RESUMO
BACKGROUND: Familial Combined Hyperlipidemia is an inherited disorder affecting cholesterol and triglycerides metabolism, well known myocardial infarction risk factors. The FCHL clinical presentation is usually silent until the third decade although children can be affected, and the more recent opinion is that precocious diagnosis is mandatory in preventing complications. Aim of this study is to examine the effectiveness of the diet therapy (Step-One-Diet) in a group of 13 children affected by Familial Combined Hyperlipidemia. METHODS: The patients have been submitted to a normocaloric diet, 30% fat of the total caloric daily intake according with the Dietary Intervention Study in Children (Step-One-Diet). The patients then have been submitted to a two year-follow-up and lipoprotein levels (total cholesterol, LDL-cholesterol, triglycerides and apolipoprotein B), nutritional status (macro- and micro-nutrients) as well as anthropometric data (height, weight, BMI) have been monitored. RESULTS: Results showed a 10% total cholesterol and 30% triglycerides decrease, Iron and Calcium intake show increased levels approaching to the normal ones after controlled diet, while cholesterol intake was correct on both regimen. The growth parameters show a decrease in weight only in two obese and two overweight patients. CONCLUSIONS: The present study confirmed the effectiveness and safety of the Step-One-Diet in children patients, allowing triglycerides normalization in 60% of the patients, and a 10% cholesterol decrease, in agreement with the complex genetic inheritance of the disease.