Dietary Therapy for Children with Hypercholesterolemia

RAANAN SHAMIR, M.D., Bruce Rappaport School of Medicine, Haifa, Israel EDWARD A. FISHER, M.D., PH.D., Mount Sinai School of Medicine, New York, New York

A patient information handout on diet in children with high cholesterol, written by the authors of this article, is provided on page 685.

Accumulating evidence clearly shows that atherosclerosis begins in youth. The National Cholesterol Education Program (NCEP) has recommended that children at high risk of developing coronary artery disease as adults be screened so that those with elevated low-density lipoprotein (LDL) cholesterol levels can be treated, primarily by modification of diet. The initial approach to these youthful patients is to use the NCEP step I diet. This diet provides calories and nutrients that support normal growth and development, but limits saturated fat and total fat intake to no more than 10 and 30 percent of total calories, respectively, and cholesterol intake to no more than 100 mg per 1,000 kcal per day, to a maximum of 300 mg. If the goal of reducing the LDL cholesterol level to below 130 mg per dL (3.35 mmol per L) is not achieved, the more restrictive step II diet should be initiated. However, the step II diet may not provide sufficient calories and nutrients to support normal growth and development; therefore, trained nutritionists may be required to effectively manage a child on this diet. (Am Fam Physician 2000;61:675-82,685-6.)

See editorial on page 633.

Cardiovascular disease caused by atherosclerosis is the leading cause of death worldwide.¹ Clinical evidence, epidemiologic data and postmortem studies provide convincing evidence that atherosclerosis begins in childhood. Children with homozygous familial hypercholesterolemia develop significant coronary heart disease (CHD) in the first decade of life and frequently die of myocardial infarction before 20 years of age.^2-4 Serum cholesterol levels measured at 22 years of age are predictive of the risk for developing CHD over the next 30 to 40 years.⁵ Data from the Framingham Study⁶ show that cholesterol levels measured in young adults are predictive of CHD mortality 30 years later. In persons with familial hypercholesterolemia, there is a direct association between the duration and severity of hypercholesterolemia and extravascular lipid deposition in tissues.⁷

Scope and Epidemiology of the Cholesterol Problem

Epidemiologic data support the relationship between childhood and adult cholesterol levels,^8-13 and a relationship between hyperlipidemia in the children and premature CHD in the parents.^14,15 Pathologic findings have provided further evidence of the early development of atherosclerosis: young soldiers killed in the Korean War were found, with high frequency, to have advanced coronary artery lesions.¹⁶ Quantitative postmortem estimation¹⁷ of atherosclerosis in coronary arteries and aortas of children and young adults demonstrate a significant relationship between hyperlipidemia and the extent of atherosclerosis. In agreement with the accumulating evidence, it was recently demonstrated that cardiovascular risk factors in children are related to the severity of atherosclerosis found postmortem in such persons who died as young adults.¹⁸

Evidence of Need to Treat Hypercholesterolemia

Studies in adults provide evidence that reduction of serum cholesterol concentrations lowers the risk of developing CHD.¹⁹ The effectiveness of diet in reducing total and low-density lipoprotein (LDL) cholesterol levels is well established.²⁰ Furthermore, studies in young children and adolescents have shown that when adequate calories and nutrients are provided, cholesterol-lowering diets do not impair growth or development.^21-25

Experts with the National Cholesterol Education Program recommend dietary intervention in children with a family history of cardiovascular disease or parental hypercholesterolemia.

Based on these findings, the National Cholesterol Education Program (NCEP) Expert Panel on Blood Cholesterol in Children and Adolescents recommended dietary intervention to lower LDL cholesterol levels in adolescents and children at least two years of age with a family history of cardiovascular disease or parental hypercholesterolemia.²⁶ Because dietary treatment is safe and often effective, physicians should be alert for opportunities to provide appropriate consultation and treatment or to refer patients and families to other health care professionals for consultation.

