We have described previously in the Finnish population an inactivating point mutation (C3T) in the human FSH receptor (FSHR) gene. In women, this mutation causes hypergonadotropic ovarian failure with arrest of follicular maturation and infertility, whereas in men, there is variable suppression of spermatogenesis, but no absolute infertility. To determine whether the same FSHR mutation occurs i...

The association of ataxia, hypergonadotropic hypogonadism and hearing loss is extremely rare. Considerable heterogeneity exists in the literature of the neurological manifestations, age of onset, clinical severity and associated abnormalities. We describe a 24-year-old woman with secondary hypergonadotropic amenorrhea, early-onset progressive spinocerebellar ataxia (SCA), late-onset sensorineur...

In order to prove the acute stimulatory effects of estrogen on pituitary gonadotropin release, we have performed the present experiments in 8 women with a hypergonadotropic state due to surgical castration or primary ovarian failure. They received gonadotropin releasing hormone (Gn-RH) for 12-21 h at the constant rate of 20 micrograms/h. In 5 of the women, estradiol-17 beta was concomitantly ad...

BACKGROUND Premature Ovarian Insufficiency (POI) is classically defined as 4-6 months of cessation of menses (amenorrhea) in women under 40, associated with menopausal level of serum gonadotropins FSH > 40 IU/L and hypo-estrogenism and is also referred to as hypergonadotropic hypogonadism. This disorder can manifest as primary amenorrhea without the onset of menses (menarche), or as secondary a...

Puberty is the period of transition from childhood to adulthood characterized by attainment adult height and body composition, accrual bone strength acquisition secondary sexual characteristics, psychosocial maturation reproductive capacity. In girls, menarche a late marker puberty. Primary amenorrhea defined as absence in ≥ 15-year-old females with developed characteristics normal growth or ≥1...

Federal University of São Paulo (UNIFESP), São Paulo SP, Brazil: Department of Neurology and Neurosurgery; Department of Endocrinology; Department of Ophthalmology. The association between cerebellar ataxia and hypogonadism was first described by Gordon Holmes in 1907. It represents a highly heterogenous syndrome with insidious onset. The hypogonadism of most patients with Gordon Holmes syndrom...

The clinical examination revealed a size 145 cm (-3 SD/M. SEMPE) with a total impuberism. The hormonal tests showed a hypergonadotropic hypogonadism. The abdominal CT scan revealed a left hemi-abdominal mass with a large vascular pedicle (supernumerary spleen?) Figure 1. Our patient was followed for cardiac heart failure. The echocardiographic exploration found a left systolic ventricular dysfu...

OBJECTIVE Deficiency of sex steroids has a negative impact on bone mineral content. In studies conducted on postmenopausal women and animal studies, elevated follicle-stimulating hormone (FSH) levels were found to be correlated with a decrease in bone mineralization and osteoporosis. The aim of the present study was to evaluate bone mineral density (BMD) in adolescent girls with hypogonadotropi...

background: ovarian cysts are among the diseases which cause reproductive failure and economic losses in dairy herds. high yelding dairy cattles are suseptable to reproductive failure caused by ovarian cysts, as a result of their exposure to stressful coditions during lactation. objectives: this study aimed to monitor metabolic profile tests in dairy cows with ovarian cysts in comparison with c...

Bone loss associated with menopause leads to an increase in skeletal fragility and fracture risk. Relevant animal models can be useful for evaluating the impact of ovarian failure on bone loss. A chemically induced model of menopause in which mice gradually undergo ovarian failure yet retain residual ovarian tissue has been developed using the chemical 4-vinylcyclohexene diepoxide (VCD). This s...