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Original Research Communications |
1 From the Divisions of Gastroenterology and Nutrition and Haematology, the Research Institute, The Hospital for Sick Children, Department of Medicine, St Michael's Hospital, Toronto, and the Departments of Paediatrics and Medicine, University of Toronto.
Background: Patients with cystic fibrosis (CF) are at risk of developing vitamin K deficiency because of pancreatic insufficiency, hepatobiliary disease, or both.
Objective: Our objective was to determine the prevalence of vitamin K deficiency in unsupplemented patients with CF and to identify risk factors that might be associated with the deficiency.
Design: Ninety-eight patients with CF—83 who were pancreatic insufficient (age: 15.2 ± 10.7 y; range: 0.6–45.8 y), 15 who were pancreatic sufficient (age: 26.2 ± 11.6 y; range: 6.5–45.3 y), and 62 healthy individuals (age: 16.2 ± 12.8 y; range: 1–45 y)—were studied prospectively. None had taken vitamin K supplements. Eight pancreatic-insufficient patients had advanced CF-associated liver disease. Plasma prothrombin in vitamin K absence (PIVKA-II) was measured by immunoassay. All control subjects had PIVKA-II concentrations <3 µg/L.
Results: Seventy-eight percent of pancreatic-insufficient patients had PIVKA-II concentrations 
3 µg/L (22.8 ± 35.7 µg/L). All patients with CF-associated liver disease had abnormal PIVKA-II concentrations. The mean PIVKA-II concentration of pancreatic-insufficient patients with liver disease was greater than that of those without liver disease (46.6 ± 65.3 compared with 15.3 ± 26.1 µg/L; P < 0.05). Five pancreatic-sufficient patients had mildly elevated PIVKA-II concentrations. Six (7%) pancreatic insufficient patients (3 with CF-associated liver disease) had mildly prolonged prothrombin time but no clinical bleeding. There was no correlation between PIVKA-II concentrations and severity of fat malabsorption or antibiotic use.
Conclusions: Vitamin K deficiency is common in unsupplemented patients with CF and pancreatic insufficiency and routine supplementation should be considered in all of these patients.
Key Words: Cystic fibrosis • vitamin K • PIVKA-II • cystic fibrosis–associated liver disease • prothrombin • 
-glutamyl hydrolase
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