Abstract

Summary: This study was designed to evaluate prospectively the continuing effects of dietary management in 44 children with familial hypertriglyceridemia. For obese children, weight reduction programs were instituted by metabolic dietitians. For nonobese children, for obese children with successful weight reduction (or for those who could not lose weight but would follow an altered diet), a modification of the National Institutes of Health (NIH) type IV diet was provided. Adherence to the diet program was monitored by monthly reassessment for 6 months in the outpatient clinic. In 43 children whose average age was 13 years at the time of diagnosis, mean ± SE plasma triglycerides were reduced after 6 months of weight reduction-NIH type IV diet from 253 ± 33 to 116 ± 8 mg/dl, P < 0.01. After 6 months on diet, the group mean decrement in weight (1 ± 1 kg) was not significant, and decrements in weight failed to correlate with decrements in plasma triglycerides, r = 0.131. Despite this failure to reduce weight appreciably, after 6 months on diet plasma triglyceride levels were reduced to normal (< 140 mg/dl) in 32 of the 43 children. At 8 months' follow-up in 13 children, mean plasma triglyceride was 170 ± 31 mg/dl, having been 290 ± 86 at time of diagnosis, P < 0.01. Plasma triglyceride levels at 8 months were normal in 5 of the 13 children. Plasma triglycerides were normal in 4 of 5 children with evaluation at 1 year, in 3 of 7 at 18 months, and in 4 of 14 at 22–26 months. When weight gain was proportionately greater than accretion of height, and where no attention to either caloric intake or composition was given, triglycerides remained elevated, whereas dietary adherence was generally accompanied by reduced or normal triglycerides. Amelioration of familial hypertriglyceridemia on the NIH type IV diet is a realizable goal in children, but requires persistent, repetitive reexamination and reinstruction. Speculation: Dietary management of pediatric familial hypertriglyceridemia may be important as a primary, longitudinal approach to reduction of the increased atherosclerotic risk attendant to familial hypertriglyceridemia.

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