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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,…

Early detection of pulmonary exacerbations in mucociliary clearance diseases: Molecular fingerprinting by exhaled breath and microbiome analysis

Objectives: The primary objective is to establish whether expiratory VOC analysis by electronic nose and GC-MS can: Ia) discriminate between patients (CF, PCD) with and without an exacerbation and Ib) discriminate between different microbial species…

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

Validation of *in vitro counterpart* models to enable personalized care in CF

The specific objectives are:1. What is the difference of in vitro CFTR function in CF patients with either mild (compound heterozygote A455E) mutations or severe (homozygote F508del) mutations with mild or severe clinical disease? 2. What is the…

Biomarkers in acute and chronic bacterial endobronchial infections in Cystic Fibrosis
A Pilot study

Primary Objective: The purpose of this pilot study is to determine cut offs for both Procalcitonin and MR-proADM in stable CF patients. These measurements will be compared with Procalcitonin and MR-proADM values in CF patients with a pulmonary…

Myocardial function, oxygen consumption and efficiency assessment in the right ventricle of patients with idiophathic pulmonary hypertrophy using positron emission tomography.

The study investigates the role of the RV efficiency in the deterioration of RV function to failure in PH (measured with stroke volume index as the parameter of RV function); efficiency will be related to MVO2, alterations in substrate metabolism…

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.

Exercise training in patients with idiopathic Pulmonary Arterial Hypertension

1. Does exercise training improve muscle function and exercise tolerance in patients with IPAH?2. Does exercise training in IPAH result in morphological and biochemical changes of the quadriceps muscle?3. Does exercise training in IPAH result in…

Optimising screening for early disease detection in familial pulmonary fibrosis

Our primary objective is to determine the prognostic value of parameters for early disease detection of familial pulmonary fibrosis in the study population. All putative parameters for detection of preclinical disease have been shown to be…

Growing up after surgery for congenital diaphragmatic hernia (CDH): Problems for life? A study to evaluate optimal medical and psychosocial care in CDH up till adulthood (CDH-FU)

Primary Objective: To evaluate persisting pulmonary morbidity and echocardiographic signs of pulmonary hypertension in young adults born with CDH that interfere with physical fitness and participation in society.Secondary Objective(s): To evaluate…

Congenital Lung Abnormalities on MRI

Our main objective is to validate a MRI scan protocol for imaging parenchymal abnormalities in CPAM and describe the appearance of these abnormalities on MRI. Furthermore, we want to image postoperative changes in developing lungs of patients…

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 (…

Performance and patient acceptance of a commercially available beverage as compared to an oral glucose solution for oral glucose tolerance tests in cystic fibrosis (CF) patients who are screened for CF-related diabetes.

Primary Objective: To compare the performance of a glucose tolerance test (*AATT*) with a commercially available beverage to the results of the conventional OGTT with respect to diagnosing IGT and CFRD in patients with CF. Secondary Objective(s): To…

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…

Early identification of progressive pulmonary fibrosis
Precision Medicine for more Oxygen - ILD extension

1. Identify biomarkers and risk factors that associate with fibrosis progression or predict transformation into a rapid progressive (fibrosing) phenotype and acute exacerbations in IPF and FPF patients.2. Identify biomarkers and risk factors that…