Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by variants in the gene encoding the cystic fibrosis transmembrane conduction regulator (CFTR) protein. Loss of CFTR function disrupts chloride, bicarbonate and regulation of sodium transport, producing a…
November 2018
The Remaining Barriers to Normalcy in Cystic Fibrosis IV
Induced pluripotent stem cells for treating cystic fibrosis: State of the science
Induced pluripotent stem cells (iPSCs) are a recently developed technology in which fully differentiated cells such as fibroblasts from individual CF patients can be repaired with [wildtype] CFTR, and reprogrammed to differentiate into fully differentiated cells characteristic of…
Inflammation in cystic fibrosis: An update
Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease progression making it an active area of research and important therapeutic target. In this review, we explore the most recent research on the major contributors…
Reevaluating approaches to cystic fibrosis pulmonary exacerbations
Cystic Fibrosis (CF) lung disease is characterized by intermittent acute episodes of worsening signs, symptoms, and pulmonary function; these so‐called pulmonary exacerbations (PEx) appear to be important drivers of long‐term declines in lung function, quality of life, and…
Biology and management of methicillin resistant Staphylococcus aureus in cystic fibrosis
Staphylococcus aureus is one of the earliest bacteria isolated from the respiratory tract in people with cystic fibrosis (CF). Its methicillin resistant form, MRSA, has gained attention due to the rapid increase in the last decades and worse outcomes…
The myriad challenges of respiratory fungal infection in cystic fibrosis
Fungal infection in cystic fibrosis (CF) is a recognized challenge, with many areas requiring further investigation. Consensus definitions exist for allergic bronchopulmonary aspergillus in CF, but the full scope of clinically relevant non‐allergic fungal disease in CF—asymptomatic colonization,…
Overcoming psychosocial challenges in cystic fibrosis: Promoting resilience
Individuals living with cystic fibrosis (CF), and their families, have experienced significant improvements in treatment and related research that have enhanced outcomes and survival. Despite such advancement, the burden of living with CF still exists. Many psychosocial stressors…