ePS05.8 Vitamin K status and supplementation in children with cystic fibrosis (CF)

Objectives 1.To establish the prevalence of Vitamin K deficiency [raised plasma Prothrombin In Vitamin K Absence (PIVKA II (>0.2 au/ml)] and suboptimal Vitamin K status (low stores) indicated by a K1 2.To evaluate the effectiveness of age related doses of Vitamin K 1 (Phytomenadione) on a weekly basis to correct low K stores and a daily basis for Vitamin K deficiency. Patients and Methods 36 children with CF [31 patients with PICF and 5 with Pancreatic sufficient CF (PSCF)] had baseline measurements by immunoassay for plasma PIVKA II and Vitamin K1. Those with a low Vitamin K1 level received a weekly dose of Phytomenadione (10 mg for those over 7 years of age and 5 mg for those under 7 years of age) and those with deficiency received a daily dose of Phytomenadione (10 mg for those >7 years of age and 5 mg for those Results Twenty-six children (72%) had a compromised Vitamin K status, with 81% (n = 21) of these children having low vitamin K stores and 19% (n = 5) having deficiency. Of the 26 children 75% were PI and 25% were PS; none of those with PS were deficient. Following a year of supplementation 90% (19) of those receiving weekly Phytomenadione had their low stores corrected and 100% of those on a daily dose had their deficiency corrected. Conclusions This study found that the majority of children with PICF and some with PSCF have low vitamin K stores and this can be corrected using an age related weekly dose of Phytomenadione. Deficiency in PICF can be corrected using a daily age related dose of Phytomenadione.