B. Koletzko et M. Herzog, HYPERLIPIDEMIA IN CHILDREN AND ADOLESCENT S - DIAGNOSIS AND TREATMENT, Schweizerische medizinische Wochenschrift, 128(13), 1998, pp. 477-485
Severe hyperlipidemias should be diagnosed and treated even in childho
od and adolescence, because vascular lipid deposition in the form of f
atty streaks and progressive atherosclerotic lesions start to develop
early in life. The heterozygous form of familial hypercholesterolemia
found in about 1 of 500 newborn infants, and polygenic forms of hyperc
holesterolemia, are the most frequent forms of primary genetic hyperch
olesterolemias found in children. Secondary hyperlipidemias, e.g. in d
iabetes mellitus, hypothyroidism and renal disease, are relatively fre
quent in children and adolescents and need to be searched for in the d
iagnostic evaluation, because they can be influenced by treatment of t
he underlying disorder. Children and adolescents with severe forms of
hyperlipidemias should be diagnosed and treated early in life. Dietary
modification is the basis of treatment of affected children and can l
ower LDL cholesterol by about 15-20%. In patients with severe hypercho
lesterolemia, dietary cholesterol intake should not exceed 150 mg/day
in children or 250-300 mg/day in adolescents, Even more important is a
reduction of the intake of saturated fats and trans fatty acids and t
heir replacement by polyunsaturated and particularly monounsaturated f
ats. Some additional lowering of LDL cholesterol may be achieved by th
e preferential use of vegetable over animal proteins and of complex ca
rbohydrates over sugars. Repeated motivation, counseling and intensive
practical training of the patient and family, supported by appropriat
e teaching materials, are essential for effective dietary treatment. A
dditional drug treatment is considered in children from the age of 8-9
years of age onwards if, in spite of adequate dietary modification, L
DL cholesterol remains above 190 mg/dl (4.9 mmol/l), or above 160 mg/d
l (3.9 mmol/l) in the presence of additional risk factors. The drugs o
f first choice are anion exchange resins (colestyramin or colestipol)
because of their well documented efficacy and safety. More convenient
to take but often somewhat less effective is beta-sitosterol. If effic
acy or compliance with resins or sitosterin is unsatisfactory, fibrate
s (e.g. bezafibrate, fenofibrate) may be considered as a drug of secon
d choice. Cholesterol synthesis inhibitors are not recommended for gen
eral use in children at this time.