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

Objective: Patients with cystic fibrosis (CF) and pancreatic insufficiency (PI) commonly have vitamin K deficiency, and those with CF-associated liver disease (CFLD) have universal vitamin K deficiency. We evaluated the effec tiveness of an oral fat-soluble vitamin combination (ADEKs (R)) to treat pa tients with vitamin K deficiency. Study design: Patients with PI and CF (mean age, 15 years; range, 0.6 to 46 years) including 6 with advanced CFLD M ere prospectively enrolled in a st udy of a fat-soluble vitamin combination taken on a daily basis. None had r eceived vitamin K supplementation for at least 4 months before the study. F at-soluble vitamin combination supplementation was given for a minimum of 4 months; the mean vitamin K intake was 0.18 mg/d (SD = 0.1, range, 0 to 0.3 ). The primary outcome was change in plasma PIVKA-II (prothrombin in vitami n K absence). Results: Before supplementation 58 (81%) of 72 patients had abnormal PIVKA- II levels (>2.9 ng/mL). After supplementation 29 (40%) had abnormal PIVKA-I I levels (P = .001). All 6 patients with advanced CFLD had abnormal PIVKA-I I levels (median, range of 20.8, 5.5 to 55 ng/mL) before treatment, which c orrected to normal in 50% (4.1, 2.1 to 65 ng/mL). Four patients, 2 with CFL D, had a prolonged prothrombin time (>13.5 seconds) at both time periods. Conclusions: An oral fat-soluble vitamin combination with a modest amount o f vitamin K can, as a daily supplement, improve the PIVKA-II levels in pati ents with PI and CF.