Laron syndrome

Laron syndrome, also called growth hormone insensitivity or growth hormone receptor deficiency, is an inherited autosomal recessive disorder in which the body cannot respond to growth hormone and so makes too little insulin-like growth factor 1, even though growth hormone levels in the blood are high. It is usually caused by mutations in the growth hormone receptor gene. It is named for Zvi Laron, who first described the syndrome in 1966. People with it typically have very short stature, obesity, facial and skull differences, small genitalia in males and low blood sugar. About 250 people worldwide are known to have it, most of Semitic ancestry. Because growth hormone therapy does not work, it is treated mainly with recombinant IGF-1 (mecasermin). Research has noted a lower and later incidence of cancer and type 2 diabetes among affected people.