Executive summary The past six decades have seen remarkable improvements in health
outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young …

Pancreatic insufficiency (PI) affects about 85% of the cystic fibrosis population. Although
most are PI soon after birth, some will have pancreatic sufficiency (PS) for some or all of their …

Establishing the diagnosis of cystic fibrosis (CF) is straight forward in the majority of patients:
they present with a clear clinical picture (most frequently chronic respiratory symptoms plus …

Rationale: Neutrophils are recruited to the airways of individuals with cystic fibrosis (CF). In
adolescents and adults with CF, airway neutrophils actively exocytose the primary granule …

Background Newborn screening (NBS) algorithms for cystic fibrosis (CF) vary in the United
State of America and include different cystic fibrosis transmembrane conductance regulator …

Beginning in the 1960s, mandatory newborn screening (NBS) of essentially all infants has
been a major public health success story. NBS is not just a blood test, rather, it is a complex …

While typically considered a pulmonary disease, cystic fibrosis patients develop significant
nutritional complications and comorbidities, especially those who are pancreatic insufficient …

Background Nutritional status is paramount in Cystic Fibrosis (CF) and is directly correlated
with morbidity and mortality. The first ESPEN-ESPGHAN-ECFS guidelines on nutrition care …

Objective To characterize the phenotypic expression of children with conductance regulator‐
related metabolic syndrome (CRMS)/cystic fibrosis screen positive inconclusive diagnosis …

This is the second in a series of four papers updating the European Cystic Fibrosis Society
(ECFS) standards for the care of people with CF. This paper focuses on establishing and …