Clin Nutr. 2010 Feb 17
Drivdal M, Løken EB, Hagve TA, Bergstad I, Aagenæs O.
Regional Department of Eating Disorders, Division of Psychiatry,
Building 37A, Oslo University Hospital, Ullevaal, N-0407 Oslo, Norway.
BACKGROUND&AIMS: Patients with lymphoedema cholestasis syndrome 1/
Aagenaes Syndrome need a fat reduced diet when cholestatic. We wanted
to assess the need for dietary counselling outside cholestatic
episodes, and hypothetized that no counselling was needed.
METHODS: Fifteen patients above 10 years of age without symptoms of
cholestasis were compared with a sex and age matched control group.
Diet from a four-day weighed record and blood samples were compared
between the two groups and with general Norwegian recommendations.
RESULTS: The patients had a similar diet to the healthy controls,
except for statistically significant lower intake of energy from total
fat (p=0.04) and saturated fat (0.02), and fish (0.05). The patients
met the dietary recommendations for macronutrients, except for
saturated fat, monounsaturated fat, refined sugar and fibre.
Supplements were needed to meet the micronutrient recommendations.
Patients had a significantly lower serum level of alpha-tocopherol
(0.01) compared with the control group, and the serum 25-OH D level
was below reference ranges.
CONCLUSIONS: The patients would benefit from counselling on fat
quality, carbohydrates including fibre intake, and individual needs
for vitamins D and E. To secure serum 25-OH D and alpha-tocopherol
levels within reference ranges, regular examinations to determine the
need for supplementary vitamins D and E are recommended. European
Society for Clinical Nutrition and Metabolism