Vitamin K status in cystic fibrosis

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 22 445 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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