The clinical features of insulin receptor disorders found in Japan are
summarized. About 20 cases of primary receptor mutations (type A synd
rome of insulin resistance, leprechaunism and Rabson-Mendenhall syndro
me) and 16 cases of autoantibodies against insulin receptor (type B sy
ndrome of insulin resistance) are described in Japan. There was a tria
l of IGF-1 for some of the patients, revealing its usefulness.