Preventing early, prolonged vitamin E deficiency: an opportunity for better cognitive outcomes via early diagnosis through neonatal screening.
OBJECTIVE
To evaluate whether early diagnosis of cystic fibrosis (CF) through newborn screening (NBS) and early vitamin E status are associated with cognitive function.


METHODS
We assessed cognitive function for 71 children without meconium ileus (ages 7.3-16.9 years) enrolled in the screened (S) or control (C) group of the Wisconsin CF Neonatal Screening Project. The Test of Cognitive Skills, 2nd edition generated the cognitive skills index (CSI; mean = 100, SD = 16). Vitamin E deficiency at diagnosis was defined as plasma alpha-tocopherol (alpha-T) below 300 microg/dL (<300E). Primary analyses evaluated CSI scores across the 4 levels of group (S or C) by using alpha-T status (<300E or >300E) with analysis of covariance.


RESULTS
After adjusting for covariates, CSI in the C<300E group was significantly lower than each of the other groups (C>300E, S<300E, and S>300E; P < .05). The highest proportion of CSI scores >84 occurred in the C<300E group (41%). Patients in this group also had the lowest mean head circumference z-scores at diagnosis.


CONCLUSIONS
Our results show that prolonged alpha-T deficiency in infancy is associated with lower subsequent cognitive performance. Thus, diagnosis via NBS may benefit the cognitive development of children with CF, particularly in those prone to vitamin E deficiency during infancy.
Koscik
Rebecca L
RL
Department of Biostatistics/Medical Informatics, Nutritional Sciences, and Pediatrics, University of Wisconsin, Madison, WI 53792, USA. koscik@biostat.wisc.edu
Lai
HuiChuan J
HJ
Laxova
Anita
A
Zaremba
Kathleen M
KM
Kosorok
Michael R
MR
Douglas
Jeff A
JA
Rock
Michael J
MJ
Splaingard
Mark L
ML
Farrell
Philip M
PM
D000293:Adolescent; D000367:Age Factors; D000704:Analysis of Variance; D016022:Case-Control Studies; D002648:Child; D015362:Child Nutrition Disorders / Q000097:blood / Q000209:etiology / Q000517:prevention & control*; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology / Q000517:prevention & control*; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D042241:Early Diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015997:Neonatal Screening / Q000458:organization & administration*; D016032:Randomized Controlled Trials as Topic; D012307:Risk Factors; D012720:Severity of Illness Index; D014801:Vitamin A / Q000097:blood; D014811:Vitamin E Deficiency / Q000097:blood / Q000209:etiology / Q000517:prevention & control*; D014922:Wisconsin; D024502:alpha-Tocopherol / Q000097:blood
D014801:Vitamin A; D024502:alpha-Tocopherol