Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen.
OBJECTIVE
To prospectively evaluate the biochemical status of vitamins A, D, and E in children with cystic fibrosis (CF).


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


METHODS
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.
Feranchak
A P
AP
Section of Pediatric Gastroenterology, Hepatology, and Nutrition, The Children's Hospital, Denver, CO 80218, USA.
Sontag
M K
MK
Wagener
J S
JS
Hammond
K B
KB
Accurso
F J
FJ
Sokol
R J
RJ
D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism*; D005500:Follow-Up Studies; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015997:Neonatal Screening; D020799:Pancrelipase / Q000627:therapeutic use; D011446:Prospective Studies; D013997:Time Factors; D014801:Vitamin A / Q000097:blood; D014802:Vitamin A Deficiency / Q000175:diagnosis / Q000453:epidemiology*; D014807:Vitamin D / Q000097:blood; D014808:Vitamin D Deficiency / Q000175:diagnosis / Q000453:epidemiology*; D014810:Vitamin E / Q000097:blood; D014811:Vitamin E Deficiency / Q000175:diagnosis / Q000453:epidemiology*; D014815:Vitamins / Q000627:therapeutic use
D005765:Gastrointestinal Agents; D014815:Vitamins; D014801:Vitamin A; D014807:Vitamin D; D014810:Vitamin E; D020799:Pancrelipase