title: 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.
abstract: 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.
  
  
  OBJECTIVE
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Metabolism, Chiba Children's Hospital, 579-1 Heta Cho, Midori-Ku, Chiba, Japan. nagasa-hirono@k2.dion.ne.jp
authors.forename: Hironori
authors.initials: H
authors.lastname: Nagasaka
authors.forename: Takashi
authors.initials: T
authors.lastname: Miida
authors.forename: Kenichi
authors.initials: K
authors.lastname: Hirano
authors.forename: Akemi
authors.initials: A
authors.lastname: Ota
authors.forename: Tohru
authors.initials: T
authors.lastname: Yorifuji
authors.forename: Tomozumi
authors.initials: T
authors.lastname: Takatani
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Tsukahara
authors.forename: Masaki
authors.initials: M
authors.lastname: Takayanagi
authors.forename: Shu-Ping
authors.initials: SP
authors.lastname: Hui
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Chiba
mesh_terms: D001057:Apolipoproteins E / Q000097:blood*; D008076:Cholesterol, HDL / Q000097:blood*; D008078:Cholesterol, LDL / Q000097:blood; D005260:Female; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012016:Reference Values
chemical_list: D001057:Apolipoproteins E; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL