ABSTRACTS: Oral Presentation Abstracts
Introduction: Patients with Cystic fibrosis (CF) are at risk of low levels of fat soluble vitamins. 1/3 of CF- patients had CF-related liver disease (CF-RL) defined as pathological hepatic ultrasound and/or impaired liver biochemistry. It is unknown whether deficit of fat soluble vitamins is related to CF-RL.
Methods: 20 patients with known CF-RL treated with ursodeoxycholic acid (+URSO) were matched by age, BMI, chronic P.Aeg. infection, Diabetes Mellitus (DM) and sex with 20 CF-patient without known CF-RL (-URSO). All patients were daily supplemented with fat soluble vitamins(ADEKs: <10 y 1 tablet, >10 y 2 tablets). Vitamin E was determined by fluorescence detection at 292 nm using HPLC, vitamin A (retinol) was determined by HPLC (UV detection), vitamin D (25 hydroxy) was measured by RIA. All patients went through hepatic ultrasound, and scored according to William ultrasound scoring scale (WUSS). Pathological WUSS was set to be above 3 point. Hepatic transit-time (HTT) was measured in 28 patients. HTT is the time lapse from an i.v. bolus injection of sulphurhexafluoride in a cubital vein until arrival in a hepatic vein . Pathological HTT was set to be below 24 sec. for >18 y (No reference value for children).
Results: Mean age was 18.6 y (range 4.5–35.1). 82% of the +URSO patients had either pathological WUSS or HTT versus 44% in the -URSO group (p=0.05). In univariate analyses, the mean vitamin A, D and E were not related to URSO treatment, WUSS or HTT. 17% had vitamin A<1.05 mumol/l, 20% had vitamin D<20 nmol/l and 41% had vitamin E<4,4 mg/l. 2 patients had 3 vitamins below the lower limit of reference, 16 patients had sufficient levels of all 3 vitamins. In multiple regression analysis’s (backward) including P. Aeg. infection, DM, age, WUSS, HTT and ±URSO. Vitamin A was negative associated with WUSS (p=0.02) (whole model: R2=34%), vitamin E was positive associated with HTT (p=0.06) (whole model: R2=24%). Vitamin D was negative associated with D.M (p=0.009) (whole model: R2=46%).
Conclusion: Despite daily supplements with fat soluble vitamins, CF-patients are at risk for deficit of fat soluble vitamins, indicating poor absorption. Despite the difficulties with diagnosing CF-RL, our data indicate that CF-RL is associated with decreased levels of vitamin A and E. Other factors increasing oxidative stress may also be important such as DM.
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