Approach to Drug Therapy of Hypercholesterolemia

TABLE 1 Dietary Intervention Points in Children and Adolescents with Cardiovascular Risk Factors* Category Total cholesterol (mg per dL) LDL cholesterol (mg per dL) Dietary intervention Acceptable <170 (4.40 mmol per L) <110 (2.85 mmol per L) Recommended population eating pattern Borderline 170 to 199 (5.15 mmol per L) 110 to 129 (3.35 mmol per L) AHA step I diet, other risk factor intervention High >200 (5.20 mmol per L) >130 (3.40 mmol per L) AHA step I diet; step II if necessary LDL = low-density lipoprotein; AHA = American Heart Association. *--Interventions based on cholesterol levels. Risk factors include a family history of premature cardiovascular disease or hypercholesterolemia. Adapted with permission from National Cholesterol Education Program Coordinating Committee. Report of the Expert Panel on Blood Cholesterol in Children and Adolescents. Bethesda, Md.: National Heart, Lung, and Blood Institute, 1991. NIH publication no. 91-2732.

The approach to drug therapy for hypercholesterolemia in childhood can also be found in the NCEP guidelines²⁶ and in recent reviews^27,28 of the subject. The recommendation in the original NCEP guidelines is to augment dietary treatment with the use of bile acid sequestrants in children more than 10 years of age who, after six months to one year of dietary treatment, have failed to lower their LDL cholesterol level to less than 190 mg per dL (4.90 mmol per L), or to below 160 mg per dL (4.10 mmol per L) if they have a family history of premature cardiovascular disease or two or more other risk factors (e.g., low high-density lipoprotein [HDL] levels, diabetes, obesity, hypertension, cigarette smoking and physical inactivity). A number of systemically acting lipid-lowering drugs have proved to be successful in secondary^29,30 and primary³¹ prevention of CHD in adults. Because this article's focus is on dietary therapy and because of the lack of data on the safety of prolonged use of these medications in children, we will not further discuss their use in this article.

Which Children Should Be Screened?

The NCEP has recommended a selective approach in screening children for hypercholesterolemia. Screening should be performed in children more than two years of age who have a positive family history of premature cardiovascular disease or parental hypercholesterolemia.

Positive family history of premature cardiovascular disease is defined as a parent, grandparent or first-degree aunt or uncle who experienced one of the following before the age of 55: myocardial infarction, angina pectoris, peripheral vascular disease, cerebrovascular disease, sudden cardiac death or documented coronary atherosclerosis.

Parental hypercholesterolemia is defined as a total blood cholesterol level of 240 mg per dL (6.20 mmol per L) or higher. Cholesterol levels should also be obtained in patients who smoke, have diabetes mellitus, are obese or have hypertension.

