Congenital generalized lipodystrophy with insulin-resistant diabetes

✍ Scribed by Soichi Kodama; Masato Kasuga; Atsushi Seki; Michindo Ninomiya; Takashi Sakurai; Yorihiko Morishita; Masafumi Matsuo; Tamotsu Matsuo

• Publisher: Springer
• Year: 1978
• Tongue: English
• Weight: 481 KB
• Volume: 127
• Category: Article
• ISSN: 0340-6997
• DOI: 10.1007/bf00445766

✦ Synopsis

Hyperglycemia, glucose intolerance, hyperinsulinemia and resistance to exogenous insulin were found in a 10-year-old Japanese boy diagnosed as having congenital generalized lipodystrophy.

Studies on insulin receptors of circulating mononuclear leucocytes indicated that insulin-resistant diabetes combined with congenital generalized lipodystrophy may be due to disturbance of insulin binding to membrane receptors. No insulin-binding antibody or antibody that impairs insulinreceptor binding was found.

Plasma glucagon showed an exaggerated response to L-arginine before treatment. After treatment with a controlled diet and an oral sulfonylurea (500 mg/day) for 4 weeks, there was improvement in the plasma glucagon response to L-arginine. Improvement in the hyperglycemia, hyperinsulinemia and acanthosis nigricans was also observed.

On the other hand, on completion of a 7-day high-fat diet, a marked increase in serum free fatty acids, triglycerides and flqipoproteins was observed. The total plasma post-heparin lipolytic activity during the high fat diet was within the normal range. However, the level of protamine-inactivated activity was 3 times that of the control.