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

J Pediatr. 1999 Nov;135(5):601-10. doi: 10.1016/s0022-3476(99)70059-4.


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

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

Design: 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.

Results: 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.

Conclusions: 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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Child
  • Child, Preschool
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / drug therapy
  • Cystic Fibrosis / metabolism*
  • Follow-Up Studies
  • Gastrointestinal Agents / therapeutic use
  • Humans
  • Infant
  • Infant, Newborn
  • Neonatal Screening
  • Pancrelipase / therapeutic use
  • Prospective Studies
  • Time Factors
  • Vitamin A / blood
  • Vitamin A Deficiency / diagnosis
  • Vitamin A Deficiency / epidemiology*
  • Vitamin D / blood
  • Vitamin D Deficiency / diagnosis
  • Vitamin D Deficiency / epidemiology*
  • Vitamin E / blood
  • Vitamin E Deficiency / diagnosis
  • Vitamin E Deficiency / epidemiology*
  • Vitamins / therapeutic use


  • Gastrointestinal Agents
  • Vitamins
  • Vitamin A
  • Vitamin D
  • Vitamin E
  • Pancrelipase