Anterior Pituitary Function in the Prader-Labhart-Willi (PLW) Syndrome

Abstract

Serum FSH, LH, PRL, TSH, GH, estradiol (E2) and testosterone (T) were measured in 2 male and 3 female patients with the Prader-Labhart-Willi Syndrome. Adrenal and thyroid function was within normal limits, as was GH secretion for 3 of the patients. Serum TSH and hPRL were normal in all patients tested before and after TRH administration. In contrast, LRH administration caused a variable increase in serum FSH, LH, E2 and T with the largest increments observed in patients with more advanced sexual maturation. Clomiphene did not alter serum FSH, LH and T. Following human chorionic gonadotropin (hCG) administration the increase in serum T was variable in the two male patients tested. The above data suggest that there is a considerable degree of variation in the function of the hypothalamic-pituitary-gonadotropin axis in the Prader-Labhart-Willi syndrome. Long-term administration of LRH or clomiphene is the most helpful procedure that can be used to allow a precise evaluation of the functional reserve of the hypothalamic-pituitary-gonadotropin axis in such patients.