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Vitamin K status in cystic fibrosis
E A Cornelissen1, A F van Lieburg, K Motohara
1Department of Paediatrics, University Hospital Nijmegen, The Netherlands.
Insights
Vitamin K deficiency is rare in children with cystic fibrosis, as indicated by protein induced by vitamin K absence-II (PIVKA-II) levels. Routine vitamin K1 supplementation is not recommended, with a maximum daily dose of 1 mg if needed.
Area of Science:
- Biochemistry
- Pediatrics
- Nutritional Science
Background:
- Protein induced by vitamin K absence-II (PIVKA-II) indicates impaired vitamin K-dependent carboxylation.
- Cystic fibrosis (CF) may affect vitamin K status.
Purpose of the Study:
- To assess vitamin K deficiency prevalence in pediatric cystic fibrosis patients.
- To evaluate the impact of vitamin K1 supplementation in these patients.
Main Methods:
- Measured plasma PIVKA-II and vitamin K1 concentrations in 24 children with CF.
- Compared levels in 16 unsupplemented children with 8 vitamin K1-supplemented children.
Main Results:
- Only one unsupplemented child had detectable PIVKA-II, with low vitamin K1 levels.
- Other unsupplemented children had normal vitamin K1 levels.
- Supplemented children exhibited significantly elevated vitamin K1 levels.
Conclusions:
- Vitamin K deficiency is uncommon in cystic fibrosis.
- Coagulation monitoring is advised, but routine vitamin K1 supplementation is not generally recommended.
- If supplementation is necessary, a cautious starting dose of 1 mg daily is suggested.
Abstract:
Appearance of PIVKA-II (protein induced by vitamin K absence-II) in serum is a biochemical sign of insufficient vitamin K-dependent carboxylation of prothrombin. Plasma concentrations of PIVKA-II and vitamin K1 were determined in 24 children with cystic fibrosis. Eight were supplemented with vitamin K1. The purpose of the study was to determine the occurrence of vitamin K deficiency in cystic fibrosis and to evaluate the effect of vitamin K supplementation. PIVKA-II was detectable in only one unsupplemented child. In this patient, the concentration of vitamin K1 was below the limit of detection of 60 ng/l. Vitamin K1 levels in the other unsupplemented children were normal (mean 476 ng/l = 1 mmol/l). The supplemented patients showed extremely high levels of vitamin K1 (mean 22445 ng/l = 50 nmol/l). In conclusion, vitamin K deficiency occurs infrequently in cystic fibrosis. Checking the coagulation system is advised, but routine vitamin K supplementation is not recommended. If additional vitamin K is needed, the starting dose should not exceed 1 mg daily.
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