Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia.
OBJECTIVE
Cholestasis predisposes to fat-soluble vitamin (FSV) deficiencies. A liquid multiple FSV preparation made with tocopheryl polyethylene glycol-1000 succinate (TPGS) is frequently used in infants with biliary atresia (BA) because of ease of administration and presumed efficacy. In this prospective multicenter study, we assessed the prevalence of FSV deficiency in infants with BA who received this FSV/TPGS preparation.


METHODS
Infants received FSV/TPGS coadministered with additional vitamin K as routine clinical care in a randomized double-blinded, placebo-controlled trial of corticosteroid therapy after hepatoportoenterostomy (HPE) for BA (identifier NCT 00294684). Levels of FSV, retinol binding protein, total serum lipids, and total bilirubin (TB) were measured 1, 3, and 6 months after HPE.


RESULTS
Ninety-two infants with BA were enrolled in this study. Biochemical evidence of FSV insufficiency was common at all time points for vitamin A (29%-36% of patients), vitamin D (21%-37%), vitamin K (10%-22%), and vitamin E (16%-18%). Vitamin levels were inversely correlated with serum TB levels. Biochemical FSV insufficiency was much more common (15%-100% for the different vitamins) in infants whose TB was ≥2 mg/dL. At 3 and 6 months post HPE, only 3 of 24 and 0 of 23 infants, respectively, with TB >2 mg/dL were sufficient in all FSV.


CONCLUSIONS
Biochemical FSV insufficiency is commonly observed in infants with BA and persistent cholestasis despite administration of a TPGS containing liquid multiple FSV preparation. Individual vitamin supplementation and careful monitoring are warranted in infants with BA, especially those with TB >2 mg/dL.
Department of Pediatrics, Children’s Hospital Pittsburgh of UPMC, Pittsburgh, PA 15224, USA. benjamin.shneider@chp.edu
Benjamin L
BL
Shneider
John C
JC
Magee
Jorge A
JA
Bezerra
Barbara
B
Haber
Saul J
SJ
Karpen
Trivellore
T
Raghunathan
Philip
P
Rosenthal
Kathleen
K
Schwarz
Frederick J
FJ
Suchy
Nanda
N
Kerkar
Yumirle
Y
Turmelle
Peter F
PF
Whitington
Patricia R
PR
Robuck
Ronald J
RJ
Sokol
D001361:Avitaminosis / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D001656:Biliary Atresia / Q000150:complications* / Q000601:surgery; D001663:Bilirubin / Q000097:blood; D019587:Dietary Supplements; D004311:Double-Blind Method; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011092:Polyethylene Glycols / Q000627:therapeutic use; D011171:Portoenterostomy, Hepatic; D011182:Postoperative Care; D012177:Retinol-Binding Proteins / Q000032:analysis; D014801:Vitamin A / Q000097:blood; D014807:Vitamin D / Q000031:analogs & derivatives / Q000097:blood; D014810:Vitamin E / Q000031:analogs & derivatives* / Q000097:blood / Q000627:therapeutic use; D014812:Vitamin K / Q000627:therapeutic use*; D024502:alpha-Tocopherol / Q000097:blood
D012177:Retinol-Binding Proteins; D014801:Vitamin A; D014812:Vitamin K; D014807:Vitamin D; D014810:Vitamin E; D011092:Polyethylene Glycols; C104450:25-hydroxyvitamin D; D024502:alpha-Tocopherol; C014225:tocophersolan; D001663:Bilirubin