Clinical characteristics of idiopathic hypogonadotropic hypogonadism in females: report of 16 cases
10.3760/cma.j.issn.1000-6699.2012.12.013
- VernacularTitle:女性特发性低促性腺激素性性腺功能减退症的临床研究——附16例病例回顾
- Author:
Tingting ZHANG
;
Xin LI
;
Ling QU
;
Bing XUE
;
Xiaojin LI
;
Xianling WANG
;
Jianming BA
;
Zhaohui Lü
;
Jingtao DOU
;
Yiming MU
- Publication Type:Journal Article
- Keywords:
Idiopathic hypogonadotropic hypogonadism with normal olfaction;
Females
- From:
Chinese Journal of Endocrinology and Metabolism
2012;(12):1001-1004
- CountryChina
- Language:Chinese
-
Abstract:
Clinical characteristics,including primary and secondary sexual characteristics,basal endocrine profiles,and imaging results were reviewed.Follow-up data were recorded.16 patients had normal karyotypes,manifest amastia,infantile genitalia,amenorrhea,and delayed epiphyseal fusion at the knee and wrist joints.Serum gonadotropic hormone levels were significantly below normal values.15 patients underwent a gonadotropin-releasing hormone (GnRH) stimulation test and 6 patients had a prolonged GnRH stimulation test.16 patients underwent pituitary or brain magnetic resonance imaging (MRI),which showed small pituitaries in three patients,wing tips of suspicious nodules in 2 patients,an empty sella turcica in 1 patient,and a missing right olfactory bulb and tract in 1 patient.1 patient had no detectable uterus or accessory organs,while the other patients had primordial uteri.1 patient was diagnosed as a case of severe osteoporosis.1 patient suffered from pituitary stalk interruption syndrome.An artificial menstrual cycle due to hormone replacement therapy was not sustained after discontinuation of hormone therapy.As disease severity and the date of initiating hormone replacement varied,the results of treatment were quite different.For patients of reproductive age,it was rare to see a reversal of idiopathic hypogonadotropic hypogonadism after discontinuation of hormone therapy.
