Harry Heijerman
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View article: Evaluation of Elexacafor/Tezacaftor/Ivacaftor therapy after lung transplantation in Cystic Fibrosis: The Dutch National KOALA study
Evaluation of Elexacafor/Tezacaftor/Ivacaftor therapy after lung transplantation in Cystic Fibrosis: The Dutch National KOALA study Open
ETI for PwCF post-LTx shows favorable effects on CRS, GI symptoms, and quality of life, but not on BMI and HbA1c. Due to its high cost, careful consideration and further studies are required. Monitoring renal function and CNI trough levels…
View article: Considerations for the use of inhaled antibiotics for <i>Pseudomonas aeruginosa</i> in people with cystic fibrosis receiving CFTR modulator therapy
Considerations for the use of inhaled antibiotics for <i>Pseudomonas aeruginosa</i> in people with cystic fibrosis receiving CFTR modulator therapy Open
The major cause of mortality in people with cystic fibrosis (pwCF) is progressive lung disease characterised by acute and chronic infections, the accumulation of mucus, airway inflammation, structural damage and pulmonary exacerbations. Th…
View article: Development and validation of a novel personalized electronic patient-reported outcome measure to assess quality of life (Q-LIFE): a prospective observational study in people with Cystic Fibrosis
Development and validation of a novel personalized electronic patient-reported outcome measure to assess quality of life (Q-LIFE): a prospective observational study in people with Cystic Fibrosis Open
Dutch Cystic Fibrosis Foundation, Health-Holland.
View article: Treatment with inhaled antibiotics in bronchiectasis, side effects, and evaluation of the tolerance test; analysis from the BATTLE randomized controlled trial
Treatment with inhaled antibiotics in bronchiectasis, side effects, and evaluation of the tolerance test; analysis from the BATTLE randomized controlled trial Open
Introduction Tobramycin inhalation solution (TIS) is a treatment option for patients with frequent exacerbations of bronchiectasis. A possible side effect of TIS is the development of chronic cough and bronchospasm, whereby the guidelines …
View article: Radiological and long‐term clinical response to elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease
Radiological and long‐term clinical response to elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease Open
Introduction A triple combination of CFTR modulators ELE/TEZ/IVA (elexacaftor/tezacaftor/ivacaftor, Trikafta™) has been evaluated in clinical trials for people with cystic fibrosis (pwCF) and was approved to the European and US market. Dur…
View article: The effect of azithromycin on sputum inflammatory markers in bronchiectasis
The effect of azithromycin on sputum inflammatory markers in bronchiectasis Open
Background Long term macrolide treatment has been found beneficial in bronchiectasis (BE) -pathogical bronchial dilatation- possibly due to a combined anti-bacterial and immunomodulatory effect. The exact mechanism of inflammatory response…
View article: The effect of azithromycin on sputum inflammatory markers in bronchiectasis. Analysis from the BAT randomized controlled trial
The effect of azithromycin on sputum inflammatory markers in bronchiectasis. Analysis from the BAT randomized controlled trial Open
Background Long term macrolide treatment has been found beneficial in bronchiectasis (BE) -pathogical bronchial dilatation- possibly due to a combined anti-bacterial and immunomodulatory effect. The exact mechanism of inflammatory response…
View article: The prevalence of vascular and metabolic complications after lung transplant in people with cystic fibrosis in a Dutch cohort
The prevalence of vascular and metabolic complications after lung transplant in people with cystic fibrosis in a Dutch cohort Open
This study shows that the prevalence of cardiovascular risk factors increases after LTx for CF, potentially leading to major complications. These data emphasize the necessity of regular check-ups for metabolic and vascular complications af…
View article: Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands
Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands Open
The isolation prevalence of 3.6%, poor treatment outcomes and associated lung function decline emphasize that NTM pulmonary disease (NTM-PD) is a significant health issue among pwCF in the Netherlands. Its prevention and treatment require …
View article: Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia
Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia Open
Cystic fibrosis is caused by genetic defects that impair the CFTR channel in airway epithelial cells. These defects may be overcome by specific CFTR modulating drugs, for which the efficacy can be predicted in a personalized manner using 3…
View article: Effects of long-term Tobramycin Inhalation Solution (TIS) once daily on exacerbation rate in patients with non-cystic fibrosis bronchiectasis
Effects of long-term Tobramycin Inhalation Solution (TIS) once daily on exacerbation rate in patients with non-cystic fibrosis bronchiectasis Open
Background: Use of long-term tobramycin inhalation solution (TIS) has been shown beneficial in cystic fibrosis (CF) and earlier findings also suggest a benefit in non-CF bronchiectasis. We investigated the efficacy and safety of maintenanc…
View article: Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype
Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype Open
This reports describes a first example that intestinal organoids were used to identify a previously unknown CFTR mutation as a minimal function mutation. The individual FIS-based definition of minimal residual function, response to ele/tez…
View article: Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation
Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation Open
Objectives: [1] To investigate the cardiorespiratory fitness (CRF) levels in children and adolescents with cystic fibrosis (CF) with no ventilatory limitation (ventilatory reserve ⩾ 15%) during exercise, and [2] to assess which physiologic…
View article: Prediction of Real-World Long-Term Outcomes of People with CF Homozygous for the F508del Mutation Treated with CFTR Modulators
Prediction of Real-World Long-Term Outcomes of People with CF Homozygous for the F508del Mutation Treated with CFTR Modulators Open
The clinical response to cystic fibrosis transmembrane conductance regulator (CFTR) modulators is variable within people with cystic fibrosis (pwCF) homozygous for the F508del mutation. The prediction of clinical effect in individual patie…
View article: Management of Individual Patient Expectations When Starting with Highly Effective CFTR Modulators
Management of Individual Patient Expectations When Starting with Highly Effective CFTR Modulators Open
Highly effective CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ELE/TEZ/IVA will become available for an increasing number of people with cystic fibrosis (pwCF) in the near future. Before the start of this therapy, many question…
View article: Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia
Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia Open
Cystic Fibrosis (CF) is caused by genetic defects that impair the cystic fibrosis transmembrane conductance regulator (CFTR) channel in airway epithelial cells. These defects may be overcome by specific CFTR modulating drugs, for which the…
View article: The relation between psychological distress and medication adherence in lung transplant candidates and recipients: A cross‐sectional study
The relation between psychological distress and medication adherence in lung transplant candidates and recipients: A cross‐sectional study Open
Aims and objectives To explore the prevalence of psychological distress such as anxiety, depression and post‐traumatic stress disorder and its associations with medication adherence in lung transplant patients. Background Psychological dis…