Lipid spectrum in paediatric patients with cystic fibrosis

Objectives and study: Current recommendation for dietary fat intake in children and adolescents with
cystic fibrosis (CF) is as high as 35-40 energy percent (En%) to meet the advised 120% energy intake
and thereby compensate for malabsorption and high resting energy expenditure. This will generally
result in a high saturated fat intake, which was found to be well above the advised limit of 10 En% for
healthy counterparts. This might cause abnormalities in serum lipids and therefore an increased risk
on developing cardiovascular disease later in life. Whether the high (saturated) fat intake in children
and adolescents with CF is indeed associated with an abnormal serum lipid spectrum is unknown. So
we aimed to investigate this in a paediatric CF patient population and describe the lipid spectrum,
expressed as total cholesterol, low-density lipoprotein cholesterol (LDL-cholesterol), high-density
lipoprotein cholesterol (HDL-cholesterol) and triglycerides, and the triglycerides to HDL-cholesterol
ratio (TG/HDL-ratio) as well as the correlation between the lipid spectrum and dietary fat intake and
nutritional status.
Methods: Between March 1st 2013 and August 31st 2015 we randomly collected 112 fasting
measurements of the lipid spectrum in paediatric patients with CF (aged 14.0±2.4 years, 55 girls). We
obtained 73 corresponding completed 3-day dietary food records with calculated fat intake (En%) and
saturated fat intake (En%). As a measure of nutritional status we included z-scores body mass index
(BMI). Data of lipid spectrum were compared with those of healthy controls according to age and
gender and were presented as percentage of the reference values (% ref val). Correlations between
the lipid spectrum, dietary fat intake and nutritional status were studied by using the Spearman’s rank
correlation coefficient.
Results: In our study sample we found relative low levels of cholesterol, LDL-cholesterol and HDLcholesterol and high levels of triglycerides compared to healthy controls (Table). In 21% of the
patients, we found a TG/HDL-ratio ≥1.3, which is considered to be a risk factor for the development of
cardiovascular disease. Furthermore we found a significant correlation between HDL-cholesterol and
total fat intake (r 0.38, p<0.01) and HDL-cholesterol and saturated fat intake (r 0.25, p<0.04) as well as
between TG/HDL-ratio and total fat intake (r -0.34, p<0.01). No other correlations between the lipid
spectrum and dietary fat intake or nutritional status as such were found (p≥0.08).
Table: Lipid spectrum of 112 paediatric patients with CF (55 girls).
Lipid spectrum Median (range) expressed as % ref val
Total cholesterol 79 (68-91)
LDL-cholesterol 70 (57-78)
HDL-cholesterol 85 (72-101)
Triglycerides 125 (94-172)
Conclusion: We described low levels of total cholesterol, LDL-cholesterol and HDL-cholesterol and
high levels of triglycerides in children and adolescents with CF, although we found no clear
correlations between the lipid spectrum and dietary fat intake. Additionally we found an increased
TG/HDL-ratio in 21% of our paediatric patients w