Primary Outcome Measures:
- The change in plasma choline and its metabolites with increasing age in children with CF compared to a healthy reference group without CF [ Time Frame: 12 months ] [ Designated as safety issue: No ]
Secondary Outcome Measures:
- The relationship between choline and acetylcholine, and markers of oxidative stress, inflammation and disturbed methyl metabolism [ Time Frame: 12 months ] [ Designated as safety issue: No ]
The objective of this clinical project is to determine the status of choline and related metabolites in a large group of children with Cystic Fibrosis (CF). Based on the results it will be possible to determine which children are deficient or at risk for choline deficiency, and might benefit most from supplementation to sustain improvement in the plasma choline, and its metabolites. This will be a cross-sectional study that will, over a 6 month period, look to enroll as many patients as possible from 140 children with CF who are seen regularly at the CF Clinic at B.C.'s Children's Hospital. The study will involve the collection of blood and urine at the time of a scheduled appointment. All procedures for enrollment, collection of chart data and blood samples will follow procedures used in our previous studies. Body weight and height will be measured and routine blood work including liver enzymes, hematology, serum zinc, selenium and vitamins A and E will be completed as part of the clinic appointment. CF genotype, gender, birth date, hematology, clinical chemistry, anthropometric and nutritional measures, liver ultrasound, biopsy reports where available, pancreatic function (elastase, chymotrypsin/secretin-CCK) and all medications including mineral, vitamin and nutritional and enzyme supplements will be recorded from chart data. Blood samples will be used for analyses of choline and its metabolites and various biomarkers in redox pathways. A urine sample will be collected and used to assay excretion of related metabolites. Creatine will be analyzed to avoid the need for quantitative 24 hour urine collection.