Dyslipidemia in young Japanese children: its relation to familial hypercholesterolemia and familial combined hyperlipidemia.

Background: Most cases of dyslipidemia found in adults are non-familial. However, in children, especially young children, dyslipidemias other than familial hypercholesterolemia (FH) have not yet been characterized.

Methods: From April 1990 to March 1999, 56 181 children were screened, and 1380 showed abnormal levels of apolipoprotein B (more than 2.5 standard deviations above the mean). Among these, 1198 were re-examined and further characterized by measuring lipids and apolipoproteins, and by their familial histories.

Results: Seventy-seven percent of the children (928 of 1198) recalled were diagnosed as being dyslipidemic. Ninety-one children were FH, 423 were type IIa, 128 were type IIb, 98 were type IV, and 188 were hypoalphalipoproteinemia. The presumed incidence of FH was 0.19%, IIa 0.87%, IIb 0.26%, IV 0.20%, and hypoalphalipoproteinemia 0.39%, taking into account the percentage of subjects who refused recall. At regular follow-ups, in many children with type IIb, the phenotypic expression changes from type IIb to IIa or IV. Thus, lipid and apolipoprotein levels were determined in 77 family members in 34 families of children with type IIb. Forty-five family members were dyslipidemic (type IIa 18, type IIb 11, type IV 16). As a result, 27 children (79%) with type IIb met the criteria for familial combined hyperlipidemia.

Conclusions: Children with dyslipidemia had more family or genetic background than adults. Unexpectedly, children with type IIb were mostly familial combined hyperlipidemia. Thus, setting appropriate eating patterns during childhood might be important for normalizing risk factors for atherosclerotic coronary heart disease, especially in children with FH or type IIb.