The reproductive endocrine system in cystic fibrosis: 2. Changes in gonadotrophins and sex steroids following LHRH.

Hypothalamic-pituitary-gonadal function was assessed in forty-seven patients with cystic fibrosis (CF) by the 3-hr infusion of 100 microgram of synthetic gonadotrophin-releasing factor. LHRH and the results compared with a group of children being evaluated for short stature and delayed puberty ('controls'). Levels of gonadotrophins and sex steroids were measured prior to and during the infusion. In prepubertal boys, LH and FSH release evoked by LHRH was significantly greater (P less than 0.001) in 'control' subjects than in CF patients. In pubertal boys, LH and FSH release was also greater in 'controls' than in CF, though to a lesser degree (P less than 0.05). In pubertal girls, responses to LHRH were comparable for LH and slightly greater (P less than 0.05) in 'controls' for FSH. In the earliest pubertal groups of both sexes (male-Tanner genitalia stage 2; females-Tanner breast stage 2), LH secretion was similar in patients with CF and 'control' subjects. Significant increments of testosterone and oestradiol in pubertal CF patients do not occur until 6 h after the LHRH infusion begins, in contrast to a rise at 3 h in 'control' subjects. These data suggest that prepubertal boys with CF, who are the most impaired in height, weight and skeletal maturation, also have measurable abnormalities of LHRH-releasable gonadotrophin secretion. Despite continued impaired weight growth, pubertal patients do attain essentially normal gonadotrophin secretory responses to LHRH administration and are similar to subjects with constitutional delayed adolescent development in reproductive endocrine physiology.