Extreme insulin resistance syndrome.

Congenital extreme insulin resistance syndrome has rarely been reported in Taiwan. In 1982, a girl of a consanguineous marriage was noted to have increased body hair, an enlarged clitoris, and acanthosis nigricans at birth. Two months later, she received an operation for bilateral polycystic evaries. She was found to have diabetes at 8 years old and was treated with insulin. In March 1999, she was referred to our clinic with growth retardation and poor glycemic control. She had a characteristic face with a saddle nose, broad mouth, large low-set ears, absence of subcutaneous fat, and deformed nails. Although a very high dose of insulin (> 10 IU/kg/day) was used, her glycemic control was very poor (HbA1c 13.8%). Pediatricians should remain alert for the manifestations of extreme insulin resistance.