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Jørgensen, M. H.1; Ott, P.2; Michaelsen, K. F.3; Porsgaard, T.4; Jensen, F.5; Lanng, S.1

Journal of Pediatric Gastroenterology and Nutrition: June 2004 - Volume 39 - Issue - p S46
ABSTRACTS: Oral Presentation Abstracts

1Department of Pediatric, Rigshospitalet, Copenhagen,2Department of Medicine V, University hospital of Aarhus, Aarhus,3Department of Human Nutrition, The Royal Veterinary and Agricultural University, Frederiksberg,4Biochemistry and Nutrition group, BioCentrum, Technical University of Denmark, Lyngby,5Ultrasound section, Rigshospitalet, Copenhagen, Denmark

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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[1] (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 [2]. 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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1.Williams, S.G. et al. Journal of Hepatology 1995;22;513–21.
2.Albrecht, T. et al. Lancet 1999;353;1579–83.
© 2004 Lippincott Williams & Wilkins, Inc.