Food Diary Directions: Circle the foods from both sides of the list that your child eats every week. Milk, cheese and dairy products Skim or low-fat (1%) milk Whole and 2% milk Low-fat buttermilk   Evaporated skim milk Evaporated whole milk Nonfat dry milk Dried whole milk solids Nonfat nondairy creamer Nondairy creamers, cream, half-and-half Nonfat sour cream Sour cream Nonfat or low-fat yogurt Whole-milk (regular) yogurt Nonfat or low-fat frozen yogurt and frozen desserts, sorbet, sherbet, popsicles Ice cream, whole-milk frozen yogurt Nonfat dairy whipped toppings Whipped cream, nondairy whipped toppings made with saturated fat Skim milk or low-fat cheeses with less than 5 g of fat per ounce Whole-milk cheeses, processed cheese spreads Nonfat or light cream cheese Regular cream cheese Nonfat or low-fat ricotta and cottage cheese Whole-milk ricotta and cottage cheese Up to four egg yolks per week, egg substitutes Fried or scrambled eggs Meat and proteins Chicken or turkey breast without skin Dark poultry meat, duck Fish or shellfish or low-fat fish sticks or fish cakes (keep shellfish to one serving per week) Seafood prepared with fat, regular or breaded fish sticks or fish cakes Ground chicken breast or turkey breast Ground turkey (dark meat included), chicken nuggets Beef (sirloin, tenderloin, round or flank) or lamb (leg or loin) Prime, marbled cuts of meat Lean ground beef Regular ground beef Fat-free hot dogs Regular hot dogs or sausage Tuna packed in water Tuna packed in oil Fat-free luncheon meats Regular luncheon meats such as salami, bologna, liverwurst Pork (tenderloin, center loin) Pork spare ribs, bacon Protein tips Bake, broil, poach, grill, boil or "fry" in a nonstick pan Fry in oil, braise in fat Eat less than 6 ounces of meat, fish or poultry per day Eat more than 6 ounces of meat, fish or poultry per day Eat meatless sources of protein such as tofu, legumes (beans and peas), vegetarian entrees Eat meatless sources of protein such as tofu, legumes (beans and peas), vegetarian entrees Fruits and vegetables Five servings per day of fruits and raw or frozen vegetables, served raw or steamed, broiled, baked or stir-fried with a minimum of fat Coconut, vegetables fried in deep fat or served with cream, cheese or butter sauces Baked, mashed or boiled potatoes French fries, hash browns, potato chips Breads, cereals and starches Low-fat whole-grain breads, crackers, scones and muffins High-fat crackers, regular biscuits, muffins, croissants, doughnuts, sweet rolls Bagels, English muffins, pita breads Pastries and scones Corn tortillas   Oatmeal and whole-grain cereals, low-fat granola Hot cereal with added fat, regular granola Rice and pasta Pasta and rice mixes with high-fat sauces, fried rice Beans and peas Beans and peas cooked with added fat Breadsticks Regular egg noodles, crispy chow mein noodles Yolk-free noodles   Low-fat cakes, cookies High-fat desserts and baked goods Pretzels, baked tortilla chips, unbuttered popcorn Potato chips, corn chips, buttered popcorn Fats Monounsaturated fats: Canola (rapeseed oil), olive oil Saturated fats: Coconut oil, palm oil, palm cocoa, butter, lard Polyunsaturated fats: Safflower oil, sunflower oil, sesame seed oil, cottonseed oil, soybean oil, corn oil   Soft-tub margarines made with liquid safflower oil, sunflower oil or corn oil Butter, stick margarines Nonfat or low-fat salad dressings Regular salad dressings, in excessive amounts, dressings made with sour cream or cheese Cocoa powder, chocolate syrup Chocolate with butter fat Nuts as snacks in moderation   Natural or low-fat peanut butter Regular peanut butter List any other foods your child consumes on a regular basis not listed above: FIGURE 1. A three-day food diary for the parent of a child being screened for hypercholesterolemia. A realistic goal for children and adolescents is to reduce low-density lipoprotein cholesterol levels to 130 mg per dL (3.35 mmol per L) or below.

Initial Evaluation and Follow-up

TABLE 2 Recommended Daily Caloric Intake in Children and Adolescents* Calories to maintain weight Age (years) Girls Boys 2 1,160 1,250 3 1,270 1,530 4 1,430 1,520 5 1,550 1,680 6 1,760 1,840 7 1,530 1,590 8 1,720 1,750 9 2,000 1,970 10 2,260 2,230 11 1,730 1,930 12 1,950 2,180 13 2,180 2,430 14 2,350 2,780 15 2,130 2,540 16 2,240 2,780 17 2,250 2,970 18 2,250 3,130 *--At the 50th weight percentile. Reprinted with permission from National Research Council. Recommended dietary allowances. 10th ed. Washington, D.C.: National Academy of Sciences, 1989.

Lipid and lipoprotein measurements should be made by an experienced laboratory, especially one that participates in the voluntary quality control program of the Centers for Disease Control and Prevention (CDC). Studies should be performed twice, and an average of the two values should be used to determine further evaluation and treatment.

In children with a positive family history of premature cardiovascular disease, a fasting lipoprotein analysis (total cholesterol, total triglycerides, HDL and LDL cholesterol levels) should be obtained. In children, fasting means consuming nothing except water after midnight. Table 1 provides acceptable, borderline and high LDL cholesterol levels in children.

If there is no family history of premature cardiovascular disease, but the parents have hypercholesterolemia or other risk factors, a nonfasting total cholesterol level is a sufficient initial test. Acceptable levels are less than 170 mg per dL. If the total cholesterol is elevated above this level, a fasting protein analysis should be performed.

