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
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
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DOI：
10.1210/jc.2007-1621
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发表时间：
2008-03-01
影响因子：
5.8
通讯作者：
Chiba, Hitoshi
中科院分区：
文献类型：
--
作者：
Nagasaka, Hironori;Miida, Takashi;Chiba, Hitoshi
背景：高密度脂蛋白（HDL）由富含载脂蛋白E （apoE）和缺乏载脂蛋白E的HDL颗粒组成。富含apoe的HDL水平在出生时较高，但在出生后随着低密度脂蛋白(LDL)-胆固醇的相互升高而降低。目的：该研究的目的是阐明家族性高胆固醇血症（FH）患儿出生后是否富含载脂蛋白e的HDL降低，FH是一种由LDL清除受损引起的疾病。方法：我们测量了10名FH儿童（1名纯合子和9名杂合子）、12名非FH兄弟姐妹和75名健康对照者在生命第一年富含载脂蛋白e的hdl -胆固醇和ldl -胆固醇。结果：出生时，纯合子FH患儿检测不到富含载脂蛋白e的hdl -胆固醇，而杂合子FH患儿比非FH患儿和对照组低（4 +/- 2 vs 12 +/- 4和11 +/- 4 mg/dl， P < 0.001）。在3-4个月时，纯合子和杂合子FH患儿富含载脂蛋白e的hdl -胆固醇升高，非FH患儿兄弟姐妹和对照组降低。在12个月时，四组中富含载脂蛋白e的高密度脂蛋白胆固醇水平相似（6-7毫克/分升）。相比之下,低密度脂蛋白胆固醇浓度总是两倍杂合的FH孩子non-FH兄弟姐妹和控件(出生时,50 + / - 15和25 + / - 7和25 + / - 5 mg / dl, P < 0.001;在3 - 4个月大,159 + / - 29与71类似于16和73 + / - 15 mg / dl, P < 0.001;在12个月的年龄,156 + / - 29和75 + / - 18和76 + / - 17毫克/分升,P < 0.001)。结论：FH患儿出生时ApoE- rich HDL水平较低，出生后1年升高至正常水平，与正常患儿相反。
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 similar to 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.