The Frequency and Potential Implications of HFE Genetic Variants in Children With Cystic Fibrosis.

Background: Genetic modifiers have been identified that increase the risks of lung disease and other complications, such as diabetes in people with cystic fibrosis (CF). Variants in the hemochromatosis gene (HFE) were reported in a study of adults to be associated with worse lung disease.

Objectives: To ascertain the frequency of HFE variants, particularly C282Y (c.

Clinical outcomes at 9-10 years of age in children born with cystic fibrosis transmembrane conductance regulator related metabolic syndrome.

Background And Objectives: There are limited data on cystic fibrosis (CF) transmembrane conductance regulator-related metabolic syndrome (CRMS) outcomes beyond infancy. The goal of this study was to analyze outcomes of infants with CRMS up to the age of 9-10 years using the CF Foundation Patient Registry (CFFPR).

Methods: We analyzed data from the CFFPR for individuals with CF and CRMS born between 2010 and 2020.

Cystic fibrosis year in review 2020: Section 2 pulmonary disease, infections, and inflammation.

The outlook for those with cystic fibrosis (CF) has never been brighter with ever increasing life expectancy and the approval of the highly effective CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor. With that being said, the progressive pulmonary decline and importance of lung health, infection, and inflammation in CF remains. This review is the second part in a three-part CF Year in Review 2020.

Lactation Consultation Sustains Breast Milk Intake in Infants With Cystic Fibrosis.

Guidelines recommend breastfeeding in cystic fibrosis (CF) but breastfeeding rates decline rapidly in CF. We initiated a quality improvement project to improve breastfeeding in CF by incorporating International Board-Certified Lactation Consultants (IBCLC) into the initial CF-diagnosis visit to support mothers who were breastfeeding at diagnosis. In the preintervention group, only 8/14 (57%) continued to provide breast milk after the first visit, whereas postintervention, 16/17 (94%) mothers continued to do so (P = 0.

Improving inpatient cystic fibrosis pulmonary exacerbation care: two success stories.

Background: Pulmonary exacerbations (PEx) in cystic fibrosis (CF) are a frequent cause of hospitalisations and lead to long-term decline in pulmonary function. Successful CF inpatient care requires the coordination of multiple providers and complex therapies. Children's Hospital of Wisconsin (CHW) and Children's Healthcare of Atlanta (CHoA) independently identified PEx inpatient care for focused improvements, with emphasis on improving care coordination and patient outcomes.