title: Fat-soluble-vitamin status during the first year of life in infants with cystic fibrosis identified by screening of newborns.
abstract: We investigated the fat-soluble-vitamin status during the first year of life in 36 infants with cystic fibrosis (CF) consecutively identified by screening of newborns. At initial evaluation (at age 51.0 +/- 26.7 d) 36% of patients were hypoalbuminemic, 21% had low serum retinol, 35% had low serum 25-hydroxyvitamin D. 38% had low serum alpha-tocopherol and low ratios of serum vitamin E to total lipids, and none had elevated protein in vitamin K absence (PIVKA). Hypoalbuminemia was more common in breast-fed than in formula-fed infants. Seventy-two-hour fecal fat excretion correlated inversely with serum alpha-tocopherol. Treatment with oral pancreatic enzyme supplements, a multiple vitamin, and additional vitamin E was associated with normalization of serum albumin, retinol, and 25-hydroxyvitamin D and negative PIVKA at age 6 and 12 mo. Approximately 10% of patients remained vitamin E deficient. Biochemical evidence of fat-soluble-vitamin deficiencies is common before age 3 mo in patients with CF and, except for vitamin E, these deficiencies corrected with standard therapy.
authors.affiliation: Department of Pediatrics, University of Colorado School of Medicine, Denver 80262.
authors.forename: R J
authors.initials: RJ
authors.lastname: Sokol
authors.forename: M C
authors.initials: MC
authors.lastname: Reardon
authors.forename: F J
authors.initials: FJ
authors.lastname: Accurso
authors.forename: C
authors.initials: C
authors.lastname: Stall
authors.forename: M
authors.initials: M
authors.lastname: Narkewicz
authors.forename: S H
authors.initials: SH
authors.lastname: Abman
authors.forename: K B
authors.initials: KB
authors.lastname: Hammond
mesh_terms: D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000097:blood* / Q000175:diagnosis; D005260:Female; D006801:Humans; D006887:Hydroxycholecalciferols / Q000097:blood; D007223:Infant; D007228:Infant Nutrition Disorders / Q000097:blood / Q000175:diagnosis; D007231:Infant, Newborn; D008055:Lipids / Q000097:blood; D008297:Male; D015997:Neonatal Screening; D009752:Nutritional Status; D012995:Solubility; D014801:Vitamin A / Q000097:blood; D014810:Vitamin E / Q000097:blood; D014812:Vitamin K / Q000097:blood; D014815:Vitamins / Q000097:blood*
chemical_list: D006887:Hydroxycholecalciferols; D008055:Lipids; D014815:Vitamins; D014801:Vitamin A; D014812:Vitamin K; D014810:Vitamin E