Cystic fibrosis is the most common life-threatening recessively inherited disease in Caucasians leading to Cystic fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction. CFTR is involved in the active excretion of chloride at the apical membrane of epithelial cells and its dysfunction leads to pulmonary and digestive disorders. The survival rate has improved over time, especially due to early provision of care in specialized reference centers and the implementation of newborn bloodspot screening (NBS) which has been common practice in several countries for many years.
The ability to accurately monitor disease progression and the response to therapy has become essential. The main aim is to avoid the lung function decline leading to death.
Therefore, to enable the early detection of pulmonary exacerbation, tele-monitoring with spirometric connected devices has been developed. For children with normal lung function, the lung clearance index, a biomarker of ventilation inhomogeneity is becoming a standard procedure to explore the impact of therapies.
In this Research Topic we aim to explore and provide updates on:
- The impact of long-term neonatal screening
- Describe new understanding regarding the lung-gut axis with respect to its microbiome
- Implement disease monitoring, via connected devices at home and to explore their utility
- Explore the advances in lung function exploration
- Develop new imaging patterns for structural damages follow-up
- Explore the advances in nutrition and the CF related diabetes (CFRD)
- Follow the various aspects and the impact of new therapies
We welcome manuscripts on new supports in cystic fibrosis (CF), which can include CF neonatal screening, CF nutrition, CF related diabetes, CF lung function exploration and evolution, bacteriological aspects and microbiome, advance in CF imaging, and the various aspects and the impact of new therapies
The types of manuscript could be Original Research, Study Protocols, Case Reports, Methods and Review type articles.
Cystic fibrosis is the most common life-threatening recessively inherited disease in Caucasians leading to Cystic fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction. CFTR is involved in the active excretion of chloride at the apical membrane of epithelial cells and its dysfunction leads to pulmonary and digestive disorders. The survival rate has improved over time, especially due to early provision of care in specialized reference centers and the implementation of newborn bloodspot screening (NBS) which has been common practice in several countries for many years.
The ability to accurately monitor disease progression and the response to therapy has become essential. The main aim is to avoid the lung function decline leading to death.
Therefore, to enable the early detection of pulmonary exacerbation, tele-monitoring with spirometric connected devices has been developed. For children with normal lung function, the lung clearance index, a biomarker of ventilation inhomogeneity is becoming a standard procedure to explore the impact of therapies.
In this Research Topic we aim to explore and provide updates on:
- The impact of long-term neonatal screening
- Describe new understanding regarding the lung-gut axis with respect to its microbiome
- Implement disease monitoring, via connected devices at home and to explore their utility
- Explore the advances in lung function exploration
- Develop new imaging patterns for structural damages follow-up
- Explore the advances in nutrition and the CF related diabetes (CFRD)
- Follow the various aspects and the impact of new therapies
We welcome manuscripts on new supports in cystic fibrosis (CF), which can include CF neonatal screening, CF nutrition, CF related diabetes, CF lung function exploration and evolution, bacteriological aspects and microbiome, advance in CF imaging, and the various aspects and the impact of new therapies
The types of manuscript could be Original Research, Study Protocols, Case Reports, Methods and Review type articles.