CD36 deficiency predisposing young children to fasting hypoglycemia
CD36 deficiency predisposing young children to fasting hypoglycemia
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DOI：
10.1016/j.metabol.2010.08.008
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发表时间：
2011-06-01
影响因子：
9.8
通讯作者：
Miida, Takashi
中科院分区：
文献类型：
--
作者：
Nagasaka, Hironori;Yorifuji, Tohru;Miida, Takashi
脂肪酸（FA）β - 氧化缺陷会导致低血糖。我们的目的是确定CD36（一种长链脂肪酸的膜转运蛋白）缺乏是否会使儿童易患低血糖。在禁食过夜后，我们在51名有发作性低血糖病史的学龄前儿童和49名年龄匹配的健康对照儿童的禁食12小时、14小时和16小时点测量了碳水化合物和脂肪酸代谢的参数。同时，检测血小板和单核细胞上CD36的表达以确定表型。51名低血糖儿童中有6名，49名对照儿童中无人被诊断为I型CD36缺乏。分别有4名和3名儿童被诊断为II型CD36缺乏。I型CD36缺乏组的低血糖经常复发。在任何禁食点，I型CD36组的血糖和胰岛素浓度均显著低于其他组：血糖，与对照组相比P < 0.001，与II型/野生型CD36低血糖组相比P < 0.01或P < 0.001；胰岛素，与对照组相比P < 0.001，与II型/野生型CD36低血糖组相比P < 0.01。I型组的游离脂肪酸浓度始终比其他组高1.5 - 2.0倍，而总酮体浓度始终约为其他组的三分之二。在II型、野生型和对照组之间，除野生型组的游离脂肪酸浓度显著较低（P < 0.05）外，其他参数无显著差异。这些结果表明，I型CD36缺乏而非II型CD36缺乏会使学龄前儿童易患低血糖。（C）2011爱思唯尔公司。保留所有权利。
Fatty acid (FA) beta-oxidation defects cause hypoglycemia. Our aim was to determine if CD36-a membrane transporter for long-chain FAs-deficiency predisposes children to hypoglycemia. After overnight fasting, we measured parameters for carbohydrate and FA metabolisms at 12-, 14-, and 16-hour fasting points in 51 preschool children with histories of episodic hypoglycemia and 49 age-matched healthy controls. Simultaneously, the expressions of CD36 on platelets and monocytes were examined to determine the phenotypes. Six of the 51 hypoglycemic children and none of the 49 control children were diagnosed as having type I CD36 deficiency. Four and 3 children were diagnosed as having type II CD36 deficiency, respectively. Hypoglycemia was often recurrent in the type I CD36 group. At any fasting point, the type I CD36 group showed significantly lower blood glucose and insulin concentrations than the other groups: glucose, P < .001 vs control group and P < .01 or P < .001 vs type II/wild-type CD36 hypoglycemic groups; insulin, P < .001 vs control group and P < .01 vs type II/wild-type CD36 hypoglycemic groups. Free FA concentration in the type I group was always 1.5- to 2.0-fold higher than that in the other groups, whereas the total ketone body concentration was consistently about two thirds of that in the other groups. Among the type II, wild-type, and control groups, there were no significant differences in the parameters except that the wild-type group showed significantly lower FFA concentration (P < .05). These results suggested that type I CD36 deficiency but not type II CD36 deficiency predisposes preschool children to hypoglycemia. (C) 2011 Elsevier Inc. All rights reserved.