RT Journal
A1 Shires R, Whyte MP, Avioli LV
T1 IDiopathic hypothalamic hypogonadotropic hypogonadism with polyostotic fibrous dysplasia
JF Archives of Internal Medicine
JO Archives of Internal Medicine
YR 1979
FD October 1
VO 139
IS 10
SP 1187
OP 1189
DO 10.1001/archinte.1979.03630470095031
UL http://dx.doi.org/10.1001/archinte.1979.03630470095031
AB A 22-year-old woman had polyostotic fibrous dysplasia (POFD) and idiopathic hypothalamic hypogonadotropic hypogonadism (isolated gonadotropin deficiency). Recurrent fracture of dysplastic bone during childhood was associated with primary amenorrhea, clinical and laboratory evidence of estrogen deficiency, and subnormal circulating and urinary gonadotropin levels during adolescence. Gonadorelin (luteinizing hormone-releasing hormone) stimulation initially showed a luteinizing hormone (LH) response but absent follicle-stimulating hormone (FSH) response. After three months without estrogen and progesterone and after four days of gonadorelin "priming," a subsequent gonadorelin infusion produced an enhanced LH and FSH response. All other tests of peripheral and trophic hormone levels and pituitary trophic hormone reserves were normal. Whereas POFD is known to occur with sexual precocity and other endocrinopathies, to our knowledge this is the first report of its association with isolated gonadotropin deficiency.(Arch Intern Med 139:1187-1189, 1979)