The Long-Term Uptake of Home Spirometry in Regular Cystic Fibrosis Care: Retrospective Multicenter Observational Study.

Background: Home spirometers have been widely implemented in the treatment of people with cystic fibrosis (CF). Frequent spirometry measurements at home could lead to earlier detection of exacerbations. However, previous research indicates that the long-term use of home spirometry is not well maintained by people with CF.

Remote monitoring and teleconsultations can reduce greenhouse gas emissions while maintaining quality of care in cystic fibrosis.

Background: Remote care usefulness and climate change co-benefits should be addressed simultaneously to incentivize political action.

Objectives: To assess the changes in healthcare consumption, lung function and greenhouse gas (GHG) emissions during the COVID-19 pandemic in Dutch cystic fibrosis (CF) care.

Design: Retrospective multicentre observational study in five Dutch CF centres.

Potential, Pitfalls, and Future Directions for Remote Monitoring of Chronic Respiratory Diseases: Multicenter Mixed Methods Study in Routine Cystic Fibrosis Care.

Background: The current literature inadequately addresses the extent to which remote monitoring should be integrated into care models for chronic respiratory diseases (CRDs).

Objective: This study examined a remote monitoring program (RMP) in cystic fibrosis (CF) by exploring experiences, future perspectives, and use behavior over 3 years, with the aim of developing future directions for remote monitoring in CRDs.

Methods: This was a mixed methods, multicenter, observational study in 5 Dutch CF centers following a sequential explanatory design.

Pulmonary function in patients with adolescent idiopathic scoliosis: an explorative study of a wearable smart shirt as a measurement instrument.

Purpose: Adolescent idiopathic scoliosis (AIS) presents various challenges, including respiratory symptoms that impact pulmonary function. This study aims to explore the feasibility of using a smart shirt for continuous monitoring of lung volumes and heart rate during routine activities in AIS patients.

Methods: A single-center exploratory feasibility study was conducted with AIS patients aged 16-22 years with a thoracic curvature of ≥ 30 degrees and absence of respiratory comorbidities.

Evaluation of the drug-drug interaction between triazole antifungals and cystic fibrosis transmembrane conductance regulator modulators in a real-life cohort.

Limited data on the clinical management of drug-drug interactions between triazoles and Cystic Fibrosis transmembrane conductance regulator (CFTR) modulators are available. We retrospectively evaluated azole target attainment and dose adaptations in patients from two Dutch CF centres concomitantly receiving triazoles and CFTR modulators. In total, 21 patients with 59 triazole trough concentrations were evaluated.

FDA-approved drug screening in patient-derived organoids demonstrates potential of drug repurposing for rare cystic fibrosis genotypes.

Background: Preclinical cell-based assays that recapitulate human disease play an important role in drug repurposing. We previously developed a functional forskolin induced swelling (FIS) assay using patient-derived intestinal organoids (PDIOs), allowing functional characterization of CFTR, the gene mutated in people with cystic fibrosis (pwCF). CFTR function-increasing pharmacotherapies have revolutionized treatment for approximately 85% of people with CF who carry the most prevalent F508del-CFTR mutation, but a large unmet need remains to identify new treatments for all pwCF.

Home monitoring in asthma: towards digital twins.

Purpose Of Review: We highlight the recent advances in home monitoring of patients with asthma, and show that these advances converge towards the implementation of digital twin systems.

Recent Findings: Connected devices for asthma are increasingly numerous, reliable and effective: new electronic monitoring devices extend to nebulizers and spacers, are able to assess the quality of the inhalation technique, and to identify asthma attack triggers when they include a geolocation function; environmental data can be acquired from databases and refined by wearable air quality sensors; smartwatches are better validated. Connected devices are increasingly integrated into global monitoring systems.

Forskolin-induced organoid swelling is associated with long-term cystic fibrosis disease progression.

Rationale: Cystic fibrosis (CF) is a monogenic life-shortening disease associated with highly variable individual disease progression which is difficult to predict. Here we assessed the association of forskolin-induced swelling (FIS) of patient-derived organoids with long-term CF disease progression in multiple organs and compared FIS with the golden standard biomarker sweat chloride concentration (SCC).

Methods: We retrieved 9-year longitudinal clinical data from the Dutch CF Registry of 173 people with mutations in the cystic fibrosis transmembrane conductance regulator () gene.

Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation.

Background: The natural food supplements curcumin and genistein, and the drug ivacaftor were found effective as CFTR potentiators in the organoids of individuals carrying a S1251N gating mutation, possibly in a synergistic fashion. Based on these in vitro findings, we evaluated the clinical efficacy of a treatment with curcumin, genistein and ivacaftor, in different combinations.

Methods: In three multi-center trials people with CF carrying the S1251N mutation were treated for 8 weeks with curcumin+genistein, ivacaftor and ivacaftor+genistein.

Rectal Organoids Enable Personalized Treatment of Cystic Fibrosis.

