title: Treatment of vitamin K deficiency in cystic fibrosis: Effectiveness of a daily fat-soluble vitamin combination.
abstract: 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 effectiveness of an oral fat-soluble vitamin combination (ADEKs) to treat patients with vitamin K deficiency.
  
  
  METHODS
  Patients with PI and CF (mean age, 15 years; range, 0.6 to 46 years) including 6 with advanced CFLD were prospectively enrolled in a study of a fat-soluble vitamin combination taken on a daily basis. None had received vitamin K supplementation for at least 4 months before the study. Fat-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 vitamin 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-II levels (P =.001). All 6 patients with advanced CFLD had abnormal PIVKA-II levels (median, range of 20.8, 5.5 to 55 ng/mL) before treatment, which corrected to normal in 50% (4.1, 2.1 to 65 ng/mL). Four patients, 2 with CFLD, had a prolonged prothrombin time (>13.5 seconds) at both time periods.
  
  
  CONCLUSIONS
  An oral fat-soluble vitamin combination with a modest amount of vitamin K can, as a daily supplement, improve the PIVKA-II levels in patients with PI and CF.
authors.affiliation: Division of Gastroenterology/Nutrition, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario, Canada, M5G 1X8.
authors.forename: D C
authors.initials: DC
authors.lastname: Wilson
authors.forename: M
authors.initials: M
authors.lastname: Rashid
authors.forename: P R
authors.initials: PR
authors.lastname: Durie
authors.forename: A
authors.initials: A
authors.lastname: Tsang
authors.forename: D
authors.initials: D
authors.lastname: Kalnins
authors.forename: M
authors.initials: M
authors.lastname: Andrew
authors.forename: M
authors.initials: M
authors.lastname: Corey
authors.forename: J
authors.initials: J
authors.lastname: Shin
authors.forename: E
authors.initials: E
authors.lastname: Tullis
authors.forename: P B
authors.initials: PB
authors.lastname: Pencharz
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000150:complications; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011498:Protein Precursors / Q000097:blood; D011516:Prothrombin; D011517:Prothrombin Time; D014812:Vitamin K / Q000008:administration & dosage; D014813:Vitamin K Deficiency / Q000150:complications / Q000188:drug therapy*; D014815:Vitamins / Q000008:administration & dosage*
chemical_list: D015415:Biomarkers; D011498:Protein Precursors; D014815:Vitamins; D014812:Vitamin K; C008208:acarboxyprothrombin; D011516:Prothrombin