In a child with a high cholesterol level (total cholesterol or LDL cholesterol), screening tests for secondary causes of hypercholesterolemia (in particular, diabetes and diseases of the thyroid, liver and kidney) should be performed. Certain medications such as steroids, anticonvulsants and oral contraceptives can also be secondary causes. A more complete list of secondary factors can be found in the NCEP summary.²⁶

Management

The goal of dietary treatment is to reduce LDL cholesterol levels. Table 1 provides the levels of total and LDL cholesterol at which dietary intervention is indicated in children and adolescents with a family history of hypercholesterolemia or premature cardiovascular disease. The recommended initial step to reduce cholesterol levels in children is the institution of a "heart-healthy" diet--one that is low in cholesterol and saturated fat and high in complex carbohydrates, and provides adequate energy for growth and the maintenance of a desirable weight.

AHA Step 1 Diet
The American Heart Association (AHA) step I diet is equivalent to the NCEP step I diet and is well established for this purpose in adults and children more than two years of age.³² In a step I diet, no more than 30 percent of total calories come from fat, less than 10 percent of total calories come from saturated fat and dietary cholesterol is restricted to 100 mg per 1,000 kcal, not to exceed 300 mg per day. Many foods that are high in cholesterol are also high in saturated fat. A step I diet substitutes foods rich in monounsaturated and polyunsaturated fats for those rich in saturated fats. To provide effective dietary guidance, a complete dietary assessment is required. This is often difficult to accomplish in the typical office setting, but suggestions for estimating dietary intake and modifying the diet are noted below, as well as in Figure 1 and the accompanying patient information handout.

A realistic goal for children and adolescents is to reduce low-density lipoprotein cholesterol levels to 130 mg per dL (3.35 mmol per L) or below.

Goals for Dietary Therapy
Although the ideal goal for dietary intervention is to lower the LDL cholesterol level to less than 110 mg per dL (2.85 mmol per L), in some cases this will not be possible. There are at least two reasons for this: (1) the child already consumes a heart-healthy diet or (2) the elevation may be high enough that the percentage of reduction achieved by dietary modification (typically no more than 20 percent and frequently less than 10 percent) still may not lower LDL cholesterol to the target goal. A more realistic goal for children and adolescents with a high level of LDL cholesterol is to reduce the level to lower than 130 mg per dL (3.35 mmol per L).

Follow-Up and Initiation of AHA Step II Diet
To confirm the effectiveness of dietary changes, the LDL cholesterol level should be checked three to six months after treatment is started and yearly thereafter. Because lowering LDL cholesterol levels reduces the progression of CHD, any lowering of LDL cholesterol levels is valuable, even if the goal is not accomplished.

If the step I diet does not achieve the goal, the NCEP recommended dietary approach is for a trial of the more restrictive step II diet, which contains less than 7 percent of total calories from saturated fat and restricts dietary cholesterol to less than 75 mg per 1,000 kcal. Despite the seemingly modest reductions of the step II diet, there is the potential to develop nutritional deficiencies because of a more limited food selection. Therefore, patients placed on the step II diet should be closely monitored and should receive consultation with a clinician who has considerable nutritional expertise.

Monitoring for Other Cardiovascular Risk Factors
Other risk factors for cardiovascular disease, such as cigarette smoking, physical inactivity and obesity, are sometimes present in childhood and adolescence. Although they are not specifically addressed in this article, recommendations should be made for appropriate lifestyle modifications, such as maintaining a desirable weight, exercising, reducing sedentary activities (e.g., television watching) and smoking cessation. Involving all family members in the dietary changes and lifestyle modifications is important for long-term adherence to these treatment modalities.

TABLE 3 Sample Recommended Intake of Total and Saturated Fat Based on Daily Caloric Intake*   Calories from fat Grams from fat Total calories Total fat (30% of calories) Saturated fat (10% of calories) Total fat (30% of calories) Saturated fat (10% of calories) 2,000 600 calories 200 calories 67 g 22 g *--Based on recommendations for a nine-year-old girl at the 50th percentile for weight. Based on Table 2, her recommended caloric intake is 2,000 calories per day. NOTE: For a step I diet in this patient, the maximal number of calories from all types of fat (total fat) is derived by multiplying 2,000 by 30 percent and from saturated fat, by multiplying 2,000 by 10 percent. To convert the fat calories (total fat or saturated fat) to grams, use this conversion formula: 1 g of fat = 9 calories. Thus, 600 calories of total fat is equivalent to 600 divided by 9, or 67 g.

