CD36 deficiency predisposing young children to fasting hypoglycemia.
Fatty acid (FA) β-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.
Nagasaka
Hironori
H
Division of Metabolism, Chiba Children's Hospital, Chiba 266-0007, Japan. nagasa-hirono@k2.dion.ne.jp
Yorifuji
Tohru
T
Takatani
Tomozumi
T
Okano
Yoshiyuki
Y
Tsukahara
Hirokazu
H
Yanai
Hidekatsu
H
Hirano
Ken-ichi
K
Hui
Shu-Ping
SP
Hirayama
Satoshi
S
Ito
Tetsuya
T
Chiba
Hitoshi
H
Miida
Takashi
T
D001786:Blood Glucose / Q000378:metabolism; D018955:CD36 Antigens / Q000172:deficiency* / Q000235:genetics*; D002675:Child, Preschool; D005215:Fasting / Q000502:physiology*; D005227:Fatty Acids / Q000378:metabolism; D005260:Female; D005434:Flow Cytometry; D020022:Genetic Predisposition to Disease; D006801:Humans; D007003:Hypoglycemia / Q000235:genetics*; D007223:Infant; D007328:Insulin / Q000097:blood; D007657:Ketone Bodies / Q000097:blood; D008297:Male; D009154:Mutation / Q000235:genetics / Q000502:physiology; D010084:Oxidation-Reduction; D010641:Phenotype; D011446:Prospective Studies
D001786:Blood Glucose; D018955:CD36 Antigens; D005227:Fatty Acids; D007328:Insulin; D007657:Ketone Bodies