title: Endocrine studies of the ovulatory disturbances in Wilson's disease (hepatolenticular degeneration).
abstract: Women with Wilson's disease may have severe oligomenorrhea or amenorrhea whose cause is unknown. The endocrine profile of four such cases was investigated by measuring basal values and the response to dynamic tests of hypothalamic, pituitary, thyroid, and adrenal function, which all proved normal. Ovarian function was disturbed, as witnessed by low estradiol, high total testosterone (T) levels with normal free T, and mildly elevated androstenedione. An interference of ovarian follicular aromatase activity possibly due to copper intoxication could explain these findings as the cause of the ovulatory disturbances of Wilson's disease.
mesh_terms: D000293:Adolescent; D000328:Adult; D000568:Amenorrhea / Q000097:blood / Q000209:etiology; D000735:Androstenedione / Q000097:blood; D004958:Estradiol / Q000097:blood; D005260:Female; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000150:complications / Q000503:physiopathology*; D006801:Humans; D009839:Oligomenorrhea / Q000097:blood / Q000209:etiology; D010050:Ovarian Function Tests / Q000379:methods; D010053:Ovary / Q000378:metabolism*; D010396:Penicillamine / Q000627:therapeutic use; D013739:Testosterone / Q000097:blood
chemical_list: D013739:Testosterone; D000735:Androstenedione; D004958:Estradiol; D010396:Penicillamine