Association between physical activity, adiposity, and lipid abnormalities in children with familial hyperlipidemia.

BACKGROUND: The benefits of physical activity in children have been studied extensively; however, its role in children with familial hyperlipidemia (FH) is unknown. OBJECTIVE: To determine associations between physical activity, adiposity, and lipid profiles in children with FH. DESIGN: A physical activity questionnaire was completed by 147 children with FH. Correlations between activity levels, body mass index (BMI), and fasting lipid profiles were determined. RESULTS: The mean age of patients was 12.5+/-3.2 years with a mean total cholesterol of 6.17 mmol/l (238 mg/dl), low-density lipoprotein-cholesterol of 4.43 (171), high-density lipoprotein-cholesterol of 1.08 (42), and triglyceride levels of 1.51 (134). Patients had greater weight for height indices than normal, with a mean BMI z score of +0.90+/-1.30 SD (P<0.001 versus normal), and with 21% of the participants being more than 2 SD above normal. Higher BMI z scores significantly correlated with higher triglyceride levels (r=0.33; P<0.0001) and greater time spent in sedentary pursuits (r=0.24; P=0.004), in particular watching television (r=0.26; P=0.003). The increased time that other family members spent in physical activity significantly correlated with a lower BMI z score (r=-0.21; P=0.01) of the patient and greater time spent in physical activity (r=0.24; P=0.003). There was no association between patients' physical activity levels and lipid profile or BMI. CONCLUSION: Similar to the general population, children with FH are also at risk of becoming overweight. Increased adiposity significantly correlated with the greater sedentary activities of the patient, lower physical activities of the family, and higher triglyceride levels. Physical activity levels of the patient correlated with family activity levels.

A randomized crossover trial of combination pharmacologic therapy in children with familial hyperlipidemia.

We sought to determine whether a low-dose combination of a bile acid-binding resin (colestipol) with an hydroxymethylglutaryl CoA reductase inhibitor (pravastatin) would result in improved acceptability, compliance, and effectiveness in lipid-lowering compared with conventional therapy with a higher dose of a bile acid-binding resin only, with fewer side effects. We performed a randomized, crossover open-label clinical trial with two 18-wk medication regimens separated by an 8-wk washout period in 36 children and adolescents with familial hypercholesterolemia or familial combined hyperlipidemia. The regimens included colestipol 10 g/d (10 pills) versus a combination of colestipol 5 g/d with pravastatin 10 mg/d (six pills). All patients were maintained on a fat-reduced diet. Acceptability was better with the combination regimen. Mean compliance was similar and suboptimal (approximately 60%) with all medication components. Mean relative LDL cholesterol lowering was significantly better with the combination regimen (-17 +/- 16% versus -10 +/- 13%; p = 0.045), although insufficient to achieve recommended target values in the majority of patients on either regimen. Both regimens were equally free of adverse effects, with no important effect on chemistry or hematologic values. Patient-reported adverse effects were more common with the conventional-dose colestipol-only regimen. Compliance with medication regimens using the bile acid-binding resins is suboptimal, although combination with a low dose of a statin may result in better lipid lowering.