The present study determined the plasma
amino acid status in children with
cystic fibrosis (CF) and
pancreatic insufficiency (PI) in the modern medical and nutritional care setting and investigated the effect of
choline supplementation on
amino acid status. A total of 110 children aged 5 to 18 years with CF and PI were randomized to receive
choline-enriched structured
lipid (LYM-X-SORB) or placebo with similar energy and fat content. Plasma
amino acids were measured at baseline and 3 and 12 months. We hypothesized that
choline supplementation would result in lower plasma
homocysteine concentrations in children with CF. At baseline,
dietary protein intake was high and the
amino acid profile was within laboratory reference ranges in most participants.
Alanine and
cysteine were elevated in 24% and 36% of participants, respectively. Children with baseline
alanine above reference range had improved
weight, body mass index, and fat-free mass. Low
homocysteine was found in 62% of children 11 years and older. After 3 and 12 months, there was no effect of
choline supplementation on
methionine or
homocysteine status. Compared with placebo,
choline supplementation resulted in increased
glycine and decreased
threonine,
histidine,
valine, and total branch chained
amino acids at 12 months. In conclusion, daily
choline supplementation with LYM-X-SORB did not alter
methionine-
homocysteine metabolism but did result in alterations in other
amino acids in children with CF and PI.