Reversing Choline-Depletion in Children with Cystic Fibrosis and Exocrine Pancreatic Insufficiency

A clinical trial with a choline-rich structured lipid found that daily intake can significantly reduce choline depletion in cystic fibrosis patients with exocrine pancreatic insufficiency.

Researchers randomized 110 children with cystic fibrosis and pancreatic insufficiency to either placebo or the supplement, which is called LYM-X-SORB (LXS) and periodically assessed dietary choline intake, plasma cholines, plasma and fecal phospholipids, coefficient of fat absorption (CFA), pulmonary function, growth status, body composition, and safety measures. They also used magnetic resonance spectroscopy to measure muscle choline and liver fat in a subgroup of patients for comparison with comparable figures from healthy subjects of comparable age, sex and body size.

After 12 months, baseline dietary choline had increased 3-fold in the LXS group. Concentrations of plasma choline, betaine, and dimethylglycine increased in the LXS group but not in the placebo group (P = 0.007). Plasma lysophosphatidylcholine and phosphatidylcholine levels also increased in the LXS group (but not the placebo group) while the phosphatidylcholine-to-phosphatidylethanolamine ratio in LXS group fecal samples (but not placebo group samples) decreased (P ≤ 0.05). LXS use, moreover, was associated with a 6% increase in CFA (P = 0.001).

In the comparison with healthy subjects, the cystic fibrosis patients were found, at baseline, to have significantly higher levels of liver fat and significantly lower levels of choline in their calves. Use of LXS was associated with increased concentrations of choline in the muscles and an improvement of plasma choline status. However, use of the supplement was not associated with any evident changes in safety measures.

“LXS had improved choline intake, plasma choline status and muscle choline stores, compared with placebo,” the study authors wrote in the Journal of Pediatric Gastroenterology and Nutrition. “The choline-rich supplement was safe, accepted by participants and improved choline status in children with cystic fibrosis.”

Choline depletion is common in cystic fibrosis patients with exocrine pancreatic insufficiency — even those who take enzymes to aid digestion — and often results in abnormalities of both the liver and the muscle as well as abnormal levels of fatty acids.

Previous research indicates that children with cystic fibrosis suffer choline deficiency, at least in part, because they excrete significantly more phosphatidylcholine and lysophosphatidylcholine than healthy subjects. A 2005 study that appeared in The American Journal of Clinical Nutrition, for example, analyzed 5-day food records and fecal samples from 18 children with cystic fibrosis and 8 healthy children.

“Excretion of fecal fat (12.9 ± 1.7 g/d [cystic fibrosis group] and 3.9 ± 0.7 g/d [healthy group]), phospholipid (median: 130 g/d and 47.7 mg/d), phosphatidylcholine (19.6 g/d and 2.1 mg/d), and lysophosphatidylcholine (60.3 g/d and 16.9 mg/d) was significantly higher in children with CF than in control children, respectively (P < 0.05),” the study authors wrote.

The new study is not the first to find that supplemental choline can boost choline levels in children with cystic fibrosis. A 2007 study published in The American Journal of Clinical Nutrition found that supplements not only increased levels of choline but also affected concentrations of other compounds in the body.

“Supplementation with lecithin, choline, or betaine resulted in a significant increase in plasma methionine, S-adenosylmethionine (SAM), the ratio of SAM to S-adenosylhomocysteine (SAM:SAH), and the ratio of glutathione to glutathione disulfide (GSSD) and a decrease in SAH (n = 35),” the study authors wrote. “Supplementation with choline or betaine was associated with a significant decrease in plasma SAH and an increase in SAM:SAH, methionine, and glutathione:GSSG. Supplementation with lecithin or choline also increased plasma methionine and SAM.”