Assessing Dietary Habits and Recommending Changes

Families need to learn to select foods that are low in total fat, particularly saturated fat, and cholesterol. The diet should include a variety of foods to ensure adequate intake of carbohydrates, protein and other essential nutrients, such as calcium and iron. Sufficient calories need to be consumed to ensure adequate growth and development.

In addition to lowering total fat and saturated fat intake, evidence has accumulated about the adverse effect of transunsaturated fatty acids (resulting from hydrogenation of oils, such as in margarines) on blood cholesterol levels.³³ Hydrogenated fat consumption can be minimized by choosing liquid and soft fats over hardened fat products.

Evaluation of Dietary Habits

Ask the parents or child to keep a three-day food record before the next appointment (Figure 1). If they have not yet done this at the time of the follow-up visit, consider asking them to complete the food diary while they are in the waiting room, using a checklist of common foods. The items in the food diary can be arranged in two columns: foods listed on the left side are acceptable for eating on a daily basis; foods on the right side should be limited.

The physician can quickly scan the food diary and determine if the child is consuming too many inappropriate foods just by checking the right side of the page. Parent and child can be directed to use foods listed on the left side of the list as substitutes for those on the right side. They can also be referred to the AHA Cookbook for Kids. In addition, suggestions for lunchbox and snack ideas (see patient information handout) are helpful and should be provided. Families with Internet access should be encouraged to visit the AHA Web site (http://www.americanheart.org).

Table 2 provides the recommended daily caloric intake for girls and boys based on age and the 50th percentile of weight.³⁴ Once the recommended total daily caloric intake is determined, as illustrated in Table 3, the maximal number of calories and grams that should come from fat and saturated fat can be calculated based on the NCEP recommendations for a step I diet (up to 30 percent of the daily caloric intake from all types of fat and up to 10 percent from saturated fat). Similar calculations can be made for the step II diet, using 7 percent of total daily calories as the upper limit for saturated fat. Total fat intake in children and adolescents should not be reduced to less than 20 percent of the daily caloric intake.³²

Some of the material in this article and other relevant information can also be found on the American Heart Association Web site (http://www.americanheart.org).

The authors thank committee member Stephen Daniels, M.D., Ph.D., and ad hoc writers Marilyn Cerino, R.D., and Betsy V. Harris, M.Ed., R.D., L.D., for their help in the preparation of the manuscript, and Jill F. Fisher, for editorial assistance.

This article is one in a series developed in collaboration with the American Heart Association. Guest editor of the series is Rodman D. Starke, M.D., Senior Vice President of Science and Medicine, American Heart Association, Dallas.

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The Authors

RAANAN SHAMIR, M.D.,
is currently director of the Division of Pediatric Gastroenterology and Nutrition in the Department of Pediatrics at Rambam Medical Center, Haifa, Israel. He is also a lecturer in pediatrics at Tel Aviv University Sackler Faculty of Medicine. Dr. Shamir is a graduate of the Hebrew University Hadassah Medical School, Jerusalem, and obtained clinical and research training in pediatrics, nutrition and lipid metabolism at the Beilinson Medical Center, Israel, the Children's Hospital of Philadelphia and the Medical College of Pennsylvania, Philadelphia.

EDWARD A. FISHER, M.D., PH.D.,
is director of lipoprotein research at the Zena and Michael A. Wiener Cardiovascular Institute of the Mount Sinai School of Medicine of the City University of New York, N.Y. He is also a professor of medicine and pediatrics, and an attending physician at the Lipid Disorders Clinic at Mount Sinai Hospital. A graduate of the New York University School of Medicine, New York City, Dr. Fisher obtained clinical and research training at Duke University School of Medicine, Durham, N.C., Boston Children's Hospital, Massachusetts Institute of Technology, Boston, and the National Institutes of Health, Washington, D.C.

Address correspondence to Edward A. Fisher, M.D., Ph.D., Mount Sinai School of Medicine, 1 Gustave Levy Place, Box 1030, New York, NY 10029. Reprints are not available from the authors.

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