Background: Patients with cystic fibrosis (CF) are at risk of developing vi
tamin K deficiency because of pancreatic insufficiency, hepatobiliary disea
se, or both.
Objective: Our objective was to determine the prevalence of vitamin K defic
iency 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 take
n vitamin K supplements. Eight pancreatic-insufficient patients had advance
d CF-associated liver disease. Plasma prothrombin in vitamin K absence (PIV
KA-II) was measured by immunoassay. All control subjects had PIVKA-II conce
ntrations <3 mu g/L.
Results: Seventy-eight percent of pancreatic-insufficient patients had PIVK
A-II concentrations greater than or equal to 3 mu g/L (22.8 +/- 35.7 mu g/L
). All patients with CF-associated liver disease had abnormal PIVKB-II conc
entrations. The mean PIVKA-II concentration of pancreatic-insufficient pati
ents with liver disease was greater than that of those without liver diseas
e (46.6 +/- 65.3 compared with 15.3 +/- 26.1 mu g/L; P < 0.05). Five pancre
atic-sufficient patients had mildly elevated PIVKA-II concentrations. Six (
7%) pancreatic insufficient patients (3 with CF-associated liver disease) h
ad mildly prolonged prothrombin time but no clinical bleeding. Then was no
correlation between PIVKA-II concentrations and severity of fat malabsorpti
on or antibiotic use.
Conclusions: Vitamin K deficiency is common in unsupplemented patients with
CF and pancreatic insufficiency and routine supplementation should be cons
idered in all of these patients.