Insulin Resistance in a Boy with Congenital Generalized Lipodystrophy
作者:
HIROKAZU,
TSUKAHARA KIYOSHI,
KIKUCHI HIDESHI,
KUZUYA EIKO,
ITO YOKO,
ODA ATSUSHI,
KOSAKI TAKAKO,
KAKEHI HARUO,
NISHIMURA KAZUNURI,
YAMADA YASUNAO,
YOSHIMASA HIROO,
IMURA HARUKI,
期刊:
Pediatric Research
(OVID Available online 1988)
卷期:
Volume 24,
issue 6
页码: 668-672
ISSN:0031-3998
年代: 1988
出版商: OVID
数据来源: OVID
摘要:
We have studied insulin resistance in a 12-year-old Japanese boy who presented with congenital generalized lipodystrophy. Oral glucose tolerance test exhibited a diabetic pattern with normal fasting plasma glucose. Results from euglycemic glucose clamp study showed decreases in both insulin sensitivity and responsiveness. Both the patient's erythrocytes and Epstein-Barr virus transformed lymphocytes showed low-normal insulin binding with a slight reduction in binding affinity in the latter. Insulin binding to the cultured fibroblasts was decreased due to a lowered affinity. In addition, they displayed a rightward shift of the insulin dose-response curve for D-14C-glucose uptake with no decrease in the maximum uptake. Insulin-stimulated autophosphorylation and kinase activity of the wheat germ agglutinin purified receptors from the Epstein-Barr virus-transformed lymphocytes appeared normal. The reason for some discrepancies in insulin binding among the cells remains unknown, and we cannot formulate a conclusion as to whether or not a primary binding defect of insulin receptors exists and contributes to insulin resistance in the patient. The decrease in insulin responsiveness demonstrated in the glucose clamp study may result from a defect at the rate-limiting step in the postbinding process of insulin action, presumably a defect in the glucose transport system in muscle tissues. The defect may be secondary to changes in in vivo circumstances.
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