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Fat-Soluble Vitamins in Cystic Fibrosis and Pancreatic Insufficiency: Efficacy of a Nutrition Intervention

Bertolaso, Chiara; Groleau, Veronique; Schall, Joan I.; Maqbool, Asim; Mascarenhas, Maria; Latham, Norma E.; Dougherty, Kelly A.; Stallings, Virginia A.

Journal of Pediatric Gastroenterology and Nutrition: April 2014 - Volume 58 - Issue 4 - p 443–448
doi: 10.1097/MPG.0000000000000272
Original Articles: Hepatology and Nutrition

Objectives: The aim of the study was to assess the impact of LYM-X-SORB (LXS), an organized lipid matrix that has been shown to be absorbable without pancreatic enzyme therapy on fat-soluble vitamin status in children with cystic fibrosis (CF) and pancreatic insufficiency (PI).

Methods: Children with CF and PI were randomized to daily LXS or an isocaloric placebo comparison supplement for 12 months. Serum vitamins A (retinol), D (25-hydroxyvitamin D[25D]), E (α-tocopherol, α-tocopherol:cholesterol ratio), and K (percentage of undercarboxylated osteocalcin [%ucOC] and plasma proteins induced by vitamin K absence factor II [PIVKA II]) were assessed at baseline and 12 months. Dietary intake was determined using 3-day weighed food records and supplemental vitamin intake by a comprehensive questionnaire.

Results: A total of 58 subjects (32 boys, age 10.3 ± 2.9 years [mean ± standard deviation]) with complete serum vitamin, dietary and supplemental vitamin data were analyzed. After adjusting for dietary and supplemental vitamin intake, serum retinol increased 3.0 ± 1.4 μg/dL (coefficient ± standard error) (adjusted R 2 = 0.02, P = 0.03) and vitamin K status improved as demonstrated by a decreased percentage of undercarboxylated osteocalcin of −6.0% ± 1.6% by 12 months (adjusted R 2 = 0.15, P < 0.001). These changes occurred in both the LXS and placebo comparison groups. No changes in serum 25D or α-tocopherol were detected. Both nutrition interventions increased caloric intake a mean of 83 ± 666 kcal/day by 12 months.

Conclusions: Vitamins A and K status improved, whereas vitamins D and E status was unchanged during 12 months of LXS and isocaloric placebo comparison supplement in children with CF and PI.

Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

Address correspondence and reprint requests to Chiara Bertolaso, MD, The Children's Hospital of Philadelphia, 3535 Market Street, Room 1556, Philadelphia, PA 19104 (e-mail:

Received 15 April, 2013

Accepted 26 November, 2013 registration no.: NCT00406536.

This study was supported by NIDDK (R44DK060302) and the Nutrition Center at The Children's Hospital of Philadelphia. The study was also supported by the National Center for Research Resources (grant UL1RR024134) and is now at the National Center for Advancing Translational Sciences (grant UL1TR000003). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

V.A.S. received consulting honoraria and travel expenses from companies with interest in CF care. The other authors report no conflicts of interest.

© 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,