Ap. Feranchak et al., Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen, J PEDIAT, 135(5), 1999, pp. 601-610
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 scre
enmg program.
Design: Vitamin status (serum retinol, 25-hydroxy vitamin D, ratio of alpha
-tocopherol/total lipids) and serum albumin were assessed at diagnosis (4 t
o 8 weeks), ages 6 months, 12 months, and yearly thereafter, to age 10 year
s.
Results: Deficiency of 1 or more vitamins was present in 44 (45.8%) of 96 p
atients at age 4 to 8 weeks as follows: vitamin A 29.0%; vitamin D 22.5%, a
nd vitamin E 22.8%. Of these patients with initial deficiency, the percent
that was deficient at 1 or more subsequent time points, despite supplementa
tion, was vitamin A 11.1%, vitamin D 12.5%, and vitamin E 57.1%. Of the ini
tial 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 I or m
ore vitamins ranged from 4% to 45% for any given year.
Conclusions: Despite supplementation with standard multivitamins and pancre
atic 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 ch
ildren with CF.