Choline Nutrition in Children With Cystic Fibrosis
Condition: Cystic Fibrosis
MeSH terms: Cystic Fibrosis; Fibrosis
Study type: Interventional

Summary: 
      Cystic fibrosis (CF) is the most common lethal, inherited disorder among Caucasians. Choline
      is an essential vitamin and as a methyl donor is critically needed to support the normal
      metabolism. Our previous studies have demonstrated that children with CF have depleted levels
      of choline. The purpose of this study is to supply a choline supplement to children with CF
      to see if their nutrition and methyl status can be improved.
    

Detailed description: 
      This will be a prospective, repeated measures study involving 34 children with CF who will
      take a supplement of water soluble choline bitartrate, 2 gm/day with meals for 6 months. The
      baseline (day 0) results for each child will serve as his/her own control, and assessments
      will be made at 3 months and 6 months choline supplementation and again 3 months after
      stopping choline.

      The supplement will be provided as capsules containing 250 mg choline bitartrate. 4 capsules
      will be taken with or immediately before each of two meals per day: breakfast and dinnertime
      meals, providing 1 gm of supplemental choline each day.

      The children will be enrolled by description of the project to the child and their parent(s)
      at a CF clinic appointment. Body weight, height and blood pressure 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. The hematology and clinical
      chemistry will be done by the Hematopathology and Clinical Chemistry labs at the B.C.'s
      Children's Hospital. CF genotype, gender, birth date, hematology, clinical chemistry,
      anthropometry, nutritional measures, pulmonary function test results, chest X-Ray and/or CT
      scans, pancreatic function test results (fecal elastase, chymotrypsin or secretin-CCK),
      medications and supplements (including enzymes, vitamins, minerals, nutrition supplements &
      Natural Health Products) and where available, liver ultrasound and biopsy reports will be
      collected from chart data. Information in the subject's medical charts relating to antibiotic
      therapy, duration of illnesses, hospitalization and diagnosis will be reviewed to ensure the
      inclusion/exclusion criteria are met.

      Assessment of pulmonary function by computer assisted spirometry which includes the measures
      of forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and forced
      mid-expiratory flow (FEF 25-75) is completed for all children as part of each regularly
      scheduled clinic visit and the results collected for this study.
    

Intervention name(s): Vitamin: Choline Bitartrate (2-hydroxyethyl) trimethylammonium salt 1:1
Intervention type(s): Dietary Supplement
Intervention description(s): This is a prospective, repeated measures study involving children with Cystic Fibrosis. Children will be assessed (1) before starting the choline supplement, (2) after taking the supplement for 6 months and after the supplement has been discontinued for 3 months.
Intervention MeSH terms: Choline
Allocation: Non-Randomized
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Treatment
Keywords: choline; cystic fibrosis; methyl metabolism; fatty acids; oxidative stress; pulmonary function