-Net
International consensus statement on quality standards for managing children/adolescents with bronchiectasis from the ERS CRC Child-BEAR-Net Open
Bronchiectasis represents a clinical syndrome of recurrent or persistent wet/productive cough with abnormal bronchial dilatation in chest computed tomography (CT) images [1, 2]. Previously considered rare [1, 3], it is globally more common…
View article: The Short Term Influence of Chest Physiotherapy on Lung Function Parameters in Children With Cystic Fibrosis and Primary Ciliary Dyskinesia
The Short Term Influence of Chest Physiotherapy on Lung Function Parameters in Children With Cystic Fibrosis and Primary Ciliary Dyskinesia Open
Airway clearance therapy (ACT) is one of the cornerstone treatment modalities to improve mucociliary clearance for patients with bronchiectasis. The progression of lung disease in patients with bronchiectasis can be evaluated by spirometry…
View article: Cystic fibrosis and alpha-1 antitrypsin deficiency: case report and review of literature
Cystic fibrosis and alpha-1 antitrypsin deficiency: case report and review of literature Open
View article: Acute exacerbations in children’s interstitial lung disease
Acute exacerbations in children’s interstitial lung disease Open
Introduction Acute exacerbations (AEs) increase morbidity and mortality of patients with chronic pulmonary diseases. Little is known about the characteristics and impact of AEs on children's interstitial lung disease (chILD). Methods The K…