Cholesterol reduction by a high-glucose diet in a patient with homozygous familial hypercholesterolemia. A preliminary report.
Homozygous familial hypercholesterolemia is refractory to standard dietary or drug therapy. Recent studies, however, suggest that a high-carbohydrate/low-fat diet may reduce circulation cholesterol levels in normal or hyperlipidemic subjects. In this regard, we treated a nine year old boy with homozygous familial hypercholesterolemia with a liquid formula diet containing 82 to 90 percent of total calories as glucose. The diet was given as a constant nasogastric infusion or as intermittent daytime drinks followed by a nighttime infusion. Plasma total and low-density lipoprotein cholesterol fell from basal levels of 719 mg/dl and 676 mg/dl to 456 mg/dl and 434 mg/dl, respectively, after one week of therapy. After approximately 14 weeks of treatment, plasma total and low-density lipoprotein cholesterol levels were 311 mg/dl and 277 mg/dl, each representing approximately a 58 percent decrease from basal levels. The fall in circulating cholesterol levels was accompanied by a regression of xanthomatous skin lesions, a rise in plasma insulin levels and no change in plasma glucose or glucagon concentrations. No adverse effects of therapy occurred. We conclude that high-carbohydrate diets may be a safe and effective adjunct in the treatment of homozygous familial hypercholesterolemia.
D000924:Anticholesteremic Agents / Q000627:therapeutic use; D002648:Child; D002784:Cholesterol / Q000097:blood*; D004040:Dietary Carbohydrates / Q000008:administration & dosage / Q000627:therapeutic use; D004041:Dietary Fats / Q000008:administration & dosage; D005947:Glucose / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000178:diet therapy* / Q000628:therapy; D007441:Intubation, Gastrointestinal; D008297:Male
D000924:Anticholesteremic Agents; D004040:Dietary Carbohydrates; D004041:Dietary Fats; D002784:Cholesterol; D005947:Glucose