Many people with cystic fibrosis who do not have genetic variants approved for treatment still respond to treatment with elexacaftor–tezacaftor–ivacaftor.
Children with cystic fibrosis who receive therapy requiring general anesthesia before the age of 2 years do not have impaired neurodevelopment in later childhood.
The growing genomic knowledge about primary ciliary dyskinesia will aid diagnosis, understanding of prognosis, and the establishment of future therapeutic trials.
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An AI support tool could aid clinicians using spirometry to accurately predict respiratory diagnoses, particularly chronic obstructive pulmonary disease.
Pediatric Infectious Diseases (I Brook and Y Shachor, Section Editors)
Children and adolescents living with cystic fibrosis (CF) are at risk for numerous types of pulmonary infections. Early in life, the most common pathogens are bacteria such as Staphylococcus aureus , Pseudomonas aeruginosa , and Haemophilus …
Analysis of the liver using imaging for persons with cystic fibrosis (CF) continues to evolve as new medical therapies are developed improving and extending life. In the 2010s, therapies targeted at modulating protein folding became available to …
People with cystic fibrosis (CF) are at risk for dysglycaemia caused by progressive beta cell dysfunction and destruction due to pancreatic exocrine disease and fibrosis. CF-related diabetes (CFRD) is a unique form of diabetes that has distinctive …
Cystic fibrosis (CF) is a complex, multisystem, life-limiting autosomal recessive disorder (Tümmler 2020 ; Bell et al. 2020 ). It is estimated that 162 428 individuals are living with CF worldwide (Guo et al. 2022 ). Birth prevalence varies between …
Lung transplantation (LTx) is a well-established, life-saving intervention in selected patients suffering from advanced lung disease such as emphysema, fibrotic lung diseases, cystic fibrosis (CF), and pulmonary hypertension. To date, however …
A common comorbidity in cystic fibrosis (CF) is CF-related diabetes (CFRD) [ 1 ], which aggravates abnormalities in nutritional status and lung capacity [ 2 ]. Insufficient insulin secretion seems to cause the progression to CFRD [ 3 , 4 ] but …
Cystic fibrosis (CF) is a complex, multisystem, life-limiting autosomal recessive disorder (Tümmler 2020 ; Bell et al. 2020 ). It is estimated that 162 428 individuals are living with CF worldwide (Guo et al. 2022 ). Birth prevalence varies between …
Cystic Fibrosis (CF) is a progressive condition resulting in reduced lung function and strongly associated with elevated anxiety and depression symptoms. Self-concept refers to an individual’s overarching sense of identity, a positive level of …
