Outcome in patients with cystic fibrosis liver disease.

Background: Liver disease is an important complication in CF.

Aims: To determine if CFLD is a risk factor for mortality in CF, and which baseline characteristics predict all-cause mortality.

Methods: Irish children with CFLD, and their age and gender matched controls were enrolled at baseline and reviewed after 10years to determine which characteristics predict mortality.

Active video games as an exercise tool for children with cystic fibrosis.

Background: Active video games are used in many hospitals as exercise tools for children with cystic fibrosis. However, the exercise intensity associated with playing these games has not been examined in this population.

Methods: Children with cystic fibrosis [n=30, aged 12.

Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.

Rationale: Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, has been shown to improve lung function, pulmonary exacerbation rate, respiratory symptoms, and weight gain compared with placebo in patients with cystic fibrosis aged 12 years or older with a G551D-CFTR mutation.

Objectives: This randomized, double-blind, placebo-controlled trial evaluated ivacaftor in patients with cystic fibrosis aged 6-11 years with a G551D-CFTR mutation on at least one allele.

Methods: Patients were randomly assigned to receive ivacaftor administered orally at 150 mg (n = 26) or placebo (n = 26) every 12 hours for 48 weeks in addition to existing prescribed cystic fibrosis therapies.

Outcome in cystic fibrosis liver disease.

Objectives: Evidence suggests that cystic fibrosis liver disease (CFLD) does not affect mortality or morbidity in patients with cystic fibrosis (CF). The importance of gender and age in outcome in CF makes selection of an appropriate comparison group central to the interpretation of any differences in mortality and morbidity in patients with CFLD.

Methods: This is a 7-year follow-up of 42 children with CFLD and their age- and sex-matched controls.

Validation of continuous glucose monitoring in children and adolescents with cystic fibrosis: a prospective cohort study.

Objective: To validate continuous glucose monitoring (CGM) in children and adolescents with cystic fibrosis.

Research Design And Methods: Paired oral glucose tolerance tests (OGTTs) and CGM monitoring was undertaken in 102 children and adolescents with cystic fibrosis (age 9.5-19.

Congenital broncho-esophageal fistula: A case report.

Bronchopulmonary foregut malformations are a group of rare congenital anomalies affecting the respiratory and upper gastrointestinal tract. The rarity of these cases means their embryological origin continues to be a source of controversy. We present the case of a female infant, born at term with a malformed right arm, an absent right kidney and aplasia of the right lung.

Cystic fibrosis-associated liver disease: a population-based study.

Objectives: The aim of this study was to explore the clinical factors associated with the development of cystic fibrosis-associated liver disease (CFALD).

Study Design: This was a case-control study of all children (age 5-18 years) with established CFALD in the Republic of Ireland between January 1999 and June 2000. Each child was pair matched for age and sex with a patient with cystic fibrosis (CF) without evidence of liver disease.