Exogenous and endogenous determinants of vitamin K status in cystic fibrosis

Sci Rep. 2015 Jul 10;5:12000. doi: 10.1038/srep12000.


Cystic fibrosis (CF) patients are at high risk for vitamin K deficiency. The effects of vitamin K supplementation are very ambiguous. Therefore, we aimed to define the determinants of vitamin K deficiency in a large cohort of supplemented - 146 (86.9%) and non-supplemented - 22 (13.1%) CF patients. Vitamin K status was assessed using prothrombin inducted by vitamin K absence (PIVKA-II) and undercarboxylated osteocalcin (u-OC). The pathological PIVKA-II concentration (≥ 2 ng/ml) and abnormal percentage of osteocalcin (≥ 20%) were found in 72 (42.8%) and 60 (35.7%) subjects, respectively. We found that liver involvement, diabetes, and glucocorticoid therapy were potential risk factors for vitamin K deficiency. Pathological concentrations of PIVKA-II occurred more frequently in patients with pancreatic insufficiency and those who have two severe mutations in both alleles of the CFTR gene. Pathological percentage of u-OC was found more frequently in adult CF patients and those not receiving vitamin K. However, it seems that there are no good predictive factors of vitamin K deficiency in CF patients in everyday clinical care. Early vitamin K supplementation in CF patients seems to be warranted. It is impossible to clearly determine the supplementation dose. Therefore, constant monitoring of vitamin K status seems to be justified.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Anti-Bacterial Agents / therapeutic use
  • Biomarkers / analysis
  • Child
  • Child, Preschool
  • Cohort Studies
  • Cystic Fibrosis / drug therapy
  • Cystic Fibrosis / pathology*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Dietary Supplements
  • Female
  • Genotype
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoassay
  • Infant
  • International Normalized Ratio
  • Male
  • Nutritional Status
  • Osteocalcin / analysis
  • Osteocalcin / chemistry
  • Polymorphism, Single Nucleotide
  • Protein Precursors / analysis
  • Prothrombin / analysis
  • Pseudomonas Infections / drug therapy
  • Regression Analysis
  • Risk Factors
  • Vitamin K / analysis*
  • Vitamin K Deficiency / etiology
  • Young Adult


  • Anti-Bacterial Agents
  • Biomarkers
  • CFTR protein, human
  • Glucocorticoids
  • Protein Precursors
  • Osteocalcin
  • Vitamin K
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • acarboxyprothrombin
  • Prothrombin