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Development of individual therapy for cystic fibrosis using primary epithelial tissue cultures

The overall aim of the study is to improve diagnostic and therapeutic options for people with CF, especially in the context of young children and novel CFTR-targeting drugs. Primary Objective: 1. to demonstrate relations between CFTR genotype,…

Assessment of real life and trained inhalation of Tobramycin Inhalation Powder (TIP) and Tobramycin Inhalation Solution (TIS) TIPTIS study.

Primary objective of the study is to record TIP inhalations on video at home to study the association between inhalation manoeuver and cough. Secondary objectives are: To study the inhalation maneuvers of patients using TIP in the home situation; to…

central airways and lung volume assessment by Magnetic Resonance Imaging in children

Primary objective: to evaluate the image quality between the two protocols using Signal to Noise Ratio (SNR) measurements Secondary objectives: 1) test if measurements of lung volume and central airways dimensions using MRI are related to validated…

Cine-MRI to diagnose glossoptosis in Robin Sequence (RS)

Aim of total study:Diagnosing all components of RS in an objective mannerAim of present substudy:Increasing the reliability and decreasing the burden of the method with which glossoptosis can be determined in newborns with RS

Characterisation of regulatory T cells and cytokine production in children diagnosed with asthma

To characterise the number, phenotype and functionality of regulatory T cells and cytokines produced in the peripheral blood of children diagnosed with asthma.

Mucoviscidosis and Inflammation, Variability through Exercise and Training: physical exercise program

Primary objective:What is the effect of a 12 weeks physical exercise program on the exercise induced inflammatory response in patients with cystic fibrosis?Secundary objectives:- What are the differences in pre-training basal immune values of…

Sinonasal pathology in children with Cystic Fibrosis

Investigation of the prevalence of sinonasal disease on CT-sinus in children with Cystic Fibrosis at different ages. This study will focus especially on the onset of sinonasal pathology in Cystic Fibrosis in relation to the development of the…

The half-lives of neutrophils in the blood and lungs of patients with cystic fibrosis

Objective of the study:The results of this study will lead to an improved insight in the lifecycle of these cells in health and diseases, and our results will be of importance for more insight in chronic inflammatory diseases particularly CF. For…

Longitudinal microcirculatory changes in newborns with Congenital Diaphragmatic Hernia; an observational study

To evaluate the predictive value of microcirculatory perfusion for the incidence of Extracorporeal Membrane Oxygenation (ECMO) dependency and consequently survival. To evaluate the effects of vasopressor drugs and iNO on microcirculatory perfusion.

Early detection of bacterial airway infections in children with primary ciliary dyskinesia and cystic fibrosis by electronic nose

We hypothesize that expiratory VOC-analysis by electronic nose is capable of: a.) discriminating CF-/PCD-patients with and without pulmonary infections and b.) discriminating CF-/PCD-patients and healthy controls.

HERNIA-study:
congenital diaphragmatic Hernia, Environment, Retinoids, Nutrition, Inheritance, other Associations

- To analyze retinoid levels in plasma and amniotic fluid from infants and mothers to establish the potential link between retinoids and CDH. - To establish a registry of blood and tissue samples that will be valuable for future studies of the…

Mucoviscidosis and Inflammation, Variability through Exercise and Training: Maximal exercise test

Primary objective: *What is the effect of an incremental maximal exercise test on inflammation in patients with cystic fibrosis and in healthy people?*Secundary objectives:- What are the differences in basal immune values between HP and CF patients…

Long-term lung function, exercise endurance, participation and quality of life in congenital diaphragmatic hernia

The aim of the present study is to evaluate the long-term pulmonary sequelae, exercise endurance and perception of exercise endurance in adult CDH patients in a cohort that has been studied earlier at a median age of 11.7 years. Lung function…

Center for Translational Pediatric Pulmonology
*Modelling the bronchial epithelium in children*

The main objective of our Center for Translational Pediatric Pulmonology (CTPP) is to establish models of bronchial epithelial function in children, by (1) epithelial organoid cultures derived from bronchial epithelial cells; (2) bronchial brushings…

Validation of bi-dimensional (2D) and three-dimensional (3D) Fourier Decomposition (FD) as Magnetic Resonance Imaging (MRI) technique to assess lung ventilation and perfusion compared to computed tomography (CT), Hyperpolarised gases (HP-MRI) and Contrast-Enhanced MRI (CE-MRI)

The final goal of this validation plan is to develop an MRI platform that can provide information about ventilation, inflammation, perfusion and structure (VIPS-MRI) in a single MRI examination of 30 minutes for safe and efficient monitoring of CFLD…

The Origins of Pediatric Respiratory diseases: laying groundwork for genetic, cellular and immunological analyses. (OPeRA)

The main objective of this proposal is to establish ex-vivo models of bronchial epithelial cells from children. Use of (1) epithelial organoid cultures derived from bronchial or nasal epithelial cells; and of (2) bronchial brushings; (3) nasal…

TAAI Erasmus Research Initiative to Fight CF: Monitoring Inflammation in CF Lung disease into a new Era

The overall aim of the study is to develop innovative minimally invasive monitoring techniques that can identify lung inflammation in pwCF when using highly effective modulators, compared to patients whom are not eligible for CFTR modulators (…

Identification of new genetic defects in telomeropathies

To identify new genetic defects in telomeropathies.