In vitro drug tests using patient-derived stem cell cultures offer opportunities to individually select efficacious treatments. Here, we provide a study that demonstrates that in vitro drug responses in rectal organoids from individual patients with cystic fibrosis (CF) correlate with changes in two in vivo therapeutic endpoints. We measured individual in vitro efficaciousness using a functional assay in rectum-derived organoids based on forskolin-induced swelling and studied the correlation with in vivo effects.

Barriers and Facilitators When Implementing Web-Based Disease Monitoring and Management as a Substitution for Regular Outpatient Care in Pediatric Asthma: Qualitative Survey Study.

Background: Despite their potential benefits, many electronic health (eHealth) innovations evaluated in major studies fail to integrate into organizational routines, and the implementation of these innovations remains problematic.

Objective: The purpose of this study was to describe health care professionals' self-identified perceived barriers and facilitators for the implementation of a Web-based portal to monitor asthmatic children as a substitution for routine outpatient care. Also, we assessed patients' (or their parents) satisfaction with this eHealth innovation.

Depression and anxiety during pregnancy: The influence of maternal characteristics.

Background: Depression and anxiety during pregnancy are associated with adverse health outcomes for both mother and child. This study aims to investigate the occurrence of symptoms of depression and anxiety in early and late pregnancy, the longitudinal changes from early to late pregnancy, and factors associated with symptoms of depression and anxiety in pregnant women in the Netherlands.

Methods: We studied 2897 women participating in the PRegnancy and Infant DEvelopment (PRIDE) Study.

A virtual asthma clinic for children: fewer routine outpatient visits, same asthma control.

eHealth is an appealing medium to improve healthcare and its value (in addition to standard care) has been assessed in previous studies. We aimed to assess whether an eHealth intervention could improve asthma control while reducing 50% of routine outpatient visits.In a multicentre, randomised controlled trial with a 16-month follow-up, asthmatic children (6-16 years) treated in eight Dutch hospitals were randomised to usual care (4-monthly outpatient visits) and online care using a virtual asthma clinic (VAC) (8-monthly outpatient visits with monthly web-based monitoring).

Hip Fractures in Elderly People: Surgery or No Surgery? A Systematic Review and Meta-Analysis.

Introduction: Increasing numbers of patients with hip fractures also have advanced comorbidities. A majority are treated surgically. However, a significantly increasing percentage of medically unfit patients with unacceptably high risk of perioperative death are treated nonoperatively.

Trajectories in quality of life of patients with a fracture of the distal radius or ankle using latent class analysis.

Purpose: This prospective study aimed to identify the different trajectories of quality of life (QOL) in patients with distal radius fractures (DRF) and ankle fractures (AF). Secondly, it was examined if subgroups could be characterized by sociodemographic, clinical, and psychological variables.

Methods: Patients (n = 543) completed the World Health Organization Quality of Life assessment instrument-Bref (WHOQOL-Bref), the pain, coping, and cognitions questionnaire, NEO-five factor inventory (neuroticism and extraversion), and the state-trait anxiety inventory (short version) a few days after fracture (i.

Factors related to long-term surgical morbidity in congenital diaphragmatic hernia survivors.

Background: Patients born with a congenital diaphragmatic hernia (CDH) have a high mortality and morbidity. After discharge, complications and long-term morbidity are still encountered. This study describes the factors related to the surgical long-term outcomes in CDH survivors.

Effects of Survivorship Care Plans on patient reported outcomes in ovarian cancer during 2-year follow-up - The ROGY care trial.

Objective: The aim of this study was to assess the long-term impact of an automatically generated Survivorship Care Plan (SCP) on patient reported outcomes in ovarian cancer in routine clinical practice. Outcome measures included satisfaction with information provision and care, illness perceptions and health care utilization.

Methods: In this pragmatic cluster randomized trial, twelve hospitals in the South of the Netherlands were randomized to 'SCP care' or 'usual care'.

Care-related Quality of Life of informal caregivers of the elderly after a hip fracture.

Background: Reforms in the Dutch healthcare system in combination with the aging of the population will lead to a strong increase in the demand for informal care in the Netherlands. A hip fracture is one of the most important causes of hospital admissions among frail elderly and informal caregivers experience stress that may have significantly negative impact on the caregivers' Quality of Life. The purpose of the study was to determine the nature, intensity and the care-related Quality of Life (CarerQoL) of informal caregivers of elderly patients in the first six months after a hip fracture.

Evaluation of monitoring strategies for childhood asthma.

The goal of monitoring pediatric asthma is to obtain and maintain asthma control, which is defined as minimizing asthma symptoms, restrictions to daily activities and the use of rescue medication. Long term goals include reducing the risk of fixed airflow limitation, and preventing asthma exacerbations and side effects of treatment. Several monitoring tools are available but no consensus exists on how to monitor patients in the most optimal way.