Endocrinological Abnormalities in Prader‐Willi Syndrome

Ten patients with typical Prader-Willi syndrome were studied for their short stature, hypogonadism, and obesity. Tne following results were obtained. 1)  GH secretion was variable, ranging from subnormal to normal, although all shared short stature in common. 2)  Two of the 4 adolescent patients were diagnosed as having hypo-gonadotropic hypogonadism. The remainder disclosed normal response to LH-RH stimulation. Of the two patients with normal LH-RH stimulation test, one showed normal testosterone production. 3)  In one child who developed overt diabetes, there remained elevated basal plasma insulin and depressed RBC-insulin binding, despite weight control. There appears to be a significant heterogeneity in endo crinological derangement of Prader-Willi syndrome.