56 results
Primary objective is to objectively investigate the therapeutic potential of Ivacaftor in Dutch CF patients carrying a mutation associated with residual CFTR function. A secondary objective is to evaluate the correlations between individual…
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,…
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…
To evaluate the long-term safety, tolerability and efficacy of ArikaceTM 590 mg for a maximum 12 cycles administered once daily, where each cycle consists of 28 days on treatment followed by 28 days off treatment.
Primary objective: To show that the variability of whole blood total and unbound plasma tacrolimus concentrations during the first 6 days post transplantation is larger than the variation of tacrolimus concentrations in stable clinical situation.…
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…
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…
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
Primary objective:To investigate the clinical response to treatment with curcumin and genistein in CF-patients with a class III S1251N mutation.. Secondary objectives:1.To evaluate the correlations between individual curcumin+genistein induced CFTR…
We aim to assess gene expression patterns in cilia-producing cells from healthy controls to develop a candidate gene list for PCD.
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…
Investigate the prevalence of rhinosinusitis and/or nasal polyps in adult patients with Cystic Fibrosis. Also several aspects of sinonasal disease are investigated; quality of life, correlation between phenotype and genotype, microbiology, anatomy…
The primary objective of this Phase 3 extension study will be to obtain essential long term safety data to augment the overall safety database. The secondary objective will be to augment the efficacy information collected in the double-blind study (…
To characterise the number, phenotype and functionality of regulatory T cells and cytokines produced in the peripheral blood of children diagnosed with asthma.
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…
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…
Objectives: The aim of this study is to:I Identify pathogenic mutations in novel genes, causing PCDII Develop a diagnostic PCD test, based on MPSIII Validate MPS technique for PCD.
Primary objective: -to determine if there is a significant difference in the incidence of oxygen dependency at day 28 and/or death within the first 28 days of life between newborns with congenital diaphragmatic hernia treated with high frequency…
To investigate the clinical pharmacokinetics of tobramycin and/or colistin after nasal administration. With this pharmacokinetic parameters the safety of this treatment can be investigated.
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…