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Reduced Apolipoprotein E-Rich High-Density Lipoprotein Level at Birth Is Restored to the Normal Range in Patients with Familial Hypercholesterolemia in the First Year of Life

Division of Metabolism (H.N., T.T., M.T.), Chiba Children’s Hospital, Chiba 266-0007, Japan; Division of Clinical Preventive Medicine (T.M.), Niigata University, Graduate School of Medical and Dental Sciences, Niigata 951-8510, Japan; Department of Cardiovascular Medicine (K.H., A.O.), Osaka University, Graduate School of Medicine, and Department of Pediatrics (T.Y.), Kyoto University, Graduate School of Medicine, Kyoto 606-8507, Japan; Department of Pediatrics (H.T.), Fukui University, Graduate School of Medicine, Fukui 910-1193, Japan; Departments of Clinical Laboratory Medicine (H.C., S.-P.H.), Hokkaido University Hospital, and Department of Pediatrics (K.K.), Hokkaido University, Graduate School of Medicine, Sapporo 060-8638, Japan

Address all correspondence and requests for reprints to: Hironori Nagasaka, M.D., Chiba Children’s Hospital, 579-1 Heta Cho, Midori-Ku, Chiba, Japan. E-mail: nagasa-hirono{at}k2.dion.ne.jp; or Takashi Miida, M.D., Ph.D., Niigata University, Graduate School of Medical and Dental Sciences, Niigata 951-8510, Japan, Department of Cardiovascular Medicine, Asahimachi 1-754, Chuo-ku, Niigata, Niigata 951-8520, Japan.

Background: High-density lipoprotein (HDL) consists of apolipoprotein E (apoE)-rich and apoE-poor HDL particles. ApoE-rich HDL level is high at birth but decreases after birth with reciprocal elevation in low-density lipoprotein (LDL)-cholesterol.

Objectives: The objective of the study was to clarify whether apoE-rich HDL decreases after birth in children with familial hypercholesterolemia (FH), a disorder caused by impaired LDL clearance.

Methods: We measured apoE-rich HDL-cholesterol and LDL-cholesterol during the first year of life in 10 FH children (one homozygote and nine heterozygotes), 12 non-FH siblings, and 75 healthy controls.

Results: At birth, apoE-rich HDL-cholesterol was undetectable in a homozygous FH child and lower in heterozygous FH children than non-FH siblings and controls (4 ± 2 vs. 12 ± 4 and 11 ± 4 mg/dl, P < 0.001). At 3–4 months, apoE-rich HDL-cholesterol increased in homozygous and heterozygous FH children and decreased in non-FH siblings and controls. At 12 months, apoE-rich HDL-cholesterol levels were similar among these four groups (6–7 mg/dl). In contrast, LDL-cholesterol concentration was always twice as high in heterozygous FH children as non-FH siblings and controls (at birth, 50 ± 15 vs. 25 ± 7 and 25 ± 5 mg/dl, P < 0.001; at 3–4 months of age, 159 ± 29 vs. 71 ± 16 and 73 ± 15 mg/dl, P < 0.001; at 12 months of age, 156 ± 29 vs. 75 ± 18 and 76 ± 17 mg/dl, P < 0.001).

Conclusion: ApoE-rich HDL level is low at birth in FH children and increases to the normal level in the first year of life, opposite to the change in normal children.