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

Summary: 
      Cystic Fibrosis (CF) is a complex disease with a wide range of clinical problems. Despite
      enzyme replacement therapy, children with cystic fibrosis (CF) may still have problems
      absorbing some nutrients. Detailed studies of the nutrient status of children with CF and
      have found low amounts of choline, an essential dietary nutrient, and altered levels of some
      amino acids in almost all patients. Choline is an essential dietary nutrient that is
      important in many important body functions, which include proving a source of methyl groups,
      the structure of cell membranes and in acetylcholine. Most choline is present in our diets in
      a fat known as phosphatidylcholine. Research studies have shown that children with cystic
      fibrosis do not absorb fat, including phosphatidylcholine very well. In previous studies, we
      showed that choline provided as a dietary supplement for 2 weeks improved choline status in
      children with cystic fibrosis.

      The purpose of this research is to find out if choline supplements over a longer duration of
      6 months will improve and maintain normal choline status in children with CF.
    

Detailed description: 
      This is a prospective study involving 34 children attending an outpatient clinic for children
      with CF. After enrollment, subjects will be followed for 9 months with each child serving as
      their own control in a repeated measures design.

      Chart data, height, weight, pulmonary function, dietary intake and a venous blood and urine
      sample will be collected at enrollment then every 3 months for 9 months. Choline
      supplementation will be from enrollment for 6 months, with follow up at 3 months post
      supplementation. To avoid additional hospital visits and blood draws, and to co-ordinate with
      pulmonary function, hematology, clinical chemistry tests as part of clinical care, each
      subject will be seen at routine scheduled clinic appointments, which are every 3 months.

      The objectives are to

        1. determine if supplementation with choline for 6 months corrects biochemical markers of
           choline deficiency and improves indices of reduced methylation capacity (including
           methionine, SAM, SAM/SAH) and redox status (GSH/GSSG) based on measures of blood and
           urine

        2. determine whether or not choline supplementation decreases plasma pro-inflammatory
           mediators

        3. determine if choline supplementation improves the low n-6 and n-3 fatty acids levels in
           children with CF,

        4. explore if choline supplementation has the potential to have clinical relevance in
           reducing recurrent inflammation, and improve pulmonary function and/or reduce liver
           disease.
    

Intervention name(s): Choline supplementation
Intervention type(s): Drug
Intervention description(s): Chart data, height, weight, pulmonary function, dietary intake and a venous blood and urine sample will be collected at enrollment then every 3 months for 9 months. Choline supplementation will be from enrollment for 6 months, with follow up at 3 months post supplementation. To avoid additional hospital visits and blood draws, and to co-ordinate with pulmonary function, hematology, clinical chemistry tests as part of clinical care, each subject will be seen at routine scheduled clinic appointments, which are every 3 months.
Intervention MeSH terms: Choline
Allocation: Randomized
Intervention model: Single Group Assignment
Masking: Single (Participant)
Primary purpose: Treatment
Keywords: Cystic Fibrosis; choline nutrition; choline and methyl metabolism; CF and oxidative stress