Treatment of vitamin K deficiency in cystic fibrosis: Effectiveness of a daily fat-soluble vitamin combination

Wilson, David C.; Rashid, Mohsin; Durie, Peter R.; Tsang, Anna; Kalnins, Daina; Andrew, Maureen; Corey, Mary; Shin, Janey; Tullis, Elizabeth; Pencharz, Paul B.

doi:10.1067/mpd.2001.113633

Cited by 71 publications

(46 citation statements)

References 14 publications

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“…Several studies showed that vitamin K supplementation induced a decrease of serum u-OC and may alter other bone markers such as NTX and BAP. [5][6][7] Our data showed a similar tendency, with the most prominent changes in the vitamin Figure 1 Boxplot showing total osteocalcin (t-OC in black), undercarboxylated osteocalcin (u-OC in grey), and carboxylated osteocalcin (c-OC in white) for vitamin K supplementation in CF patients, compared to healthy controls. Vitamin K supplementation is shown in three different groups: CF no = no supplementation; CF low = ,0.25 mg/day; and CF high = >1 mg/day.…”

Section: Discussionsupporting

confidence: 64%

Vitamin K supplementation in cystic fibrosis

Hoorn

2003

Archives of Disease in Childhood

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Section: Discussionsupporting

confidence: 64%

Vitamin K supplementation in cystic fibrosis

Hoorn

2003

Archives of Disease in Childhood

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“…Pathogenetic factors of osteoporosis in CF patients include chronic inflammation, delayed puberty, hypogonadism, glucocorticoid therapy, limited physical activity, poor tolerance to sun exposure, malnutrition and vitamin D malabsorption. Recent evidence suggests that vitamin K deficiency also plays an important role in the pathogenesis of decreased bone mineralization in CF patients [2,28,29].…”

Section: Introductionmentioning

confidence: 99%

The effect of vitamin K supplementation on biochemical markers of bone formation in children and adolescents with cystic fibrosis

Nicolaidou

Stavrinadis²,

Loukou

et al. 2006

Eur J Pediatr

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“…4 Thus, routine FSV supplementation after HPE has been advocated to prevent biochemical and clinical deficiency states of vitamins A, D, E, and K. 5 To provide these vitamins, 2 approaches are typically employed: (1) supplementation with large doses of each individual vitamin (either orally or intramuscularly) or (2) use of multiple vitamin preparations that contain increased levels of FSV. Most of the high-dose multiple vitamin preparations were developed for supplementation in infants and children with other diseases characterized by fat malabsorption, such as cystic fibrosis, 6 and not designed specifically for those with cholestasis. A liquid multiple FSV preparation made with d-a tocopheryl polyethylene glycol-1000 succinate (TPGS; a micelle forming water-soluble form of vitamin E), FSV/ TPGS, is frequently used in the management of infants with BA because of ease of administration and presumed efficacy due to the inclusion of TPGS, which enhances absorption of FSV independent of luminal bile acids.…”

mentioning

confidence: 99%

Efficacy of Fat-Soluble Vitamin Supplementation in Infants With Biliary Atresia

Shneider

Magee²,

Bezerra

et al. 2012

Pediatrics

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WHAT'S KNOWN ON THIS SUBJECT: Cholestasis predisposes to the development of fat-soluble vitamin (FSV) deficiency. D-a tocopheryl polyethylene glycol-1000 succinate and coadministered FSVs are absorbed in spite of cholestasis. WHAT THIS STUDY ADDS:Infants with biliary atresia with total bilirubin .2 mg/dL are at risk for fat-soluble vitamin (FSV) deficiency. A multivitamin preparation containing d-a tocopheryl polyethylene glycol-1000 succinate alone is not effective in treating biochemical FSV insufficiency in cholestatic infants. abstract 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, placebocontrolled 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. Pediatrics 2012;130:e607-e614 AUTHORS:

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Treatment of vitamin K deficiency in cystic fibrosis: Effectiveness of a daily fat-soluble vitamin combination

Cited by 71 publications

References 14 publications

Vitamin K supplementation in cystic fibrosis

Vitamin K supplementation in cystic fibrosis

The effect of vitamin K supplementation on biochemical markers of bone formation in children and adolescents with cystic fibrosis

Efficacy of Fat-Soluble Vitamin Supplementation in Infants With Biliary Atresia

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