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J Pediatr. 1999 Nov;135(5):601-10.

Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen.

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Section of Pediatric Gastroenterology, Hepatology, and Nutrition, The Children's Hospital, Denver, CO 80218, USA.



To prospectively evaluate the biochemical status of vitamins A, D, and E in children with cystic fibrosis (CF).


A total of 127 infants identified by the Colorado CF newborn screening program.


Vitamin status (serum retinol, 25-hydroxy vitamin D, ratio of alpha-tocopherol/total lipids) and serum albumin were assessed at diagnosis (4 to 8 weeks), ages 6 months, 12 months, and yearly thereafter, to age 10 years.


Deficiency of 1 or more vitamins was present in 44 (45.8%) of 96 patients at age 4 to 8 weeks as follows: vitamin A 29.0%, vitamin D 22.5%, and vitamin E 22.8%. Of these patients with initial deficiency, the percent that was deficient at 1 or more subsequent time points, despite supplementation, was vitamin A 11.1%, vitamin D 12.5%, and vitamin E 57.1%. Of the initial patients with vitamin sufficiency, the percent who became deficient at any time during the 10-year period was as follows: vitamin A 4.5%, vitamin D 14.4%, and vitamin E 11.8%. The percent of patients deficient for 1 or more vitamins ranged from 4% to 45% for any given year.


Despite supplementation with standard multivitamins and pancreatic enzymes, the sporadic occurrence of fat-soluble vitamin deficiency and persistent deficiency is relatively common. Frequent and serial monitoring of the serum concentrations of these vitamins is therefore essential in children with CF.

[Indexed for MEDLINE]

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