Non-cholesterol Sterols in Healthy Children and Low Cholesterol Dietary Treatment of Children Suffering from Familial Hypercholesterolemia

Overview

Objective:
To estimate the normal values of precursors of endogenic cholesterol synthesis (lathosterol, desmosterol, lanosterol) as well as of phytosterols (campesterol, sitosterol) in healthy children or adults and evaluate their diagnostic significance during dietary treatment of children patients suffering from familial hypercholesterolemia.

Design:
Retrospective analysis of data from routine examination of above mentioned markers during diagnostics, introducing of treatment and its monitoring within last 3 years in metabolic outpatient department.

Material and methods:
GC/MS estimation of fasting plasmatic values in children patients as well as in healthy control groups of children and adults.

Results:
Reference values for lathosterol in plasma: a) 5–6 yrs girls 4.0 ± 0.78 μmol/l, boys 3.7 ± 0.92 μmol/l; b) 7–10 yrs girls 6.03 ± 0.63 μmol/l, boys 6.0 ± 0.60 μmol/l; c) 11–15 yrs girls 7,17 ± 0,45 μmol/l, boys 6.13 ± 0.51 μmol/l; d) 16–18 yrs girls 5.66 ± 0.82 μmol/l, boys 6.31 ± 1.3 μmol/l. Adults (blood donnors 20–65 yrs): women 6.36 ± 0.7 μmol/l, men 7.44 ± 1.06 μmol/l. Campesterol: a) 5–6 yrs girls 9.89 ± 1.18 μmol/l, boys 9.73 ± 1.38 μmol/l; b) 7–10 yrs girls 10.95 ± 0.95 μmol/l, boys 9.65 ± 0.9 μmol/l; c) 11–15 yrs girls 10.45 ± 0.68 μmol/l, boys 9.2 ± 0.77 μmol/l; 16–18 yrs girls 10.1 ± 1.24 μmol/l, boys 15.67 ± 1.96 μmol/l; Adults: women 10.7 ± 0.4 μmol/l, men 9.1 ± 1.61 μmol/l. Sitosterol: a) 5–6 yrs girls 7.45 ± 0.87 μmol/l, boys 7.5 ± 1.1 μmol/l; b) 7–10 yrs girls 7.7 ± 0.7 μmol/l, boys 7.2 ± 1.0 μmol/l, c) 11–15 yrs girls 7.1 ± 1.5 μmol/l, boys 7.2 ± 1.4 μmol/l; d) 16–18 yrs girls 5.78 ± 0.92 μmol/l, boys 7.36 ± 1.45 μmol/l. Adults women: 8.65 ± 0.77 μmol/l, men 7,72 ± 1,19 μmol/l. During strict low cholesterol dietary treatment (200 mg/d) of familial hypercholesterolemias the moderate increase of lathosterol mean values was observed with similar changes for desmosterol. Campesterol and sitosterol were not significantely changed.

Conclusions:
Precursors of cholesterol synthesis and phytosterols are useful metabolites for differentiation of patients with familial hypercholesterolemia and they are helpful in controlling of dietary treatment.

Key words:
lathosterol, desmostereol, campesterol, sitosterol, low cholesterol dietary treatment, familial hypercholesterolemia in children