Idiopathic (hereditary) hyperlipemia and hypercholesteremia in children

Except for fatty serum and variable degrees of hepatosplenomegaly, children with idiopathic hyperlipemia may remain asymptomatic for many years. Abdominal crises and xanthomata are not necessary concomitants of the disorder in children. The finding of mild hyperlipemia in the mother of siblings with marked hyperlipemia emphasizes the hereditary nature of this disorder. The clinical findings in a child with idiopathic hypercholesteremia and xanthomatosis have been presented. The presence of hypercholesteremia and the absence of overt coronary artery disease in many members of this child's family emphasizes the poorly undestood interrelationships between heredity and environment in the pathogenesis of atherosclerosis. Treatment of hyperlipemia with a low-fat diet tends to correct the abnormal lipid pattern in serum. Coincidental regression of hepatosplenomegaly may also occur. Therapy with nicotinic acid resulted in temporary improvement in the chemical abnormality in one child with idiopathic hyperlipemia. As opposed to experiences with adult hypercholesteremics, high doses of nicotinic acid did not decrease the cholesterol level of a child with idiopathic hypercholesteremia.